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Show NANOS ABSTRACTS Editor's Note: The abstracts from platform and poster presentations at the annual winter North American Neuro- Ophthalmology Society ( NANOS) meeting of that year always appear in the June issue of the Journal of Neuro - Ophthalmology. Platform presentations appear first, followed by poster presentations. Only the first author's name is listed. Abstracts of the 28th Annual North American Neuro- Ophthalmology Society ( NANOS) Meeting, Copper Mountain, Colorado, February 9- 14, 2002 PLATFORM PRESENTATIONS Endocrinologic Abnormalities in Idiopathic Intracranial Hypertension ( Pseudotumor Cerebri) in Men First Author: Andrew Lee, MD, Iowa City, Iowa Background: Idiopathic intracranial hypertension ( IIH) is uncommon in men. Endocrinologic abnormalities have been reported in some cases of IIH. Methods: We identified cases of IIH in men and performed endocrinologic testing in 8 patients including serum free T4, thyroid stimulating hormone ( TSH), estradiol, Follicle stimulating hormone ( FSH), luteinizing hormone ( LH), testosterone, sex hormone binding globulin, free testosterone, and percentage free testosterone. Results: Two patients had abnormal estradiol, four had abnormal FSH and LH, and seven had low testosterone levels. Conclusion: Endocrinologic abnormalities in male patients with IIH may be a clue to the pathogenesis of the disorder. Perceptual Correlates Of The Human Dorsal Light Reflex First Author: Michael Brodsky, MD, Little Rock, Arkansas Background: Dissociated vertical divergence ( DVD) has been attributed to a primitive dorsal light reflex that emerges in humans when single binocular vision is precluded in infancy. If this is the case, then DVD should be associated with a subjective sensation of tilt. Methods: Alternate cover testing was performed in 9 patients with dissociated vertical divergence, who viewed a vertical object held in the sagittal plane. Results: Alternate occlusion disclosed a tilt in the subjective visual vertical in 8 of the 9 patients with DVD. Upon occlusion of the fixating eye, the upper pole of a vertical object held in the sagittal plane was perceived as initially tilted toward the side of the covered eye. The ensuing cy-clovertical divergence movement served to restore normal vertical orientation to the visual environment. Conclusions: This sequence of perceptual alterations supports the notion that DVD is a human dorsal light reflex, which restores vertical orientation when unequal binocular visual input produces a visual tilt in the roll plane. Cerebral Venous Pressure Studies in Idiopathic Intracranial Hypertension First Author: lohn King, MD, Melbourne, Australia Objective: To measure cerebral venous sinus pressures in patients with idiopathic intracranial hypertension ( IHH), and to study the effect of simultaneously lowering intracranial pressure by cervical puncture. Methods: Twenty- one patients with IIH and 10 patients with other illnesses underwent cerebral venography and manometry by the transfemoral route. In 11 patients, 8 with IIH, a second neuroradiologist performed a Cl- 2 puncture immediately after the initial measurements. The intracranial pressure was lowered by removal of 20- 25 mis of CSF and the venous pressure measurements were repeated. Results: Nineteen of 21 cases with IIH showed a pressure drop of between 9 and 46 mm Hg across the transverse sinuses ( TS). Of the 10 patients with diseases other than IIH, 7 had normal studies, but 3 showed the same pressure drop across the TS, 2 had diabetes with unilateral TS thrombosis and one had minocycline- induced intracranial hypertension. Lowering the intracranial pressure by Cl- 2 puncture abolished the pressure drop across the TS. Conclusion: Cerebral venous hypertension is common in IIH but can be relieved by lowering intracranial pressure. It is suggested that the obstruction to venous outflow is „ 156 , pOL10.1097/ 01. wno. 0000019666.41Q46, be Copyright © Lippincott Williams & Wifk JNCUTO ins. Unauthorized reproduction o • hthalmol, Vol. 22, No. 2, 2002 ns article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 caused by partial collapse of the transverse sinuses, a secondary effect due to raised intracranial pressure. Comparison of Opening Pressures During Lumbar Puncture in the Prone and Lateral Positions in Patients with Idiopathic Intracranial Hypertension First Author: Daniel Jacobson, MD, Marshfield, Wisconsin Background: Performing LP in the office in some patients with IIH is difficult due to their body habitus. Accordingly, radiologists are often asked to perform the procedure using fluoroscopic guidance. During this procedure, the OP is typically recorded in the prone position. But, it is not known whether the OP obtained in the prone position is comparable to that obtained in the lateral decubitus position. Objective: To compare the opening pressure ( OP) obtained during lumbar puncture ( LP) in the prone and lateral positions in patients with idiopathic intracranial hypertension ( IIH). Methods: Following informed consent, consecutive patients with suspected or confirmed IIH who required LP for diagnostic confirmation or management were prospectively enrolled. The procedures were performed using fluoroscopic guidance. The OP was first recorded in the prone position. After the patient was then placed in the lateral position with their legs fully extended, the OP was again recorded. The Wilcoxon signed rank test ( two- tailed) was used to determine whether there was a significant difference between the OP recorded in these two positions. The Spearman rank correlation test ( two- tailed) was used to determine whether there was a relationship between body mass index ( BMI) and OP. Results: Of the nine patients enrolled thus far, two were excluded: one because she probably did not have IIH and one because she refused LP. Of the remaining seven patients, all were women and ranged in age from 18 to 39 years ( median age, 25 years) and had BMI that ranged from 22 to 47kg/ M2 ( median BMI, 33kg/ M2). The OP in the prone position ranged from 240 to 430mmH2O ( median, 340mmH2O), while that recorded in the lateral position ranged from 190 to 420mmH2O ( median, 320mmH2O) ( p = 0.30). The pressure was higher in the prone position than in the lateral position in five of the patients by an average of 5 lmmH20, and was higher in the lateral position than in the prone position in two of the patients by an average of 35mmH20. While a weak inverse relationship between BMI and OP in the prone ( r = - 0.40, p = 0.40) and lateral ( r = - 0.31, P = 0.50) position was suggested, the results were not significant. Conclusions: As a group, there was no significant difference in the OP in the prone and lateral positions. But, the small number of patients and substantial overlap of values in these two groups limits the power of this conclusion. Accordingly, although using the prone measurement for patients may be acceptable when the value is markedly elevated, we do not recommend relying on this for diagnostic purposes if the prone value is near the normal limit, either above or below it. Radiosurgery and Optic Nerve Tolerance: Fact and Fiction First Author: Scott Stafford, MD, Rochester, Minnesota Background: The tolerance of the ON to single fraction radiation is frequently quoted as 8 Gy ( 1,5) However, this threshold dose has been questioned and is thought to be overly conservative based on more recent data utilizing improved neuroimaging and dose- planning software ( 2,3,4). Objective: To evaluate the radiation tolerance of the optic nerve ( ON) after radiosurgery. Methods: We reviewed the dose plans of 218 Gamma Knife ® procedures ( 215 patients) for patients with benign tumors of the sellar and parasellar region ( meningiomas, n = 122; pituitary adenomas, n = 89; craniopharyngiomas, n = 7 patients) and calculated the maximum radiation dose to the optic apparatus. Malignant primary and metastatic skull base tumors were excluded as visual decline often occurs due to disease progression and is not radiation- related. Median patient age was 52 years. Previous surgery or radiation therapy ( mean dose, 50.2 Gy) was performed in 75 ( 35%) and 24 ( 11%) patients, respectively. The median prescription volume 6.3cc ( range, 0.09- 30.4cc); the median prescription dose was 18Gy ( range, 12- 30). Median clinical follow- up was 40 months ( range, 4- 115) with 94% of patients having more than 9 months of follow- up. Results: The median maximum radiation dose to the ON was 10 Gy ( range, 0.4- 16.0). Four optic nerve complications ( 1.8%) occurred at a median of 31 months after the last radiosurgical procedure. Conclusions: Visual decline was noted in only three of 156 patients ( 1.9%) receiving 8Gy or more to the ON and may be attributed to RS in one patient receiving 7Gy to the ON. Optic nerve toxicity after contemporary radiosurgery is rare, and the risk is likely increased for patients receiving prior radiation to that region. References: 1. Girkin C, Comey C, Lunsford L, et al. Ophthalmology 1997; 104: 1634- 43. 2. Leber K, Bergloff J, Pendi G. JNeurosurg 1998; 88: 43- 50. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 NANOS ABSTRACTS 3. Morita A, Coffey R, Foote R, et al. JNeurosurg 1999; 90: 42- 9. 4. Stafford SL, Pollock BE, Foote RL, et al. Neurosurgery 2001; 49: 5. 5. Tishler RB, Loeffler JS, Lunsford LD, et al. Int J Radiation Oncol-ogyBiolPhys 1993; 27: 215- 21. Toxoplasmic Anterior Optic Neuropathy First Author: James Banta, MD, Miami, Florida Objective: To describe clinical findings and course of toxoplasmic anterior optic neuropathy and to differentiate primary and secondary involvement. Methods: Observational case series Results: Toxoplasmic anterior optic neuropathy was divided into two types. Type I was defined as secondary infectious involvement of the optic nerve head from an adjacent focus of chorioretinitis that resolved with chorioretinal scarring. Type II was defined as primary involvement of the optic nerve head that resolved without chorioretinal scarring. Visual acuity improved after treatment in both Type I and Type II, however the visual prognosis was worse in Type I patients due to macular involvement. Eighty- three percent of Type II patients had a final visual acuity equal or better than 20/ 25 compared with 50% of Type I patients. Visual field defects were present in all patients, most frequently arcuate or altitudinal ( 62%). Delay in diagnosis was common ( 54%), especially in Type II patients ( 71%). Vitreous inflammation was absent on the initial examination in 31% of patients. Conclusions: Toxoplasmic anterior optic neuropathy is an uncommon manifestation of ocular toxoplasmosis. Delays in diagnosis are common because of frequent lack of typical chorioretinitis or vitreous inflammation. Adjacent macular involvement strongly influences visual outcome. Symptomatic Chiari I Malformation First Author: Kimberly Cockerham, MD, Pittsburgh, Pennsylvania Background: Chiari I malformation ( ACM) refers to the descent of the cerebellar tonsils at least 5mm below the foramen magnum. ACM patients often suffer symptoms of dizziness, imbalance, headache, retro- orbital pressure, transient visual obscurations and neck pain. Intracranial noises are also often present. The symptoms are very similar to patients with idiopathic intracranial hypertension but ACM patients do not typically develop disc edema. Objective: To perform complete neuro- ophthalmic evaluations in patients with symptomatic ACM. Methods: Prospective case series of 32 patients with significant cerebellar tonsillar descent on sagittal magnetic resonance imaging. A complete neuro- ophthalmic examination was performed to include visual field testing and HRT nerve fiber analysis. Results: 6 men and 26 women were evaluated. The mean age was 37 years ( range 8- 58). The most common visual complaint was transient visual obscurations lasting seconds ( n = 30). Pain or fullness behind the eyes was present in 29 patients. Persistently decreased vision or double vision was not present in any of those examined. Afferent function was normal including visual field testing. Fundus examination was remarkable for the lack of venous pulsations in 24 patients ( 75%). Mild hyperemia with telangiectatic vessels was noted in 3 patients. Disc edema was not present. No patients demonstrated cranial nerve deficits or nystagmus. In four patients seen following ACM decompression surgery who noted resolution of symptoms, normal spontaneous venous pulsations were present. In 3 patients where ACM decompression surgery failed to provide symptomatic improvement, spontaneous venous pulsations were not noted following surgical intervention. Conclusions: Patients with symptomatic Arnold Chiari malformation have a higher prevalence of dampened spontaneous venous pulsations. Successful surgical decompression can result in improved symptoms and return of spontaneous pulsations. Lack of spontaneous venous pulsations following ACM decompression may predict procedure failure. Autoimmune- Related Retinopathy and Optic Neuropathy ( ARRON) Syndrome First Author: John Keltner, MD, Sacramento, California Objective: To evaluate patients who have unexplained visual loss without evidence of malignancy whose clinical and immunologic profiles are consistent with autoimmune-related retinopathy and optic neuropathy ( ARRON) syndrome. Methods: The clinical information and immunologic findings from U. C. Davis and other referred patients were tabulated and analyzed. Results: This study included 12 ARRON patients, 8 women and 4 men, with the average age of presentation to U. C. Davis Ophthalmology Department at 50 years ( range 37 to 75). Visual loss in these patients was frequently asymmetric. Visual acuity varied from 20/ 20 to no light perception ( NLP). Visual field loss consisted of mild to severe loss with generalized peripheral constriction. Some patients demonstrated normal field in one eye with severe mean deviation loss or NLP in the other eye. Fundus findings showed 11 of the 12 patients had optic nerve atrophy and 8 of the 12 patients had non- specific retinal changes except ^ 160. , „.. , „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 for blood vessel attenuation in 3 patients. ERG abnormalities were present in 10 out of 11 patients. Evaluation for cancer was negative in all patients. Systemic immunologic diseases were present in 8 out of 12 patients, which included systemic lupus erythematosus ( SLE), rheumatoid arthritis ( RA), hyperthyroidism, hypothyroidism, celiac sprue, Sjogren's disease, psoriatic arthritis, and idiopathic thrombocytopenic purpura ( ITP). Immunologic evaluation on both Western blot and immunohistochemistry demonstrated a variety of autoimmune reactions to retina and optic nerve. Visual function in 7 out of 12 patients were stabilized with immunomodulation, which included