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Show Journal of Neuro- Ophthalmology 21( 3): 231- 232, 2001. © 2001 Lippincott Williams & Wilkins, Inc., Philadelphia Neuro- Ophthalmology at Large 53r Annual Meeting of the American Academy of Neurology, May 6- 12, 2001 Mark L. Moster, MD, John Guy, MD These are a selection of papers and posters covering neuro- ophthalmology and neuro- ophthalmologic aspects of neurologic disorders that were presented at the 53rd Annual Meeting of the American Academy of Neurology, Philadelphia, PA, May 6- 12, 2001 [ Neurology 2001; 56( suppl 3): A1- 481]. The selections are arranged by topic. TUMORS A series of seven infants treated for visual pathway gliomas with chemotherapy and occasionally partial resection demonstrated similar outcomes as did older children, in contrast to prior reports that have found that infants are less responsive to treatment ( Packer R, Washington, DC, P01.060). MITOCHONDRIA A new mitochondrial mutation at site 9995 was found in an Iraqi Jewish family with optic atrophy and dystonia ( Brown MD, Atlanta, GA, P02.091). VISUAL FIELDS Sita- Fast Humphrey perimetry is as reliable as Gold-mann Perimetry in most patients with severe ( 20/ 200 or worse) visual loss or severe neurologic deficits ( Szat-mary G, Atlanta, GA, S06.002). INTRACRANIAL HYPERTENSION 1. Twelve patients with idiopathic intracranial hypertension occurring during pregnancy responded well to treatment with weight control and lumbar punctures ( when necessary); acetazolamide was not necessary for control ( Kupersmith M, New York, NY, S06.003). 2. Cerebrospinal fluid pressure monitoring for an average of 1.9 days was helpful in establishing the diagnosis of pseudotumor cerebri in patients without papilledema ( Williams MA, Baltimore, MD, P02.146). EYE MOVEMENTS 1. Two patients had loss of saccadic eye movements after cardiac surgery without magnetic resonance imaging ( MRI) lesion to explain the deficit. They were disabled by inability to read or survey their environment ( Stahl JS, Cleveland, OH, S08.004). 2. Patients with Machado- Joseph disease ( SCA- 3) had loss of the vesti-bulo- ocular reflex, helping to distinguish this disorder clinically from patients with other spinocerebellar ataxic syndromes ( Gordon CR, Kfar Saba, Israel, S08.003). 3. In SCA- 7, slowing of voluntary saccades was seen even in patients with subclinical disease ( OH AK, Los Angeles, CA, P01.044). 4. Presymptomatic gene carriers of Huntington's disease had abnormalities in saccadic function, OKN gain, and pursuit ( Blekher TM, Indianapolis, IN, P01.101). 5. MRI in progressive supranuclear palsy ( PSP) showed mesencephalic atrophy, fourth ventricular enlargement, increased signal in globus pallidus and putamen, and increased interpeduncular angle ( Rolland Y, Rennes, France P04.011). 6. A study of slow vertical saccades in PSP found that it was unlikely that this was owing to involvement of the omnipause neurons and more likely to involvement of burst neurons ( Leigh RJ, Cleveland, OH, S08.002). 7. A review of 110 patients with biopsy- proven temporal arteritis found 15 ( 12.7%) with diplopia; five had third nerve paresis, three had sixth nerve paresis, and six had transient diplopia with a normal examination ( Cornblath WT, Ann Arbor, MI, S06.005). LABYRINTHINE DYSFUNCTION 1. A new bedside test of utricular function, called the " head- heave maneuver," consists of having the examiner hold the head and move it laterally with a very short, rapid excursion. Corrective saccades are seen in patients with unilateral labyrinthine hypofunction, or cerebellar disease ( Zee DS, Baltimore, MD, S08.001). 2. A series of patients with " complex paroxysmal positional nystagmus" was described. This is a manifestation of benign paroxysmal postural vertigo ( BPPV) involving multiple semicircular canals. These patients require more complicated treatment than do those with conventional BPPV ( Fife TD, Phoenix, AZ P01.042). MULTIPLE SCLEROSIS 1. The NIH VFQ- 25 questionnaire is an effective measure for capture of visual loss in multiple sclerosis ( MS) 231 232 NEURO- OPHTHALMOLOGY AT LARGE patients who have a high degree of self- reported visual dysfunction ( Balcer LJ, Philadelphia, PA, P01.049). 2. A 20- year follow- up study of MS patients who presented with optic neuritis ( ON) or diplopia revealed that 81% of ON patients had normal visual acuity but that subtle abnormalities persisted. Those presenting with diplopia more frequently switched to secondary progressive MS with decreased visual acuity than did those with optic neuritis ( Vorobeychik G, Vancouver, BC, Canada, P03.099). 3. The ONTT found that African- Americans had a non- statistically significant tendency to develop clinically definite MS at a lower rate compared with Caucasians ( 20% versus 32%), but African Americans who developed MS had worse visual impairment than did Caucasians ( Nazarian SM, Little Rock, AR, P03.093). VISUAL PROCESSING 1. A study of functional MRI ( FMRI) in patients looking at objects displayed in non- canonical ( unusual) views was compared with subjects looking at objects displayed in canonical ( usual) views. During viewing of non-canonical objects, there was activation of the left occipitotemporal and right posterior parietal cortex and a small degree of cerebellar activation ( Terhune KP, Philadelphia, PA, P01.050). 2. Although form perception, face recognition, and color perception are processed by the parvocellular visual pathway, a study of two patients revealed a separation between performance on shape perception as compared with color or facial perception, providing evidence for separation of these functions in the ventral ( occipitotemporal) pathway ( Kim JS, Cheju, Republic of Korea, S06.004). 3. In contrast to patients with developmental ( congenital) prosopagnosia, those with acquired prosopagnosia were able to perform covert processing of faces, as evidenced by their ability to recognize faces when given the name of a person to match to the face. Presumably, congenital prosopagnosia patients do not create a store of accurate facial memories in the brain ( Barton JJS, Boston, MA, S35.007). 4. Two patients with acquired cerebral achromatopsia were able to perform saccades to isoluminant chromatic stimuli, which they could not see, demonstrating a sort of " blind-sight" ( Celesia GG, Maywood, IL, S35.005). 5. A syndrome of transient topographic disorientation was described. Patients had transient inability to navigate through familiar environments. Five of 10 patients had recurrent episodes. Studies of single- photon emission computed tomography ( SPECT) and neuropsychologic tests suggested a persistent right hemisphere dysfunction. These patients have characteristics similar to those of transient global amnesia in that they demonstrate no evidence of seizures or cerebrovascular disease ( Gil- Neciga E, Seville, Spain, P01.099). VISUAL HALLUCINATIONS 1. Visual hallucinations are considered a major feature of dementia with Lewy bodies ( DLB). Forty- seven of 66 patients with DLB ( 72%) had visual hallucinations, most often involving images of people. Auditory hallucinations or delusions were only seen in those who also had visual hallucinations. Delusions consisted of phenomena that were related to the patients' visual hallucinations ( Ferman TJ, Jacksonville, FL, P03.044). 2. Among a cohort of 99 Parkinson's disease patients, 29 began the study with visual hallucinations and 30 developed them during the study. Of the