some combination of prednisone, other immunosuppressive therapy, IVIG, and/ or plasma exchange. Conclusions: The autoimmune- related retinopathy and optic neuropathy ( ARRON) syndrome represents a diverse group of patients without cancer, but have visual loss and may exhibit antibodies to retina and optic nerve. These patients frequently have other autoimmune diseases and their visual loss may respond to steroid and other immunomodulators. The Effect of Decreased Visual Acuity on Clinical Color Vision Tests First Author: Timothy McCulley, MD, Cincinnati, Ohio Background: In patients with poor visual acuity, it is not always clear whether errors on color vision testing are due to an abnormality in color vision or inadequate acuity. Objective: To evaluate the effect of decreased visual acuity on clinical color vision tests. Methods: The ODs of 12 healthy subjects ( 11 females, 1 male, mean age 38, range 20 to 61) with no history of color vision abnormality and normal visual acuity were fogged with plus lenses at the phoropter to visual acuity of logMAR 1.88 ( 20/ 1500) at near and assessed with Farnsworth D- 15 hue discrimination test, Ishihara color plates, and Hardy- Rand- Rittler ( HRR) color plates. Subjects were similarly tested at progressively lesser degrees of fogging, at 0.1 logMAR intervals, up to acuity logMAR 0.67 ( 20/ 94) and at baseline, logMAR 0 ( 20/ 20). For all levels of visual acuity the mean number of errors made with each color vision test was compared with baseline using the paired rtest. To compare examination devices, for each subject the worst visual acuity that the number of errors did not differ from baseline was determined for each color vision test. These acuities were averaged for each color vision test and compared using repeated measures analysis of variance. Results: The number of errors on color vision testing did not significantly differ from baseline, using the cutoff p = 0.05, up to and including visual acuities logMAR 1.57 ( 20/ 750) on D- 15 panel, 1.27 ( 20/ 375) on HRR plates, and 0.88 ( 20/ 150) on Ishihara plates. The measures analysis of variance on the most decreased unaffected acuity was highly significant ( p < 0.001). All three- color vision tests were significantly different from each other ( all p < 0.005). Conclusions: Of the three- color vision assessment techniques, Ishihara plate testing was most dependent and the Farnsworth Panel D- 15 was least dependent on good visual acuity. Color vision testing was accurate up to and including acuity of logMAR 1.57 ( 20/ 750) with the D- 15 panel, 1.27 ( 20/ 375) with HRR plates, and0.88 ( 20/ 150) with Ishihara plates. Successful Treatment with Acetazolamide in Pregnant Women with Pseudotumor Cerebri First Author: Misha Pless, MD, Pittsburgh, PA Objective: To Report 6 patients who were treated with acetazolamide during pregnancy for the diagnosis of pseudotumor cerebri ( PTC). Specifically to identify potential teratogenic effects of Diamox and to evaluate the settings where acetazolamide treatment is appropriate in the setting of pregnancy. Methods: A retrospective chart review of 171 patients treated at the Neuro- ophthalmology unit of the University of Pittsburgh's Eye and Ear Institute between 1996 and 2001 with the diagnosis PTC identified 6 patients who received acetazolamide during pregnancy. Results: Between July 1996 and December 2000, 171 patients were diagnosed with PTC at the University of Pittsburgh's Eye and Ear Institute. In this database 6 women were identified who were either pregnant at the time of diagnosis or became pregnant during treatment with acetazolamide ( age range 20- 32). The study was designed to prospectively follow the 6 patients into completion of pregnancy and beyond delivery. Their infants were examined by pediatricians in the standard fashion. All 6 patients had moderate or severe PTC with Frisen grade III- V papilledema at presentation to neuro- ophthalmic care ( LP opening pressure 32 to > 55 cm CSF). All patients had mild to moderate visual function and field compromise. All 6 individuals were given both medical and surgical choices for the treatment of PTC. All patients understood the reported teratogenic effects of acetazolamide. Two patients became pregnant while being treated with acetazolamide 1 gm/ d while the remaining 4 women received the diagnosis of PTC and began treatment within the first trimester of pregnancy ( range 8- 14 weeks) with similar dosing. One child was born at 32 weeks gestation secondary to premature rupture of membranes. None of the 6 infants has had any Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 NANOS ABSTRACTS developmental delay or congenital malformation ( well-baby follow up range: 3- 24 months). Conclusions: Acetazolamide is the main treatment modality for PTC. Because of lack of human data the drug continues to be classified in class C of teratogenicity. Because many of the patients diagnosed with PTC are reproductive age women, adequate data regarding the teratogenic risk of acetazolamide treatment is critical. There is mounting anecdotal evidence that acetazolamide is indeed safe in pregnancy. This study substantiates this notion in a small cohort of patients. Acetazolamide treatment may be considered an effective and safe treatment of women with PTC after the 8th week of gestation. Video versus Graphic Representation of the Swinging Flashlight Test: Enhancing Examiner Accuracy in the Detection of Afferent Pupillary Defects First Author: Shane Kim, MD, Philadelphia, PA Background: Video documentation and graphic representation of the swinging flashlight test ( SFT) have never been systematically tested as a method to enhance observers' ability to detect afferent pupillary defects ( APDs). Objective: To compare the ability of different examiners to identify and quantify APDs presented as videos and as graphs ( pupillary diameter versus time) of the SFT. Methods: Video recordings and graphs ( N = 60) of the SFT in normals and in patients with APDs: mild (< 0.6 log unit), moderate ( 0.6- 0.9 log unit), or severe (> 0.9 log unit) were presented to ophthalmic caregivers ( N = 21) divided into 3 groups: medical students and technicians ( MST), residents ( R), and neuro- ophthalmologists ( N). For each video or graph, the examiners made a diagnosis of normal, mild, moderate, or severe APD. A scoring system was developed to assess accuracy. Results: In all groups, the mean accuracy score using graphs was higher than that of videos ( p < 0.001). Using videos, the groups recognized moderate APDs 42.9% ( MST), 66.4% ( R), and 88.1% ( N) of the time. Using graphs, identification of moderate APDs improved in all groups: 64.3% ( MST), 88.6% ( R), and 96.4% ( N). For videos, a gross error ( failing to detect a severe APD) was made 16.7% ( MST), 5% ( R), and 0% ( N) of the time. For graphs, a gross error was made 26.7% ( MST), 1.7% ( R), and 0% ( N) of the time. Conclusions: There was a direct correlation between the experience of the examiner, and the ability to identify moderate and severe APDs. The ability to detect moderate APDs increased in all groups when response curves were used. The disparity between the accuracy of experts and nonexperts in identifying moderate APDs decreased when graphs were used, suggesting that graphic representation of the SFT obtained through pupillography may be an important diagnostic tool. The Role of Unilateral Temporal Artery Biopsy First Author: Nicholas Volpe, MD, Philadelphia, Pennsylvania Background: The routine use of bilateral temporal artery biopsy ( TAB) to exclude giant cell arteritis ( GCA) has been suggested because of a small percentage of discordant results in bilateral biopsies. Objective: To examine the role of unilateral TAB in suspected GCA. Methods: We identified 181 patients who underwent TAB ( 1990- 2001). Follow- up information ( obtained by telephone or record review) for patients with unilateral negative TAB was reviewed for potential adverse outcomes due to missed or delayed diagnoses of GCA. Patients' presenting signs, symptoms and laboratory values were recorded. Comparisons of clinical profiles between subsets of patients were performed using Fisher exact tests. Results: Follow up information was available for 88 of 102 patients ( 86%) who had unilateral negative biopsies. One patient of 88 ( 1%) had a subsequent positive contralateral TAB; no adverse outcomes occurred for this patient or for any other patients with unilateral negative TAB. Compared with patients who had unilateral positive or who underwent bilateral TAB ( n = 78), those who had unilateral negative TAB ( n = 88) had a significantly lower prevalence of jaw claudication ( p = 0.005). Compared with patients diagnosed with GCA ( n = 39), those with unilateral negative TAB ( n = 88) had significantly lower frequencies of jaw claudication ( p = 0.001), " chalky white" optic disc appearance ( p = 0.002) and fever ( p < 0.0001). The most common indications for biopsy in patients with unilateral negative TAB were elevated erythrocyte sedimentation rate ( ESR) ( 74%), headache ( 69%), visual symptoms ( 58%), and ophthalmic signs ( 52%). Conclusions: Unilateral TAB was associated with a low frequency ( 1%) of subsequent positive contralateral TAB, and was not associated with adverse visual or neurologic outcomes for any patient. This suggests that a unilateral TAB is sufficient to exclude a diagnosis of GCA in populations for which clinical suspicion is low. Jaw claudication, " chalky white" optic disc appearance, and fever should raise suspicion for a diagnosis of GCA. „ 162 . , „ „ , „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 The Multifocal Electroretinogram in a Neurophthalmology Practice First Author: Candice Chen, MD, New York, New York Objective: To investigate the use of the multifocal electroretinogram ( mfERG) in a neuro- ophthalmology practice. Methods: Between March 1999 and November, 2001, 71 patients were referred from a single neuro- ophthalmology practice for mfERG testing. Each patient had a mfERG recorded with a bipolar Burian- Allen contact lens electrode or a DTL electrode after the pupil was dilated withl% tropic-amide. The mfERGs were recorded using equipment and software from EDI. The display, 50j in diameter, consisted of 103- scaled hexagons. One 7- minute run was recorded from OU. Amplitudes and latencies were examined with the VERIS software and programs written in MATLAB. Results: Sixty- one of the patients were tested for diagnostic purposes: to rule out the retina as the site of disease ( ROR, n = 39); to distinguish between diseases ( 2D, n = 9); to rule out functional or non- organic causes ( F, n = 7); and to follow progression of a retinal disease ( P, n = 6). The remaining 10 were part of ongoing studies ( e. g. the effects of ION and of the papillorenal syndrome on the mfERG). In 3 of the 61 patients seen to aid in the diagnosis, we were unable to obtain usable records due to poor fixation, technical problems or the patient's inability to tolerate the test. Of the remaining 58 patients, the mfERG was abnormal in 14 of the 37 ROR patients, 6 of 9 the 2D patients, and none of the 7 F patients. Five of the 6 progression patients could be followed with the mfERG. The sixth progression patient was unable to tolerate the test. Conclusions: The mfERG aided in the diagnosis and the planning of treatment of most of patients. Of particular importance was the mfERG's ability to distinguish diseases of the retina ( pre- ganglion cells) from diseases of the ganglion cells and the optic nerve. A Functional MRI Study of Benign Essential Blepharospasm First Author: Robert Baker, MD, Lexington, Kentucky Background: Our laboratory has focused on the etiology and treatment of BEB. we now apply the modality of FMRI to this effort. Objectives: We sought differences in blink induced brain activation patterns between 5 BEB patients and 5 normals. Methods: Blood oxygen level- dependent intensity images were collected as 16, 8mm thick slices using a t2- star gradient echo epi sequence, co- registered with anatomic images. Spatially normalized and isotropically blurred activation maps for each subject were combined within BEB and controls groups to generate maps of intersubject mean fractional signal change. Results: Greater activation during spontaneous and voluntary blinking was seen in BEB compared with controls in the anterior visual cortex, primary motor cortex, central region of the thalamus, and superior cerebellum. Differences were greatest for voluntary blinking. Conclusions: The activation observed might represent a hyperactive cortical circuit linking visual cortex, limbic system, supplementary motor cortex, cerebellum, and supranuclear motor pathways innervating the periorbital muscles. Field Investigation in Brazil of a Giant Pedigree of Leber's Hereditary Optic Neuropathy First Author: Alfredo Sadun, MD, PhD, Los Angeles, California Objective: To travel to rural Brazil to find as many members of a previously unstudied pedigree known to have Leber's hereditary optic neuropathy ( LHON). Further, to conduct extensive epidemiological, neuro- ophthalmologi-cal, psychophysical and blood examinations to conduct multi- variant analysis on epigenetic factors and gene linkage analysis. Background: A mother from rural Brazil had a 14- year- old son go blind in one eye. Her brothers had also gone blind as young adults. Molecular analysis of blood samples showed that all 4 individuals had LHON, 11778, homoplasmic, J- haplotype. Methods: Investigators from the University of Sao Paulo traveled to the remote city of Colatina, Brazil, where they were joined by an international team of investigators. An extensive questionnaire, previously used on a large LHON pedigree, was further adapted for local conditions and translated into Portuguese. The full LHON pedigree consisted of approximately 300 members. These patients moved through 6 stations: 1) epidemiological interview; 2) blood drawing for molecular analysis, biochemical studies, and gene linkage analysis; 3) comprehensive neuro- ophthal-mological examination; 4) psychophysical studies including contrast sensitivity, color vision, and threshold Amsler grid testing; 5) Humphrey visual field 30- 2 perimetry; and 6) fundus photography. Results: A total of 295 patients were found to be members of this large pedigree. 273 patients were located and examined in Brazil and 4 others in the U. S. The pedigree began in Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 NANOS ABSTRACTS 1861 when a couple immigrated to Brazil from Verona, Italy. Five of their 7 children went blind. Of these 8 generations, the percent of penetrance of generation 2- 7 went as follows: 100%, 71%, 60%, 34%, 15%, and 9%. The male-to- female ratio also varied by generation. Beginning with the second generation, this was. 6, .5, .64,1,1, and 1. Age of onset varied from 10 to 64 ( average age = 29) and current visual acuities varied from light perception to 20/ 400. Conclusions: 277 members of a 295- member pedigree with LHON 11778 were comprehensively studied. Analysis of environmental risk factors as well as gene linkage analysis of the nuclear DNA will help us address the perplexing question of why only a fraction of individuals with Leber's express the disease. The genetic and epigenetic factors that may modulate the threshold in this all or none expression of LHON have yet to be elucidated. Furthermore, this fully described database may provide an excellent opportunity for future clinical trials of any purported neuroprotective agent. Low- Contrast Sloan Letter Chart Testing as a Candidate Visual Outcome Measure for the Multiple Sclerosis Functional Composite ( MSFC) First Author: Laura Baker, MD, Philadelphia, Pennsylvania Background: Visual dysfunction is one of the most common and disabling aspects of neurologic impairment in multiple sclerosis ( MS). The MSFC, a new clinical trial outcome measure, includes a timed 25- foot walk, 9- hole peg test, and the paced auditory serial addition test, but does not yet include a measure of visual function. Adding a visual dimension to the MSFC will likely increase its applicability in MS populations. Objective: To examine low- contrast Sloan letter chart testing as a candidate visual outcome measure for the Multiple Sclerosis Functional Composite ( MSFC). Methods: Sub- study of randomized clinical trial; cross-sectional study. Binocular low- contrast Sloan letter chart ( LCSLC) testing was administered to a subgroup of participants in the International MS Secondary- Progressive Avonex Controlled Trial ( IMPACT). Data from an ongoing study of visual outcome measures at the University of Pennsylvania ( Penn MS Study- relapsing- remitting MS) were also analyzed. Results: Rank- correlations of LCSLC scores (# letters correct at 5%, 2.5%, 1.25% o contrast levels) with scores from the MSFC and Expanded Disability Status Scale ( EDSS) were significant to a modest- moderate degree, supporting a potential role for LCSLC testing in MS. Median binocular Snellen acuities were 20/ 20 ( 20/ 16- 20/ 100) in both studies. Among 5 visual outcome measures in the Penn MS Study ( including high- contrast visual acuity), LCSLC scores best-distinguished MS patients from age- matched controls on the basis of visual function, even following detailed refractions ( p < 0.006, logistic regression analysis). Conclusions: LCSLC testing captures aspects of neurologic impairment in MS not entirely captured by high-contrast visual acuity, EDSS, or MSFC scores, and demonstrates excellent potential as a candidate MSFC visual component. Hering is Hooked by Monocular Adaptation of the VOR: Lessons from Sixth Nerve Palsy First Author: Agnes Wong, MD, St. Louis, Missouri Background: The effects of paralytic strabismus on the vestibulo- ocular reflex ( VOR) have not been systematically investigated. Objective: To analyze the horizontal VOR in patients with unilateral peripheral sixth nerve palsy. Methods: Twenty- one patients ( 6 severe, 7 moderate, 8 mild) and 15 normal subjects were studied. Subjects underwent sinusoidal ± 10 deg head on body rotations in yaw at approximately 0.5 and 2 Hz. Eye movement recordings were performed using magnetic scleral search coils in OU during monocular viewing in light and in darkness. Results: In all patients, horizontal VOR gains in darkness were decreased in the paretic eye in both abduction and adduction, but remained normal in the non- paretic eye in both directions. In light, horizontal visually enhanced VOR ( VVOR) gains were normal in both eyes in moderate and mild palsy. In severe palsy, horizontal VVOR gains remained low in the paretic eye during viewing with either eye, while those in the non- paretic eye were higher than normal when the paretic eye viewed. Conclusions: In darkness, horizontal VOR gains are reduced during abduction of the paretic eye in all patients, as anticipated in sixth nerve palsy. Gains are also reduced during adduction of the paretic eye, suggesting that innervation to the medial rectus has changed. After severe palsy, vision does not increase abducting or adducting horizontal VVOR gains to normal in the paretic eye, but causes secondary increase in VVOR gains to values above unity in the non-paretic eye, when the paretic eye fixates. In mild and moderate palsy, vision enhances the VOR in the paretic eye but causes no change in the non- paretic eye, suggesting a monocular readjustment of innervation selectively to the paretic eye. This monocular adaptation in the VOR of the paretic ^ 164 „.. „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 eye reduces asymmetric movement of retinal images during head motion and prevents nystagmus. A Spatial Working Memory Component to Visuospatial Neglect First Author: Christopher Kennard, MD, London, United Kingdom Background: In the hemispatial neglect syndrome, usually due to a right inferior parietal lesion, patients tend to only search the ipsilateral half of the visual scene. Several lines of research suggest that multiple components underlie the neglect syndrome but a common theme has been a bias of attention. In an experimental paradigm using a visual search task a single case study indicated impaired spatial working memory ( SWM) across saccades contributing to abnormal search in neglect. Objective: To determine the prevalence of any such trans-saccadic spatial working memory deficit in a population of neglect patients using a multi- target search task. Methods: Sixteen patients with left neglect due to right-hemisphere infarction or hemorrhage were studied and their performance was compared with a group of 15 healthy aged- matched volunteers. Subjects viewed a computer monitor showing a series of search displays comprising 19 targets ( T's) embedded amongst 44 distracters ( L's). The subjects were instructed to search for Ts among Ls fixating each " new" target found. Whilst looking at any target they had to click a response button if they considered it to be a new discovery. Any later reclick on a previous found target indicated a failure to recall that the target had previously been detected. Results: All neglect patients found significantly fewer targets on the left half of the screen than the right. The neglect group had a significantly higher re fixation rate than the control group. Eleven of the 16 patients had re fixation rates beyond the upper 95% confidence limit for control subjects and had a higher reclick rate than healthy controls. Ten of the 16 patients had reclick rates beyond the upper 95% confidence interval for normal subjects indicating an impairment in SWM. Conclusions: In this visual search task many of the neglect patients erroneously judged previously located targets to be new discoveries, significantly more often than healthy individuals. An impairment in keeping track of search locations may, therefore, be a relatively common component of the neglect syndrome and may contribute to the severity of the condition. The Effect of Fibrous Dysplasia on the Optic Nerve Canal and Vision First Author: Edmond FitzGibbon, MD, Bethesda, Maryland Background: In fibrous dysplasia of bone ( FD), fibro-osseous tissue replaces normal bone. The anterior cranial base is frequently involved in the polyostotic form. The optic nerve passing through the sphenoid wing is often found to be partially or completely encased by FD on CT. Some surgeons have advocated prophylactic optic nerve decompression to preserve sight in these patients. Objectives: The purpose of this study was to: 1) correlate the CT findings and neuro- ophthalmic exams in FD patients, 2) quantitate the CT findings and optic canal parameters, and 3) establish evidence- based management for optic nerves encased with FD. Methods: 38 patients with FD ( age range 4- 59) were included in this study. Inclusion criteria included FD of the sphenoid wing. Exclusion criteria included prior optic nerve decompression procedure or treatment with bisphos-phonate therapy. The CT images were reformatted through the optic canal and involvement, height, and width were measured by a masked neuro- radiologist who also measured CTs of age- and sex- matched controls. Visual acuity, Humphrey visual fields, Ishihara color test and funduscopic exams were performed by a neuro- ophthalmologist masked to the CT findings. Results: Of the 68 involved canals, 70.6% had circumferential canal involvement and 29.4% had partial involvement. 5 patients had acuity or color vision abnormalities attributable to red green color blindness or amblyopia. Of the 38 patients, only one had vision loss in one eye from optic neuropathy. Conclusions: The majority ( 97%) of the FD patients with optic nerve involvement had normal eye exams. Therefore, prophylactic optic nerve decompression is not recommended based on radiographic findings alone since they do not correlate with vision loss. Prognostic Value of Optical Coherence Tomography of THE Retinal Nerve Fiber Layer in Patients with Compressive Optic Neuropathy First Author: Julie Falardeau, MD, Iowa City, Iowa Background: Currently, there is no means available to assess visual prognosis of patients with compressive optic neuropathy who are being evaluated for treatment except for degree of pallor of the optic nerve. The number of surviving, viable ganglion cell axons might be related to the thickness of the retinal nerve fiber layer and could be used to assess reversibility of visual field loss. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 NANOS ABSTRACTS Objectives: To assess the prognostic value of optical coherence tomography ( OCT) of the retinal nerve fiber layer in predicting visual recovery in patients with compressive optic neuropathy and visual field loss. Methods: 15 eyes ( 11 patients) with compressive optic neuropathy from meningioma of the optic nerve ( 3), intracranial meningioma ( 1), pituitary tumor ( 3), Grave's ( 3) and ophthalmic artery aneurysm were evaluated by OCT and visual field testing ( Humphrey SITA 24- 2). Patients were retested after decompression or radiation. The average retinal nerve fiber layer thickness was compared in eyes that improved in mean deviation after treatment with those that did not. Results: The average retinal nerve fiber layer of 11 eyes that improved was significantly ( P < 0.006) thicker ( mean = 120.9 urn) compared with 4 eyes that did not ( mean = 71.7 urn). A significant correlation was found between the average thickness of the retinal nerve fiber layer and the post-treatment mean deviation of the Humphrey visual field ( Pearson correlation coefficient = 0.658 p < 0.01). No significant correlation was found between the average retinal nerve fiber layer thickness and the pre- treatment mean deviation. Conclusions: Measurement of retinal nerve fiber layer thickness by Optical Coherence Tomography provides a new means of assessing the reversibility of visual loss and prognosis in patients with compressive optic neuropathy. Role of Cyclic AMP in Axon Regeneration in the Mammalian CNS First Author: Paul Hoffman, MD, PhD, Baltimore, Maryland Background: The lack of effective axon regeneration in the central nervous system ( CNS) of adult mammals reflects the presence of molecules associated with CNS myelin that are potential inhibitors of axon elongation. The actual ability of these molecules to block regeneration depends on the intrinsic growth state of neurons. The high intrinsic growth state of developing neurons allows axon regeneration in the CNS. This capacity is lost as the intrinsic growth state declines during postnatal development. Changes in the intrinsic growth state correlate with alterations in neuronal cyclic AMP ( cAMP) levels. Objectives: To determine whether dibutyryl cAMP ( db cAMP), a membrane- permeable cAMP analogue, can promote the regeneration of sensory axons in the dorsal columns of the spinal cord. Methods: Sensory neurons in adult rats were exposed to db cAMP in vivo by injecting this agent into the L5 dorsal root ganglion ( DRG). The central branches of axons arising from these neurons enter the spinal cord and ascend to the brain stem in the dorsal columns of the spinal cord. Seven days after db cAMP injection, the dorsal columns were cut in the thoracic spinal cord. Regenerating sensory axons were visualized in the spinal cord 2 weeks after injury using cholera toxin fragment B conjugated to horseradish peroxidase ( CTB- HRP), an anterograde tracer taken up by sensory neurons and transported intra- axonally to the tips of regenerating axons. Results: Exposing neurons to db c AMP enabled the central branches of sensory axons to regenerate in the dorsal columns of the spinal cord. No regeneration was observed from saline- injected control DRG. Conclusions: Db cAMP enabled mature sensory neurons to regenerate axons in the dorsal columns of the spinal cord. This finding provided the rationale for examining the ability of this agent to promote the regeneration of retinal ganglion cell axons in the optic nerve. Retinal Toxicity Secondary to Irofulven First Author: Michael Lee, MD, Boston, Massachusetts Background: MGI 114 ( Irofulven) is a novel antitumor agent derived from the mushroom product, illudin- S. Although the precise mechanism of action is unknown, irofulven undergoes rapid uptake in sensitive cells, binds to protein and DNA, produces single- strand DNA breaks, and causes cell cycle arrest in " S" phase resulting in cell death. It has been studied in numerous solid tumor Phase II and is currently in a Phase III clinical trial. Transient visual disturbances ( modification of color vision and contrast with normal acuity) were reported at the American Society of Clinical Oncology Annual Meeting, May 2001. Objectives: To describe ERG and visual function testing in 4 symptomatic and 4 asymptomatic patients on MGI 114. Methods: Irofulven is currently being studied in a multi-center clinical trial of women with metastatic ovarian cancer, who failed previous chemotherapy. A subset of 8 patients from one center underwent comprehensive neuro-ophthalmic evaluation and ERG testing. Results: Four patients had symptoms including photophobia ( 100%), reduced vision in bright light ( 100%), and positive visual phenomena ( 75%). Visual acuity was mildly affected. Paracentral scotomas and reduction of predominantly cone ERG responses were found in all symptomatic patients. Color plate testing was subnormal in 2 of 4. Paraneoplastic antibodies were tested in 2 of 4 and were negative. Improvement in symptoms, visual function testing, and ERG responses occurred with lower doses or cessation ^ 166 „.. , „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 of MGI114. Among 4 asymptomatic patients, 1 had unilateral cataract consistent with visual acuity, but had prolonged b- wave implicit times OU. The remaining patients had normal color vision, full visual fields, and ERG values. Conclusions: At higher doses, MGI 114 may cause cone toxicity in women with metastatic ovarian cancer, who have failed previous chemotherapy. Improvement has occurred with dosage reduction or discontinuation of the drug. Does Topical Brimonidine Tartrate Help NAION? First Author: Hilary Fazzone, MD, New York, New York Background: Topical brimonidine tartrate has been reported to have a neuroprotective benefit for retinal ganglion cells following experimental elevation of intraocular pressure and optic nerve injury in the rat. These results were the basis of the recently aborted clinical trial of brimonidine purite for acute non- arteritic anterior ischemic optic neuropathy in humans. Objectives: We hypothesized that topical brimonidine tartrate would provide neuroprotection for patients with acute NAION. Methods: We performed a retrospective review of patients with NAION who were evaluated within three weeks of the onset of visual loss and had follow up for at least 8 weeks. 14 of these patients ( treated) received brimonidine within 14 days ( mean 5.5) of the onset of visual loss. 5 patients were treated after one day of symptoms. The drops were taken QID in 11, TID in 1, and BID in 2 patients. There were 17 control patients ( untreated) who were matched to the treated group for age, gender, cardiovascular risk factors, prior aspirin use, and prior first eye NAION. The affected eye visual acuity was expressed as a decimal equivalent. The visual field defects were graded using a previously published scale ( 0 normal- 4 light perception). Results: The mean baseline acuity ( 0.56, sd 0.30) and visual field ( 1.9, sd 0.73) for the treated group was similar to the acuity ( 0.40, 0.41; p = 0.22) and field ( 1.9, sd 0.75; p = 0.96) for controls. At the 8 to 12 week examination, the mean visual acuity was 0.29 ( sd 0.30) for treated and 0.49 ( sd 0.39; p = 0.12) for untreated patients. The mean visual field grade was 2.2 ( sd0.81) for treated and 1.62 ( sd0.70; p = 0.04) for untreated patients. There was no correlation with a worse outcome and delay in initiating therapy and the average time to start the drops was 3.5 days in those who worsened. Conclusions: Topical brimonidine tartrate, given for NAION after one day, does not appear to offer neuroprotection for this disorder. Localization of the Pain with Optic Neuritis First Author: Mark Kupersmith, New York, New York Background: The pain with eye movement ( EOM) in optic neuritis is thought to be due to the movement of the affected intraorbital optic nerve, but this has not been verified. The lack of pain on eye movement in this disorder has also not been explained. Objectives: To demonstrate whether the MRI localization of the lesion can be related to the pain associated with optic neuritis or the pattern of visual field loss. Methods: The presence of eye or VI distribution pain and pain with EOM ipsilateral to the acutely affected optic nerve was recorded in 96 patients ( 73 women, 23 men, mean age 36 years). The location and length of abnormal optic nerve enhancement on MRI were documented. The presenting visual field defects were characterized as diffuse, central, arcuate, nasal or temporal. As recorded in the ONTT, the visual field sensitivity loss was graded according to the mean deviation ( minimal if > - 3dB and < - 6dB, moderate if > - 6dB and < - 20dB, severe if > - 20dB). Results: 71 patients experienced eye pain and 67 patients had pain with EOM. 20 patients had no pain. Enhancement of the orbital optic nerve occurred in 68 patients, 91% who hadVl pain ( OR 2.84; 95% CI 1.65^ 1.88) and 88% who had pain with EOM ( OR 3.53; 95% CI 1.85- 6.74). Enhancement of the canalicular, intracranial or both segments was seen, without orbital involvement, in 23 nerves, and 5 nerves had no enhancement. In these 28 patients, 32% had VI pain ( OR .35, 95% CI .21- 61) and 25% had pain with EOM ( OR .28, 95% CI .15-. 54). In the patients with enhancement limited to the canalicular ( 8) and intracranial ( 8) optic nerve, 2 and 1 had pain with EOM, respectively. Orbital optic nerve enhancement alone produced central ( 25%), diffuse ( 33%), arcuate ( 28%), nasal ( 3%) and temporal ( 0%) field defects. Canalicular nerve enhancement alone produced central ( 50%), diffuse ( 12%), arcuate ( 25%), nasal ( 0%) and temporal ( 0%) field defects. Intracranial optic nerve enhancement alone produced central ( 25%), diffuse ( 12%), arcuate ( 25%), nasal ( 0%) and temporal ( 25%) visual field defects. Nerves with longer segments of enhancement had more eyes with severe field loss ( 55% vs 32%, p = 0.008). Conclusions: The majority of patients with optic neuritis with eye or VI distribution pain or pain with EOM have involvement of the orbital segment of the optic nerve. The absence of pain, particularly with EOM, suggests the disorder is limited to the canalicular or intracranial portions of the optic nerve. Except for temporal field loss in eyes with Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 NANOS ABSTRACTS intracranial nerve lesions, no pattern of field loss appears related to the location or length of abnormal enhancement. Dibutyryl Cyclic AMP Promotes Optic Nerve Regeneration First Author: Nicholas Monsul, MD, Pittsburgh, Pennsylvania Background: Injured axons do not regenerate in the adult central nervous system ( CNS). This reflects the low intrinsic growth state of mature CNS neurons. Presumably, the CNS environment prevents the intrinsic growth state of mature neurons from increasing after injury, thus severely limiting axon regeneration. Intracellular cAMP levels appear to regulate the intrinsic growth state. Exposure to db cAMP in vivo increases the intrinsic growth state of mature neurons thereby promoting regeneration in the presence of CNS molecules that are potential inhibitors of axonal elongation. Objectives: To determine if an intraocular injection of dibutyryl cyclic AMP ( db cAMP) 24 hours before or 24 hours after axotomy can promote axon regeneration in rat optic nerve. Methods: Intraocular injections of db cAMP were given 24 hours before ( Group A) and 24 hours after ( Group B) optic nerve axotomy with a 9- 0 nylon. The animals were allowed to survive for 2, 4, 6 and 8 weeks. One day before sacrifice an anterograde intra- axonal tracer, cholera toxin subunit- B conjugated with horseradish peroxidase ( CTB- HRP) was injected into the eye to visualize regenerating axons. Sectioned optic nerves were mounted onto slides for evaluation and photography. Regenerating axons were counted at distances of 0.25, 0.5 and 1.0mm distal to the axotomy site. Results: Intraocular injections of db c AMP 24 hours before or 24 hours after optic nerve axotomy results in axon regeneration past the axotomy site. Control animals injected with NaCl showed no fibers regenerating past the axotomy site. Regenerating fibers have frequent trifurcations, a circuitous pattern and the characteristic growth cones that were not seen in eight control animals. Conclusions: Intraocular injections in vivo of db c AMP before or after axotomy promotes axon regeneration in the optic nerve. Eight Index Cases from Field Investigation in Brazil of a Giant Pedigree Of Leber's Hereditary Optic Neuropathy First Author: Peter Quiros, MD, Los Angeles, California Background: A mother from rural Brazil had a 14- year- old son go blind in one eye. Her brothers had also gone blind as young adults. Molecular analysis of blood samples showed that all 4 individuals had LHON, 11778, homoplasmic, J-haplotype. Four first- degree relatives from this family living in Massachusetts are also shown to have the same mutation. Objectives: To present preliminary data based on eight index cases from members of a previously unstudied pedigree known to have Leber's hereditary optic neuropathy ( LHON). Epidemiological, neuro- ophthalmological, psychophysical and blood examinations are summarized and subjected to multi- variant analysis on epigenetic factors and gene linkage analysis. Methods: Investigators from the University of Sao Paulo conducted thorough examinations of the 14 year old proband and three of his affected relatives. An extensive questionnaire, previously used on a large LHON pedigree, was further adapted for local conditions and translated into Portuguese. These examinations consisted of the following parts: 1) Epidemiological interview. 2) Blood drawing for molecular analysis, biochemical studies, and gene linkage analysis. 3) Comprehensive neuro- ophthalmological examination. 4) Psychophysical studies including contrast sensitivity, color vision, and threshold Amsler grid testing. 5) Humphrey visual field 30- 2 perimetry. 6) Fundus photography. Furthermore, the 4 family members living in Massachusetts were similarly examined by US investigators. Results: A total of 8 index cases are presented from this large pedigree. 4 patients were examined in Brazil and 4 others in the U. S. Epidemiological interviews revealed 7 of 8 patients to be heavy smokers and/ or drinkers. Nutrition met minimum daily requirements as established by the FDA. Molecular analysis reveals 11778, homoplasmic, J-haplotype in all patients. Biochemical studies are normal. Neuro- ophthalmic and psychophysical testing revealed severe impairments, mean Va CF 3 ft, profound color vision loss, diffuse optic atrophy. Age at onset ranged from 12- 23. Involvement of the second eye ranged from 1 week to 18 weeks. Conclusions: 8 cases are presented providing preliminary data from a large pedigree in which 277 members were comprehensively studied. Analysis of environmental risk factors reveal that exposure to toxins such as cyanide from cigarette smoke or folate depletion from ETOH consumption may play a major role in expression of the disease. These preliminary cases provide insight into the largest group of LHON patients ever studied and will help to elucidate genetic and epigenetic factors of this disease. The Eyes of Mito- mice First Author: Valerie Biousse, Atlanta, Georgia n 168 „.. „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 Background: The recent creation of several mouse models of mt diseases has provided new insights into the understanding of human mt disorders. Whether these animals have clinical or histologic ophthalmologic abnormalities is of great interest given the high frequency of such abnormalities in humans with mt disorders. Objective: To describe the currently available mouse models for mitochondrial ( mt) diseases and to investigate their ocular manifestations. Methods: We are currently evaluating the lenses, retinas, optic nerves and extraocular muscles of 3 mouse models for mt diseases. Slit lamp biomicroscopy, electrophysiology ( ERG and VEP) and histology ( light microscopy and EM) are performed on the mouse models and on age- matched controls. Results: 1) MnSOD- deficient mice ( SOD2tmlCje) represent a model of increased mt production of reactive oxygen species ( ROS). SOD2 mice die early in life from dilated cardiomyopathy. When treated with MnTBAP, mice survive to day 16- 20 and have severe neurologic disease. ERGs obtained on SOD2 treated mice at age 19 days demonstrated normal retinal function, however histopathologic studies of the retina showed retinal thinning. Abnormal mitochondria were found in the RPE and in the extraocular muscles. 2) ANT1- deficient mice are models for chronic ATP deficiency. Mice develop classic mitochondrial myopathy and dilated cardiomyopathy. Similar to age- matched controls, ANT mice older than 12 months have nuclear and posterior cataracts. ERG resting revealed supranormal responses in ANT1 mutant mice, which became larger with age. 3) The CAPR chimeric mice have growth retardation and mt myopathy and cardiomyopathy. These mice have a variety of ocular abnormalities including congenital cataracts, decreased ERG amplitudes, and hamartomas of the optic nerves. Conclusions: These mouse models demonstrate multiple and varied ophthalmologic manifestations. Further ( clinical, electrophysiologic) and histopathologic studies are ongoing to better delineate their neuro- ophthalmologic phenotype. An appropriate mouse model for Leber's hereditary optic neuropathy is also currently under development. Such mouse models should facilitate the understanding of the pathogenesis of human mt disorders and could provide a way of screening therapeutic agents potentially efficacious in human mt diseases. A Paraneoplastic Syndrome of Combined Optic Neuritis and Retinitis Defined Serologically by CRMP- 5- IgG First Author: Shelley Cross, MD, Rochester, Minnesota Background: Autoimmune serology has identified two paraneoplastic visual disorders related to small- cell lung carcinoma: retinopathy with 23 kDa recoverin (" CAR")- IgG, and optic neuritis/ vitreitis with 62 kDa CRMP- 5- IgG. Objective: To define a novel paraneoplastic ophthalmic disorder and describe neurologic accompaniments. Methods: CRMP- 5- IgG was identified in 172 patients presenting with subacute neurologic disorders. Fifteen patients had optic neuritis, with retinitis documented in 5. Histories and results of ophthalmological and neurologic examinations, fundus photographs, fluorescein angiograms, and ERGs were reviewed. Bovine retina and optic nerve antigens were analyzed using monoclonal IgGs specific for C-terminal and N- terminal residues of CRMP- 5. Results: All 15 patients ( ages 52- 74) were smokers; 8 were female. Fourteen had subacute visual loss with swollen optic discs and field defects. Vascular leakage was evident at and remote from the disc; 4/ 4 tested had abnormal ERGs. Vitreal cells were striking in 9. Two patients with myelopathy and optic neuritis bore a superficial resemblance to Devic's; one lacked visual complaints, but VER revealed prolonged optic nerve conduction. Other neurologic accompaniments included mental status changes, cranial neuropathies, movement disorders, myelopathy, peripheral nerve disorders, cerebellar and autonomic dysfunction. Small- cell lung carcinoma was confirmed in 10 patients ( 9 confined to chest); the remaining 5 had another carcinoma or provisional evidence for lung cancer. CRMP- 5- IgG was detected at 1: 1,000 to 1: 500,000 dilution. No serum had CAR- IgG. CSF in 10/ 15 was pleo-cytotic ( 7- 32 cells) with elevated protein and CRMP- 5- IgG. Vitrectomy revealed reactive lymphocytosis ( 4/ 4), predominantly CD4+ ( 1/ 1). Western blots demonstrated full- length CRMP- 5 protein in optic nerve and retina. Peroxidase staining revealed cytoplasmic immunoreactivity in retinal ganglion cells, nerve fiber layer and photoreceptor cells. Conclusions: CRMP- 5- IgG defines a novel paraneoplastic ophthalmic entity of combined optic neuritis and retinitis accompanied by vitreal inflammation. Its serological confirmation obviates need for vitreous biopsy and mandates a search for small- cell carcinoma. Pituitary Apoplexy Revisited First Author: Klara Landau, MD, Zurich, Switzerland Background: Patients with pituitary apoplexy typically present with severe headache followed by visual loss and Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 NANOS ABSTRACTS oculomotor nerve pareses, as well as altered level of consciousness and endocrine dysfunction. This potentially devastating clinical course is precipitated by a hemorrhage or infarction into a pre- existing pituitary tumor. The factors that predispose some pituitary adenomas to bleed are not entirely understood, mainly due to the rare occurrence of this syndrome. Objective: To elucidate the incidence, clinical presentation and precipitating factors for pituitary apoplexy in a large group of patients with pituitary adenomas. Methods: We reviewed the charts of 378 patients seen at the Division of Endocrinology and Diabetes, University Hospital of Zurich, between 1958 and 2000 with the diagnosis of a pituitary adenoma. Patients with pituitary apoplexy were identified and compared with the rest. In addition, for each patient with pituitary apoplexy two control patients of same age, gender, tumor size, and tumor type were matched for more detailed analysis. Results: The incidence of classic pituitary apoplexy was 8.5% ( n = 32). Sufficient data were available on 26 patients, all of whom had macroadenomas, of which most were non-secreting. In contrast, only 62% of patients in the comparison group had macroadenomas. Despite a clear female predominance in the whole population, men presented with pituitary apoplexy twice as frequently than women. Comparison of the 26 pituitary apoplexy patients with the 52 matched control patients revealed the following predisposing factors for the development of pituitary apoplexy: estrogen treatment, anticoagulation, diabetes mellitus and hypertension. Neuro- ophthalmological signs occurred more frequently in the apoplexy group whereas endocrine dysfunction was predominant in the control group. Conclusions: In our large retrospective series both greater tumor size and male sex were confirmed as risk factors for the development of pituitary apoplexy. Early diagnosis of endocrine dysfunction in men who may harbor a pituitary adenoma could efficiently prevent the life- threatening syndrome of pituitary apoplexy. Foveal Sparing- an Artefact? First Author: Susanne Trauzettel- Klosinski, MD, Tuebin-gen, Germany Background: We have shown in a former study ( 1) that macular sparing really exists and is a cortical phenomenon. However, studies on foveal sparing are still contradictory. Morphological investigations in the primate retina ( 2) showed a nasotemporal overlap of retinal ganglion cells or 0.5° to each side of the vertical meridian, widening to 1.5° in the fovea, which was interpreted as foveal sparing. This result was not in agreement with the common clinical finding of macular splitting. Objective: The purpose of this study was to examine if foveal sparing can be found in humans. Methods: The vertical field border was determined in 20 patients with hemianopia ( 10 right, 10 left) by means of a Scanning Laser Ophthalmoscope. Vertical triplets of dots were scanned directly onto the retina under simultaneous and continuous fixation control with a spatial resolution of 0.5° horizontally and 1° vertically. Patients with macular sparing (> 2°) were excluded. Results: All patients showed a vertical strip of overlap towards the hemianopic side of approximately 0.5°. However, in the 0° meridian none of them had an additional sparing, most of them ( n = 14) even had absence of any overlap. Conclusions: From our results, we conclude that foveal sparing is a morphologic phenomenon caused by the anatomic distribution of retinal ganglion cells. From a functional view, foveal sparing does not exist, because the receptive fields of the parafoveal ganglion cells project directly into the center of the fovea. This explains the clinical finding of macular splitting. References: 1. Trauzettel- Klosinski S, Reinhard J. The vertical field border in hemianopia and its significance for fixation and reading. Invest Ophthalmol Vis Sci 1998; 39: 2177- 86. 2. Bunt AH, Minckler DS, Johanson GW. Demonstration of bilateral projection of the central retina of the monkey with horseradish peroxidase neuronography. J CompNeurol 1977; 171: 619- 30. Relative Sparing of Afferent Pupillary Pathways in LHON: A Postmortem Study First Author: Swaraj Bose, MD, Irvine, California Objective: To demonstrate if afferent pupillary fibers are spared in Leber's Hereditary Optic Neuropathy ( LHON). It has been previously suggested that pupil light reaction is relatively preserved in affected eyes of patients with LHON, giving rise to visual- pupillomotor dissociation. Methods: Dil, a fluorescent dye that allows anterograde labeling of axons, was injected into the brachium of the superior colliculus in a post- mortem brain from a patient with 3460 mutation LHON and a normal control brain. After 4 weeks, serial fragmatome sections were obtained till the pretectal area, further stained with propidium iodide ( PI, stains DNA) to delineate pretectal nuclei associated with fibers traced with Dil and examined under a confocal microscope. Light microscopy and digital morphometry on the optic nerves were performed after staining with PPD ( myelin) and trichrome stain. ^ 170 „.. „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 Results: Dil labeled fibers were visible on all sections from the superior colliculus to the pretectum in the LHON and control brain along with nuclei in the cell bodies stained with PI. There was a massive loss of small diameter fibers in the LHON optic nerve. Conclusions: The fact that there is considerable staining of the pupillary fibers in LHON lends support to the clinical observation of relatively preserved pupil function in LHON. POSTER PRESENTATIONS Retrolaminar Optic Nerve Enhancement on Orbital MRI in Leber's Hereditary Optic Neuropathy: Clinical and Pathophysiologic Implications First Author: Michael Vaphiades, DO, Little Rock, Arkansas Background: LHON is a maternally inherited optic neuropathy associated with point mutations in the mitochondrial DNA. It is now recognized that retrobulbar optic nerve enhancement with gadolinium administration may occasionally occur in the acute phase of LHON ( 1). Objective: To determine whether Leber's hereditary optic neuropathy ( LHON) is associated with acute injury to the retrolaminar optic nerve. Methods: Orbital fat suppressed gadolinium enhanced magnetic resonance imaging ( MRI) scans on 5 consecutive patients with LHON were evaluated retrospectively from 1996 to present. Results: Optic nerve enhancement was noted in 2 of 5 patients overall, and 2 of 3 patients who were imaged during the acute phase of visual loss. The enhancement persisted for at least 6 months in one patient and resolved after 4 years in the other patient. Conclusions: LHON may be associated with protracted enhancement of the retrolaminar optic nerve. This enhancement may reflect a protracted metabolic disturbance of the optic nerve consequent to mitochondrial dysfunction. References: 1. Vaphiades MS, Newman NJ. Optic nerve enhancement on orbital magnetic resonance imaging in Leber's hereditary optic neuropathy. J Neuroophthalmol 1999; 19: 238- 9. 2. Riordan- Eva P, Sanders MD, Govan GG, et al. The clinical features of Leber's heredity optic neuropathy defined by the presence of a pathogenic mitochondrial DNA mutation. Brain 1995; 118: 319- 37. 3. Guy J, Mancuso A, Quisling RG, et al. Gadolinium- DPTA-enhanced magnetic resonance imaging in optic neuropathies. Ophthalmology 1990; 97: 592- 600. Optic Nerve Head Drusen and Ischemic Optic Neuropathy First Author: Andrew Lee, MD, Iowa City, Iowa Background: Ischemic optic neuropathy is a well recognized but rare association with optic nerve head drusen. Methods: Retrospective case series of all cases of ischemic optic neuropathy due associated with optic nerve drusen from two academic tertiary care centers. Results: 15 cases of ischemic optic neuropathy in patients with optic nerve head drusen are reported. Younger age and lack of typical associated vasculopathic risk factors were present in these cases when compared with typical non-arteritic ischemic optic neuropathy. Conclusion: Optic nerve drusen may be a risk factor for the development of ischemic optic neuropathy. Ophthalmoscopic or ultrasound detection of disc drusen in a patient with ischemic optic neuropathy may obviate the need for more extensive evaluation for ischemic optic neuropathy in a younger patient or one without vasculopathic risk factors. Optic Neuritis in Anti- GQlb Positive Miller Fisher Syndrome First Author: Jane Chan, MD, Las Vegas, Nevada Abstract: Only three cases of optic nerve involvement in " presumed" Miller Fisher syndrome ( MFS) have been documented in the literature. This report further confirms that optic neuritis may be seen in anti- GQlb positive MFS. This 23- year old woman presented with acute blurry vision, diplopia, and pain with eye movement. Her visual acuity was 20/ 20 OD and 20/ 200 OS with a left APD. Visual fields were normal. She had bilateral sixth nerve palsies, nystagmus in all gazes, and left optic disc edema. After one week her visual acuity improved to 20/ 20 OU, but her left disc remained edematous. She then developed rapidly progressive gait ataxia and absent deep tendon reflexes in her lower extremities. Her MRI of the brain and entire spine and MR venogram were all normal. Her CSF opening pressure was 150 mm H20 and her CSF protein was elevated at 70 mg/ dL. Her VEP revealed a delayed left PI00 latency and her BAEP was normal. EMG/ NCV study revealed prolonged F- waves. Anti- GQlb antibodies were positive at high titers. Antibodies for acetylcholine receptor, hepatitis A, B, and C; Lyme, Hu, MaTa, Yo, CV- 2, and Ri were all negative. After plasmapheresis she fully recovered two months later. In addition to the classic triad of ophthalmoplegia, ataxia, and areflexia, visual impairment presenting as optic neuritis may be a feature of anti- GQlb positive MFS. Optic disc edema associated with an ipsilateral Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 NANOS ABSTRACTS delayed PI00 latency confirms a pre- chiasmal optic nerve dysfunction in this patient. Anti- GQlb IgG antibodies are present in 80% to 100% of patients with MFS. Since high concentrations of GQlb gangliosides are known to be present in the human optic nerve, optic neuritis is probably a result of anti- GQlb IgM complement- mediated inflammatory demyelination. The additional electrophysiological evidence of abnormal prechiasmal dysfunction in this case confirms that an autoimmune optic neuropathy may occur in MFS patients. Optic Neuritis with Extensive Distension of the Optic Nerve Sheath First Author: H. E. Killer, MD, Basel, Switzerland Background: Cerebrospinal fluid is postulated to communicate freely between all interconnected cerebrospinal fluid compartments. The subarachnoid space of the optic nerve resembles a cull de sac anatomy and is part of the cerebrospinal fluid system. Objective: To report a case of unilateral optic neuritis with extensive distension of the optic nerve sheath due to fluid accumulation in the subarachnoid space. Methods: Interventional case report. Results: A 38- year- old male patient was admitted because of visual loss in the OD and pain on eye movements. On examination the best visual acuity in the OD measured 20/ 50 and 20/ 20 in the OS. 16 out of 18 Ishihara plates were identified with the OD. The papilla in the OD was prominent while the fundus examination of the OS was normal. All laboratory examinations were normal. An MRI of the brain showed no signs of demyelination but massive distension to the right optic nerve sheath with pooling of fluid in the subarachnoid space. The diagnosis of optic neuritis was made and the patient was treated with methylprednisolone. Visual functions in the OD improved over two weeks. An MRI of the orbit was repeated two month later that demonstrated normal diameters of both optic nerve sheaths. Conclusions: Optic neuritis can lead to an increase of fluid in the subarachnoid space with a consecutive rise of the local pressure. If there is a lack of communication between the subarachnoid space of the involved optic nerve and the chiasmal cistern, an optic nerve sheath compartment syndrome may result. Retinal Vasospasm During an Attack of Migraine First Author: H. E. Killer, MD, Basel, Switzerland Background: Vasospasm plays a major role in the pathogenesis of a number of diseases. Vasospasm can involve blood vessels of different organs, especially the coronaries and the finger arteries. In the brain short spasms occur mostly in the context of migraine. The blood circulation of the eye is often involved the so- called primary vasospastic syndrome and in migraine. The choroid and the vessels of the optic nerve head seem to be affected more often than the retinal vessels. Methods: Case report of a patient that suffered from a unilateral visual field defect in the OS during a episode of migraine. Fundus examination of the OS demonstrated disruption of blood flow in the lower temporal artery due to vasospasm. Conclusions: Homonymous scotomas due to occipital hypoperfusion are more common than unilateral scotomas due to retinal migraine. Retinal migraine, however, is of special interest, as the postulated hypoperfusion and the vasoconstriction can directly be observed on fundus examination. The Architecture of the Arachnoid Trabeculae and Sep-tae in the Subarachnoid Space of the Human Optic Nerve: Anatomy and Clinical Considerations First Author: H. E. Killer, MD, Basel, Switzerland Objectives: To describe the anatomy and the arrangement of the arachnoid trabeculae and septae in the subarachnoid space ( SAS) of the human optic nerve and to consider their possible clinical significance for cerebrospinal fluid dynamics and cerebrospinal fluid pressure in the SAS of the optic nerve. Methods: Postmortem study carried out in 11 subjects without ocular disease. All optic nerves used in this study were obtained no later than 7 hours after death. The study was performed with light microscopy, transmission electron microscopy, and scanning electron microscopy. Results: The subarachnoid space of the human optic nerve contains a variety of trabeculae and septae. There is a great variability concerning density and anatomic structure depending on the location within the different portions of optic nerve. In the bulbar segment ( ampulla) adjacent to the ocular globe, a dens and ramified meshwork of delicate trabeculae is arranged in a net like fashion. In the midorbital portion, the subarachnoid space is subdivided and at some locations appears even loosely chambered by broad trabeculae and velum like septae. A similar type of trabeculae as observed in the bulbar segment can be found in the inter-canalicular portion of the subarachnoid space. Conclusions: The subarachnoid space of the human optic nerve contains a complex system of arachnoid trabeculae and arachnoid septae that divide the subarachnoid space into a multichambered cerebrospinal fluid compartment. ^ 172 . , _ „ , . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 Due to the architecture of the trabeculae and septae cerebrospinal fluid dynamics are influenced by their presence. The trabeculae and septae described in this study are of delicate character such and can not be visualized even with high resolution magnetic resonance imaging ( MRI). The results of this post mortem study may contribute to the understanding of the pathophysiology of asymmetric papilledema, unilateral papilledema and probably normal tension glaucoma. Treatment of Acute Visual Loss in Idiopathic Intracranial Hypertension First Author: Mitchell Strominger, MD, Brooklyn, New York Background: Idiopathic Intracranial Hypertension ( IIH) is a condition of unknown etiology leading to elevated cerebrospinal fluid pressure with papilledema in the absence of hydrocephalus. If the disc swelling is severe, patients can present with significant reduction in visual acuity. The treatment of patients with acute visual loss includes oral carbonic anhydrase inhibitors, repeated lumber puncture, lumbar peritoneal shunts, oral or intravenous corticosteroids, optic nerve sheath fenestration or a combination thereof. In 1994 Liu, Glaser, and Schatz recommended a regimen of methylprednisolone ( 250mg IV QID x 5 days) in combination with acetazolamide ( 500 mg BID x 5 days) and ranitidine ( 150 mg BID x 5 days), followed by prednisone ( 80 mg per day) tapered over six weeks for the treatment of acute, severe visual loss. Objective: This study was to determine whether the members of NANOS followed the above recommendations. Methods: We surveyed 242 members of NANOS and asked whether they followed the recommend treatment regimen. We defined severe, acute visual loss as visual acuity worse than 20/ 30 or visual field defect of grade 3 or worse. If not, we asked them to specify their preferred treatment. Results: 44 ( 18%) returned the survey by fax. 30 ophthalmologists, 6 neurologists, 5 with training in both, and 3 did not indicate. 11 ( 25%) used the recommended treatment regimen. Of the remaining 33 ( 75%) respondents, many modifications were noted. 10 used higher doses of acetazolamide including 3 who recommended intravenous. Two tapered the prednisone more rapidly. 18 ( 55%) included optic nerve sheath fenestration or lumbar peritoneal shunting. Conclusions: There is no consensus as to the treatment of acute visual loss in IIH. Most physicians used some modification of the recommended regimen with the addition of ONSF or LP Shunting. Further studies are needed to determine the best treatment of this disorder. The Evaluation of Isolated Third Nerve Palsy Revisited: an Update on the Evolving Role of Magnetic Resonance, Computed Tomography, and Catheter Angiography First Author: Andrew Lee, MD, Iowa City, Iowa Abstract: The evaluation and management of the neuro-logically isolated third nerve palsy continues to evolve. The major concern for the clinician confronted with a patient with a third nerve palsy has been the exclusion of an intracranial aneurysm. The evolution of new imaging techniques such as computed tomography angiography ( CTA) and magnetic resonance angiography ( MRA) have provided new imaging options for clinicians. This paper reviews the pertinent recent literature on the use of these imaging studies in evaluating the patient with a third nerve palsy. Posterior Ischemic Optic Neuropathy Complicating Laparoscopic Prostatectomy First Author: Clifton Otto, MD, Tacoma, Washington Background: Posterior ischemic optic neuropathy ( PION) is a rare condition that typically occurs intra- operatively from sustained hypotension, anemia, or direct pressure to the globe, resulting in ischemic infarction of the retrobulbar portion of the optic nerve. PION can also be caused by elevated episcleral- venous pressure, as demonstrated by this first case following laparoscopic prostatectomy. Objective: To present a case of posterior ischemic optic neuropathy ( PION) secondary to laparoscopic prostatectomy. Methods: Case report with discussion of findings. Results: This 65- year- old male presented with acute onset of loss of vision in the temporal field of the OD after regaining consciousness following a fifteen- hour laparoscopic prostatectomy. The patient received 15 L of crystalloid during this procedure to maintain urine output. Marked swelling of the patient's eyelids, face and upper extremities was noted by anesthesia halfway through the procedure, but there was no prolonged hypotension, significant anemia, or evidence of direct pressure over the eyes reported during surgery. Formal visual fields revealed a temporal scotoma in the OD consistent with the patient's subjective loss of vision, a finding that was not present on previous visual fields obtained for management of glaucoma in the other eye. No optic nerve swelling or retinal hemorrhage was present. Visual fields obtained over the following eight months showed mild progressive improvement in the temporal field of the OD, corresponding with the patient's subjective observation of gradual expansion of his temporal Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 NANOS ABSTRACTS field of vision. Carotid artery duplex and MRI of the brain and orbits were both normal. Conclusions: Given the lack of intraoperative hypotension, anemia or direct pressure to the globe in this case, sustained elevation of episcleral venous pressure during surgery is the most plausible cause of this patient's optic neuropathy. Intraoperative findings suggest that measures should be taken to prevent excessive periorbital swelling during prolonged surgery. The Obvious Is Not So Obvious First Author: Adam Cohen, MD, Burlington, Vermont Objectives: To describe an unusual presentation of giant cell arteritis; to discuss a plethora of teaching points, and review the medical literature on orbitopathy secondary to giant cell arteritis. Methods: Case Report. Results: A 67- year- old man presented 12/ 99 with decreased vision in both eyes, particularly over the prior month. His medical history included biopsy- proven giant cell arteritis ( diagnosed 9/ 99), Type 2 diabetes mellitus, atrial fibrillation necessitating amiodarone ( started late 11/ 99), and alcoholism. Due to concern about the impact of systemic corticosteroids on his diabetes, and avoidance of methotrexate given his long history of alcoholism and probable liver damage, he was started on pulse cyclophosphamide therapy to facilitate the corticosteroid taper. He was admitted to the hospital 12/ 99 with a 2- week history of progressive right upper and lower extremity edema, generalized weakness, and bilateral visual loss. Bedside neuro-ophthalmologic examination revealed visual acuities of 20/ 200 OU with bilateral proptosis and " tight orbits", lid edema, conjunctival chemosis, mildly elevated optic discs with sectoral right optic disc pallor, and moderate background diabetic retinopathy with minimal macular involvement in both eyes. Computed tomography was remarkable for bilateral proptosis, with normal extraocular muscles, orbital fat, and paranasal sinuses. His Westergren erythrocyte sedimentation rate was 67; having been 22 one- week prior, and C- reactive protein was 4.7. Thyroid function tests were normal. Concurrent with discontinuation of the amiodarone, intravenous methylprednisolone one gram daily was initiated. After further visual decline, bilateral transconjunctival orbital decompression surgery was performed, with bilateral orbital fat biopsies, stains, and cultures, which were unremarkable. Despite decreased proptosis and " softer orbits", vision failed to recover in either eye. Conclusions: Giant cell arteritis may present in a variety of ways, including bilateral orbital ischemia resembling inflammatory orbital pseudotumor or dysthyroid orbitopathy. Ocular Bartonellosis Mimicking Ocular Metastases First Author: Aki Kawasaki, MD, Lausanne, Switzerland Background: Bartonella henselae is the causative agent of cat- scratch disease. In 5- 10% of cases, the eye is involved. Two well- known, ocular complications are conjunctivitis and neuroretinitis. Other posterior segment manifestations are increasingly recognized including inflammatory chorioretinal lesions ( white spots), serous retinal detachment, vaso- occlusive disease and optic disc granulomas. Objective: To report a patient who had multiple mass lesions in one eye ( optic disc, macula, peripheral retina) and positive serologies for acute Bartonella henselae infection. Methods: Case report. Results: A 12- year- old boy developed headache and visual loss in his OS to 20/ 400 acuity. Funduscopy showed marked optic disc swelling with peripapillary subretinal elevation. A large creamy- colored mass was present in the macula as well as smaller, discoid- shaped elevated lesions in the periphery. An inferior serous retinal detachment was also present. There was no evidence of ocular inflammation. Ultrasound confirmed the presence of a solid mass at the optic disc and at the macula. Workup for possible metastatic disease was negative. Bartonella serologies were initially negative then showed greater than fourfold rise in titers one month later. The patient was treated with doxy-cycline; the ocular masses disappeared within 4 weeks and vision recovered to 20/ 70. Conclusions: We report the first case of a macular mass presumably due to Bartonella inflammation. The presence of multiple mass- like lesions in the absence of systemic or ocular inflammation was suggestive of ocular metastases. We alert the clinician to yet another clinical presentation of cat- scratch disease. Orbital Infarction after Intranasal Cocaine First Author: Gregory Van Stavern, MD, Detroit, Michigan Background: Orbital infarction syndrome is uncommon, and results from ischemia of all intraocular and orbital structures. It has been reported in a variety of settings. Cocaine has potent sympathomimetic and vasoactive properties, and has been associated with retinal vascular occlusions. We describe a case of orbital infarction syndrome associated with intranasal cocaine use. _ 174 . „ _ . . „ . . © 2002 Lippincott Williams & Wilkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 Objective: To describe an uncommon syndrome, as well as a previously unreported complication of intranasal cocaine use. Results: A 36- year- old woman presented with sudden, painful visual loss O. S., and complete left ophthalmoplegia. The patient has attended a party the previous evening, and consumed alcohol, and intranasal cocaine. She lost consciousness with her left face pressed against a desktop. She awoke with complete blindness in the OS , left orbital pain, left ptosis, left proptosis, and complete left ophthalmoplegia. An urgent MRI, including fat- suppressed orbital views, showed diffuse edema of all left extraocular muscles, but no other abnormalities. MRA/ MRV were normal. A cerebral angiogram, performed 48 hours after the patient had awoken, was normal, with questionable delayed choroidal filling. Bedside examination showed VA of 20/ 20 OD, NLP OS. She had complete left ptosis, mild periorbital edema, and complete left ophthalmoplegia. The left fundus showed diffuse retinal edema. The remainder of her neuro-ophthalmic examination was unremarkable. An extensive inflammatory and hypercoagulable evaluation was unre-vealing. She was seen one week later in clinic. The orbital pain was improved. Examination was similar, except for modest improvement in left ductions and left ptosis. The retinal edema had resolved. Conclusions: Orbital infarction has been described in patients with prolonged head compression, as well as a variety of inflammatory and hypercoagulable disorders. The combination of left orbital compression and the vasoactive properties of cocaine resulted in orbital infarction syndrome in this patient. This potential complication of intranasal cocaine has not previously been reported. Unusual Visual Loss in Papilledema Associated with Parapapillary Neovascular Membrane First Author: Francois Borruat, MD, Lausanne, Switzerland Background: The incidence of visual loss amongst patients with idiopathic intracranial hypertension ( IIH) ranges from 10% to 26%. Causes of visual loss include optic atrophy, anterior ischemic optic neuropathy, arterial or venous retinal occlusion, maculopathy, and subretinal neovascular membrane ( NVM). Objective: To report an unusual case of visual loss secondary to NVM in a patient with otherwise asymptomatic IIH. Methods: Case report. Results: A previously healthy 39- year- old man presented with left visual loss for 2 weeks. Visual acuity was 20/ 20 RE and 20/ 30 LE. Visual field revealed enlarged blind spot and cecocentral scotoma in the OS . Vitreous cells were present in the OS with a slight inflammation in the left anterior chamber. Fundus examination revealed bilateral swollen optic discs with bilateral temporal superior parapapillary subretinal nodules ( 1.9 and 2.3 mm on B- scan echography) and left partial macular star. Results of lumbar puncture and blood serologies for inflammatory and infectious disorders were twice negative. Vision progressively worsened to 20/ 100 due to the progression of the left macular star. Bilateral parapapillary NVM were suspected. Intraventricular intracranial pressure was continuously monitored revealing nocturnal sustained peaks of hypertension ( max. 40cmH2O during 15- 20 minutes, 10- 12x/ night). Lumbo- peritoneal shunt was performed. Evolution was then favorable with improvement of left visual acuity to 20/ 30 and spontaneous involution of NVMs. Conclusions: Parapapillary NVM is a rare event complicating papilledema, as only 10 cases have been thus far reported in the literature. It is believed to be associated with long- standing papilledema. Indeed half of the reported cases ( including the present case) are asymptomatic until visual loss. Successful treatment of IIH is frequently followed by spontaneous involution of the NVM. However, 4/ 7 cases failed to recover visual acuity. Early recognition and treatment of IIH might improve visual prognosis of patients with associated NVM. Neuro- Ophthalmic Manifestations of Patients with Unruptured Intracranial Aneurysms First Author: M. Tariq Bhatti, MD, Gainesville, Florida Objective: To determine the frequency of neuro- ophthal-mic findings in patients with unruptured intracranial aneurysms and to correlate patient characteristics and outcome with these findings. Methods: A retrospective chart review of 186 consecutive patients with unruptured intracranial aneurysms operated at the University of Florida by a single surgeon ( ALD) between 1989 and 1994. Analysis was performed on the clinical characteristics of patients with only preoperative or postoperative ophthalmic findings. Preoperative presenting ophthalmic findings were analyzed to determine their resolution, improvement, stability, or worsening at final follow-up. Ophthalmic outcomes were correlated with patient age, duration of symptoms prior to surgery as well as size of the aneurysm( s). Results: Sixty- three ( 34%) of 186 patients with unruptured intracranial aneurysms had ophthalmic signs or symptoms. Fifty six ( 89%) were females and 7 ( 11%) were males. Fifty- seven ( 89%) were Caucasians. Age ranged from 18 to 75 years ( mean age 53 years + SD). The most common aneurysm location was the ophthalmic artery ( 33%) followed Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 NANOS ABSTRACTS by the posterior communicating artery ( 23%). In 62% ( 39/ 63) of patients, the ophthalmic findings were the presenting manifestation of the aneurysm. The presenting manifestations in descending order of frequency were diplopia ( 23%), decreased vision ( 17%), headache ( 15%), visual field defects ( 14%) and retro- orbital pain ( 6.3%). Shorter duration time of symptoms prior to surgery was not associated with an increased incidence of postoperative ophthalmic improvement (< 1 month, 45% versus > 1 month, 60%). There was a statistically significant correlation between younger age and improved outcome of the presenting sign or symptom. Furthermore, improved outcome was more likely to occur in patients with small ( 1- 14 mm) aneurysms. Large ( 15- 24 mm) and giant (> 25 mm) aneurysms were associated with an almost equal chance of improved or worsened symptoms. Forty- one percent ( 26/ 63) of patients had multiple aneurysms. Postoperatively, forty- one percent ( 26/ 63) of patients developed ophthalmic findings that were not present before surgery. Of these new findings, ptosis and anisocoria were more likely to improve when compared with ocular motility disturbances. Conclusions: Neuro- ophthalmic manifestations are common in patients with unruptured intracranial aneurysms and can often be the presenting feature. The outcome of ophthalmic findings seems to be related to age of patient and size of the aneurysm. Early surgery was not associated with a greater likelihood of improved ophthalmic outcome. A large proportion of patients with ophthalmic findings have multiple aneurysms, some of which may be remote from the visual and ocular motility pathways. Spatiotemporal Profile of Cortical Responses to Visual Motion Stimuli Analyzed by MEG First Author: Satoshi Kashii, MD, Kyoto, Japan Objective: To elucidate the spatiotemporal activity of a cortical network for processing the motion perception, a magnetic encephalographic ( MEG) study was performed in six healthy right- handed subjects. Methods: A random dot kinematogram was used for the visual stimuli. It consists of 320 white square dots randomly projected on a screen against a global dark background. The subjects were instructed to look at the fixation point at the center of the screen with both eyes. Each dot moved smoothly at a constant speed. Coherent movement of 390 milliseconds duration and random movement of 1320 milliseconds were presented alternately. During the coherent period, a certain proportion of the total dots ( i. e. 50, 70 and 100%) moved in a uniform direction, whereas the rest of the dots moved in random but constant directions. The magnetic responses were recorded in a magnetically shielded room using a 122- channel whole- head magnetometer. Results: Two components of magnetic fields were observed in the bilateral temporal and occipital areas in all subjects, with right temporal dominance. We focused on the first component in the right temporal area as they had large amplitudes and were readily distinguished. As the coherence levels rose, the peak latencies of the first component shortened markedly. The mean latencies (± SD ) for the coherence level of 100, 70 and 50% were 233 ± 21.6,250 ± 22.5 and 278 ± 35.4 milliseconds, respectively. Estimated sources of the first components in all subjects were consistently identified in the right lateral occipitotemporal cortex, the human MT/ V5. As the coherence level increased from 50,70 to 100%, the dipole strength increased from 3.8 ± 3.1, 5.0 ± 3.9, to 8.3 ± 6.9 nAm, respectively. Conclusions: These results suggest that the coherence level of dot motion stimulus is reflected in the net activation of hMT/ V5. The Effect of Optic Disk Edema on Spontaneous Venous Pulsations in the Absence of Elevated Intracranial Pressure First Author: Timothy McCulley, MD, Cincinnati, Ohio Background: Although often considered a sign of elevated intracranial pressure, alternative factors may influence the loss of spontaneous venous pulsations of the optic disk. Objective: To evaluate the effect of optic disk edema on spontaneous venous pulsations in the absence of elevated intracranial pressure. Methods: Twenty consecutive patients with unilateral optic disk edema due to anterior ischemic optic neuropathy ( n = 11) or optic neuritis ( n = 9) and a normal contralateral optic nerve were evaluated for the presence of spontaneous pulsations of the superficial veins of the optic nerve head in both eyes. The proportion of subjects with spontaneous venous pulsations present in both eyes, in the involved eye only, in the uninvolved eye only, and in neither eye was determined. Exact McNemar's test was used to evaluate an effect of optic disk edema on the presence of spontaneous venous pulsations. Results: Spontaneous venous pulsations were observed in 60% ( 12/ 20) of uninvolved eyes and 5% ( 1/ 20) of eyes with optic disk edema. This difference was statistically different ( p < 0.005). Venous pulsations were absent in both eyes of seven subjects, and present only in the uninvolved eye of twelve patients. No subject had venous pulsations present in both eyes. One patient with ischemic optic neuropathy and _ 176 . , „ . , „ . . © 2002 Lippincott Williams & Wilkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 segmental optic disk edema had a venous pulsation present on the non- edematous half of the nerve. Conclusions: Optic nerve head edema may cause the cessation of spontaneous venous pulsations in the absence of elevated intracranial pressure. Isolated Neurogenic Blepharoptosis Secondary to Traumatic Eyelid Injury First Author: Timothy McCulley, MD, Cincinnati, Ohio Background: Isolated neurogenic blepharoptosis is rare and when encountered usually due to compression of the oculomotor nerve. Objective: To describe a series of patients with isolated neurogenic ptosis secondary to traumatic eyelid injury. Methods: Case series. Results: Three previously healthy patients ( 2 males, 1 female; ages 29, 37, and 39) were evaluated for blepharoptosis following eyelid trauma. All three injuries involved forceful anterior displacement of the involved upper eyelid by a finger placed under the eyelid. Two occurred during domestic altercations and one while the patient was playing basketball. All patients were evaluated within 48 hours of injury and found to have complete ptosis with no levator function, consistent with loss of innervation to the levator palpebrae superioris muscle. Additional findings included minimal eyelid ecchymosis and edema in all cases and subconjunctival hemorrhage in two cases. All patients had full ocular motility and were orthophoric in all fields of gaze with no anisocoria. The remainder of the examinations of the involved and contralateral eyes was unremarkable with visual acuities correctable to 20/ 20. All cases were managed conservatively with observation only, and within two weeks all three patients had recovered completely with normal symmetric lid height and levator function. Conclusions: Ptosis secondary to isolated injury to the oculomotor nerve branch to levator palpebrae superioris may result from traumatic anterior displacement of the upper eyelid. Complete resolution is likely within two weeks. Surgical Treatment of Skew Deviation First Author: Robert Sanke, MD, Oklahoma City, Oklahoma Background: Skew deviation is a supranuclear vertical ocular misalignment, which may cause comitant or non-comitant hypertropia. Alternating hypertropia on lateral gaze is a common pattern, and the incomitance of this condition makes strabismus surgery challenging. Objective: To evaluate results of strabismus surgery for skew deviation. Methods: A retrospective chart review of patients with skew deviation seen from January 1992 until November 2001 was undertaken. Ten patients who underwent strabismus surgery for skew deviation were identified. Successful outcome was defined as 2 prism diopters or less of vertical deviation in the primary position, coupled with patient satisfaction on subjective questioning. Results: The most common cause of skew deviation was stroke, which was seen in 6 patients. Eight of the 10 patients were successfully treated, 6 with one surgical procedure and 2 with two procedures. Patients with alternating hypertropia ( bilateral inferior rectus skew pattern) were treated with bilateral inferior rectus resection, and in one case with subsequent bilateral superior rectus recession. Patients with non- alternating or comitant hypertropias were treated with either ipsilateral inferior rectus resection or contralateral inferior rectus recession. One case of skew occurred with a comitant exotropia and was successfully treated with vertical transposition of the horizontal rectus muscles. The two patients who failed surgical management were treated with a combination of oblique weakening procedures coupled with vertical rectus surgery. Conclusions: Skew deviation can be amenable to strabismus surgery. For alternating hyperopia, bilateral inferior rectus resection is our treatment of choice and may be coupled with superior rectus recession when needed. Non-alternating or comitant vertical deviations are well managed with conventional vertical rectus muscle surgery, but vertical transposition of the horizontal recti may be useful when there is an accompanying large horizontal deviation. Oblique muscle surgery is ineffective and not recommended for this condition. Should Phenylephrine Be Used as a Topical Mydriatic Agent in Ischemic Optic Neuropathy? First Author: Misha Pless, MD, Pittsburgh, Pennsylvania Objective: To report 4 patients with the diagnosis of non-arteritic ischemic optic neuropathy ( NA- ION) who experienced acute worsening of visual function after instillation of phenylephrine for dilated funduscopic examination. Methods: A retrospective chart review of 125 patients treated in the Neuro- ophthalmology unit of the University of Pittsburgh's Eye and Ear Institute between 1996 and 2001 with the diagnosis of NA- ION. Results: Between 1996 and 2001,4 patients ( age range 54- 82, IF 3M) were identified who experienced acute worsening of visual function immediately or shortly after Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 NANOS ABSTRACTS administration of topical mydriatic agents given for fundu-scopic visualization and diagnostic purposes. In all cases one drop each of 2.5% phenylephrine and 0.5- 1% tropic-amide was used. Three patients had classic risk factors such as hypertension, diabetes, and had a contralateral " disc at risk". The female and youngest patient had ION presumed secondary to Lupus. The range of time from acute visual loss to presentation to neuro- ophthalmic care ranged from 1- 6 days. The time of onset of the decline in visual function ranged from 45 minutes ( patient with lupus) to 12 hours after instillation of mydriatic drops. Visual acuity at diagnosis ranged from 20/ 40- 20/ 400. Conclusions: Phenylephrine is a mydriatic which has vasoconstrictive properties and which may be absorbed by the cornea, thus yielding non- negligible intraocular concentrations. Vasoconstriction of the watershed posterior ciliary capillary beds may occur as a result. This may cause further infarction of already compromised circulatory territories in edematous optic nerves. Because phenylephrine is a known vasoconstrictor in vivo and in vitro it is potentially more likely to cause deleterious vasoconstriction and an acute decline in visual function in patients with acute ION, than tro-picamide. After reviewing these data I have modified my practice so phenylephrine is no longer used to dilate pupils of patients with ION or retinal artery occlusion. Mobius Syndrome Plus First Author: Jacqueline Leavitt, MD, Rochester, Minnesota Objective: To report a patient with Mobius- like syndrome with intact lateral rectus function and multisystem abnormalities including: hypogonadism, short stature, peripheral neuropathy, mental retardation, hearing loss, and anosmia. Methods: Case report and literature review. Results: 23- year- old woman admitted for GI pain. History of intrauterine growth retardation. She has short stature, hypogonadism, hearing loss, anosmia, mental retardation, and progressive sensorimotor peripheral neuropathy. Family history was negative for ptosis or any EOM problems. Since childhood she has had facial diplegia and ophthalmopare-sis. She has incomplete asymmetric ptosis, large angle ex-otropia, intact lateral gaze but marked deficit of infraduc-tion, supraduction and adduction OU. Dolls head maneuver was negative. Pupils were 3mm and nonreactive. MRI: Asymmetric skull, colpocephaly, atrophic corpus callosum, small cerebral peduncles and periatrial white matter atrophy. Workup for mitochondrial cytopathy was negative. Conclusions: Mobius originally described facial diplegia and bilateral abducens palsy. Subsequently cases in the literature have been called Mobius syndrome that have facial diplegia associated with a host of other cranial nerve palsies. This case may represent a separate entity from Mobius syndrome and has more extensive systemic and cranial nerve involvement then previously described cases of Mobius and hypogonadism and neuropathy. Photophobia in Patients with Pseudotumor Cerebri First Author: Deborah Friedman, MD, Syracuse, New York Objective: To determine the prevalence of photophobia at the time of diagnosis of Pseudotumor Cerebri ( PTC). Methods: Neuro- ophthalmic medical records of 180 patients with a diagnosis code of PTC or papilledema from 1991 to 2000 were obtained. Eighty- two patients met predetermined criteria for the diagnosis of PTC. These records were systematically reviewed ( DIF) to determine if photophobia was reported by patients at their initial presentation of PTC. Results: Five patients ( 6%) indicated photophobia during their first interview, which was often severe. Conclusions: Our retrospective review indicates that photophobia as one of the presenting symptoms of PTC is uncommon but, when present, can be quite dramatic. Review of the literature indicates that it is rarely reported. It is unclear whether the photophobia in PTC is etiologically related to other central causes of photophobia. Adjuvant therapy for invasive sino- orbital fungal infection First Author: Roger Turbin, MD, Newark, New Jersey Background: The treatment of invasive sino- orbital fungal infection is complicated both by the presence of comorbid medical conditions, and the resulting disfigurement of radical surgical debridement. Both of these factors limit therapeutic options. Aggressive surgical debridement, often including extensive sinus surgery supplemented by exenteration and administration of systemic antifungals, is common. Despite aggressive surgical and medical intervention, however, these infections typically result in a prolonged disease course with high morbidity and mortality. Objective: To report the authors' experience in the management of invasive sino- orbital fungal infection treated with retrobulbar injection and surgical or postoperative irrigation of amphotericin B ( AmphB) as an adjuvant to systemic antifungal therapy, and limited, conservative, biopsy or debridement procedures. Methods: Retrospective chart review of a consecutive case series. Data concerning comorbid conditions, vision, ^ 17o . , . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 motility and orbital examination, radiographic findings, and clinical outcome are provided. Fungal species, treatment data, and surgical procedures are described. Results: Five patients suffered biopsy proven invasive fungal sino- orbital infection ( 2 rhizopus, 1 mucor, 1 aspergillosis, 1 rhodotorula mucilaginosa/ alternaria/ hormo-graphiella) related to comorbid conditions ( leukemia, renal transplant, diabetic keto- acidosis, corticosteroid therapy, retained intraorbital foreign body) Adjuvant therapy supplementing systemic antifungal therapy included retrobulbar/ peribulbar injection ( 3- 5 mL of 2mg/ cc AmphB) and sino- orbital irrigation ( 50- 500cc of 0.2- 0.5mmg/ cc AmphB) Two patients who presented prior to onset of complete blindness ( counting fingers, and 20/ 40 soon worsening to no light perception) in the affected eye maintained useful visual function at latest examination ( 20/ 80 and 20/ 25, respectively) Five patients showed improvement in orbital and motility examination parameters. No patient required orbital exenteration, or extensive disfiguring facial debridement. Conclusions: Adjuvant orbital therapy with Amphotericin B is safe and appears effective in controlling orbital fungal infection. In selected cases, it may prevent disfiguring surgery, may be associated with improvement in ophthalmoplegia, and may preserve visual function when begun prior to onset of complete blindness. Retinopathy in Patients with Diabetic Ophthalmoplegia First Author: Lucas Trigler, MD, Oklahoma City, Oklahoma Background: Intuition suggests that the incidences of diabetic retinopathy and CN palsy are directly proportional, but this has not been proven. Objective: To review clinical characteristics, prevalence, and severity of retinopathy in patients with diabetes with cranial nerve ( CN) III, IV, and VI palsies, and to determine if type or duration of diabetes, or severity of retinopathy correlates with cranial neuropathies. Methods: Retrospective chart reviews of patients with CN III, IV, or VI palsy were performed at the Bascom Palmer Eye Institute ( BPEI) from 1/ 91 through 12/ 97 and at the Dean A. McGee Eye Institute ( DMEI) from 1/ 94 through 7/ 01. Patients were included if DM was determined to be the etiology of the CN palsy. Prevalence and severity of retinopathy were compared with data from the Wisconsin Eye Study for Diabetic Retinopathy ( WESDR) as a control. Results: Of 2,229 patients with ocular motor CN palsy, 307 ( 13.8%) were due to DM. The prevalence of CN involvement was: VI ( 50%), III ( 43.3%), and TV ( 6.7%). At each location, the prevalence of retinopathy with respect to duration of diabetes was lower in both IDDM and NIDDM Type II patients with diabetes as compared with controls ( BPEI p = 0.009 and p = 0.005; DMEI p = 0.004 and p = 0.29). When data from both locations was combined, the difference was even more significant ( IDDM p = 0.001 and NIDDM p = 0.006). There were no significant differences between the two locations in gender, type or duration of DM, age at presentation, or frequency of CN involvement. All three populations differ with respect to time period studied and racial distribution. Conclusions: Type II patients with diabetes with ocular motor CN palsy have significantly less diabetic retinopathy than controls. Theories that explain this observation will be presented. Charles Bonnet Syndrome Precipitated by Brimonidine Tartrate Eye Drops First Author: Robert Tomsak, MD, Cleveland, Ohio Background: Brimonidine tartrate ( BT) ( Alphagan) is an alpha- 2 adrenergic agonist marketed for treatment of primary open angle glaucoma ( POAG) and may have neuroprotective effects ( 1). One report of acute delusional psychosis with auditory hallucinations in a 68- year- old man treated for POAG is published ( 2). Here I report two patients with bilateral vision and visual field loss from nonar-teritic anterior ischemic optic neuropathy and optic nerve drusen who also had elevated intraocular pressures. Both developed formed visual hallucinations ( Charles Bonnet syndrome; CBS) shortly after beginning the use of BT eye drops ( 3). Objective: To report Charles Bonnet syndrome precipitated by the use of brimonidine tartrate eye drops ( Alphagan) in two elderly visually compromised women. Methods: Observational case series of two patients. Clinical office exams and telephone interviews. Results: Brimonidine tartrate use apparently precipitated the Charles Bonnet syndrome in both cases and cessation of drug did not stop hallucinations. Conclusions: Both patients described above were candidates for the CBS but neither had visual hallucinations prior to using BT. The hallucinations did not completely stop after BT was discontinued, although confusion rapidly cleared in Case 1. Visual hallucinations caused by oral clonidine, another alpha- 2 adrenergic agonist, are well documented ( 4,5). Auditory hallucinations, depression, confusion and anxiety have been reported with BT ( 2) and clonidine ( 4,5). Brimonidine tartrate use may cause visual Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 NANOS ABSTRACTS hallucinations when administered to elderly patients with significant bilateral vision and visual field impairment. References: 1. Yoles E, Wheeler LA, Schwartz M. Alpha2- adrenoreceptor agonists are neuroprotective in a rat model of optic nerve degeneration. Invest Ophthalmol Vis Sci 1999; 40: 65- 73. 2. Kim DD. A case of suspected Alphagan- induced psychosis. Arch Ophthalmol 2000; 118: 1132- 33. 3. Schultz G, Melzack R. The Charles Bonnet syndrome: " phantom visual images". Perception 1991; 20: 809- 25. 4. Brown MJ, Salmon D, Rendell M. Clonidine hallucinations. Ann Int Med 1980; 93: 456^ 157. 5. Lavin P, Alexander CP. Dementia associated with clonidine therapy. Br Med J 1975; 1: 628. An Atypical Bruit First Author: Mitchell Wolin, MD, Greenville, South Carolina Abstract: A 46- year- old female started having diplopia about 5 days prior to presentation. This was initially noted when driving and then became more constant. She was aware of diplopia the prior week while in the hospital for a kidney biopsy. The patient had recently been under evaluation for a kidney problem. She had been found to have elevated urinary protein, and underwent kidney biopsy, and she required 2 units of blood the following day. She has had headaches for about 2 months. She had also been fatigued recently. She had been aware of a roaring sound in her head for about 5 weeks that was either constant or pulse like. This worsened with laying down. This roaring sound started about one week following an upper respiratory infection that subsequently resolved. PMII is notable for an old hear murmur. She has had headaches on a recurrent basis in the past measuring 16 ET in right gaze, 18 D in primary and 14 D in left gaze. Discs revealed 1+ edema OU. Auscultation of the head revealed a somewhat high- pitched bruit, best heard in the temples and consistent with the pulse rate. Review of the kidney biopsy showed mild focal global glomerular sclerosing change, suggestive of low- grade focal sclerosing nephropathy. It was debated whether to proceed directly to cerebral arteriography, given the suggestive findings of a dural carotid cavernous fistula. However, a CT scan was obtained which showed an apparent mass in the region of the torcula, with homogenous and intense enhancement with erosion of the inner table of the occipital bone. Angiography did not show any CC fistula, but did show findings of Significant flow through the mass and no obliteration of the veins. There is no arterial shunting to the tumor mass. A neurosurgeon felt that this did not represent a meningioma, and possibly representing an eosinophilic granuloma. An MRI scan was performed which showed the torcula mass. It was decided to proceed with an open biopsy of the mass, which was performed on 10- 29- 99 without complications. This revealed a plasmacytoma. She underwent bone marrow biopsy, which confirmed the impression of multiple myeloma. It was determined that the nephrotic syndrome was due to Bence Jones proteinuria. She subsequently underwent treatment with chemotherapy. She developed near renal failure after about 5 months, and died about 1 year after diagnosis. An audible cranial bruit in the setting of mild bilateral 6th nerve palsies and mild papilledema initially suggested the possibility of a carotid cavernous fistula. The finding of a mass lesion in the back of the head due to a plasmacytoma from undiagnosed multiple myeloma was quite unusual. The nephrotic syndrome was determined to be secondary to the urinary protein from the myeloma. Isolated Pupil- Dilated Painful Third Nerve Palsy as an Initial Presentation of Systemic Lymphoma First Author: Grace Kao, MD, Orange, California Background: " The rule of the pupil" has implied that pupil- dilated third nerve palsy is usually due to a compressive lesion, and pupil- sparing to an ischemic metabolic or non-compressive, reversible lesion. This notation is challenged in some conditions of pupil- dilated third nerve palsy with systemic lymphoma. Objective: To report a case of complete third nerve palsy violate " the rule of pupil" and possible mechanism. Methods: Case report and review of literature. Results: A 41- year- old healthy white man developed a progressive painful left third nerve palsy over 2 weeks to a complete palsy with a fixed 7.5 mm pupil after a severe flu. He otherwise had normal neurologic and neuro- ophthal-mological examination. Brain and orbit MRIs did not show any abnormality except more enhanced left superior ophthalmic vein than the right. Cerebral angiogram, CSF profile, cytology, virus titer, PCR and cultures of herpes viruses, Lyme disease, angiotensin- converting enzyme were all negative. Eye pain resolved after IV methylprednisolone 1 g/ d for 3 days but not the ocular palsy until 6 weeks later. Two months after the onset, when left third nerve almost resolved, he had another Flu followed by left facial palsy and hoarseness. Repeated MRI of brain was negative. CSF showed 129 WBC, 83% lymphocyte, protein 91 mg/ dl and rest of CSF bacteria and viral cultures, titers, PCR, ACE titer was negative as well as flow cytometry. Facial palsy resolved after one day IV methylprednisolone. Further workup revealed plural effusion and mediastinal mass on CT scan. Mediastinoscopic biopsy after steroid for 5 days revealed necrotic fibrotic tissue. He remained stable until 6 weeks later when he had dysphagia and breathing difficulty. The mediastinal mass recurred with pericardial n 1 8 0 . , „ . . „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. NANOS ABSTRACTS JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 effusion. CSF showed 219 WBC with 87% lymphocyte and 11% atypical lymphocyte, protein 160 mg/ dl, glucose 66 mg/ dl. Flow cytometry of CSF was diagnostic for T- cell lymphoblastic lymphoma. He was then treated with systemic and intrathecal chemotherapy followed by whole brain irradiation with remission until 3 months later when he developed severe multifocal motor sensory peripheral neuropathy. Left third nerve function has remained almost resolved. Conclusions: Isolated third nerve palsy with fixed dilated pupil without radiologically demonstrated lesion and normal CSF can be a preceding sign of systemic malignant lymphoma. A microinfiltrative process not radiologically visible of the third nerve proximal to the orbital apex is the presumptive mechanism. Only 4 reports in literature described isolated third nerve palsy in lymphoma and none had a complete palsy with a full blown pupil that recovered later despite systemic recurrence. Adaptive Neural Mechanism for Listing's Law Revealed in Patients with Fourth Nerve Palsy First Author: Agnes Wong, MD, St. Louis, Missouri Background: During fixation and saccades, human eye movements obey Listing's law, which specifies the eye's torsional angle as a function of its horizontal and vertical position. Objective: To investigate whether the brain adapts to defective torsional control after fourth nerve palsy. Methods: Thirteen patients with fourth nerve palsy ( 11 chronic, 2 acute), and ten normal subjects were studied using scleral search coils. With head immobile, subjects made saccades to a target that moved between straight ahead and 8 eccentric positions. From the eye position data, we computed a plane of best fit, called Listing's plane. Violations of Listing's law were quantified by computing the ' thickness' of this plane, defined as the standard deviation of the distances to the plane from the data points. Results: Patients with chronic fourth nerve palsy obeyed Listing's law in both the paretic and non- paretic eyes during fixation and saccades; however, Listing's planes of both eyes had abnormal orientations, being rotated temporally. In contrast, the paretic eye of patients with acute palsy violated Listing's law during saccades. During downward saccades, transient torsional deviations moved the paretic eye out of Listing's plane. Torsional drifts returned the eye to Listing's plane during subsequent fixation. Conclusions: During saccades, acute fourth nerve palsies violate Listing's law, whereas chronic palsies obey it, indicating that neural adaptation can restore Listing's law by adjusting the innervations to the remaining extraocular muscles. Although Listing's law is obeyed in chronic palsy, Listing's plane is rotated temporally, as a manifestation of excyclotorsion during downgaze and incyclotorsion during upgaze. In acute palsy, rapid torsional deviations and slow torsional drifts occur during and immediately after downward saccades. These saccadic intrusions are attributed to pulse- step mismatch, as a result of lesions in the trochl |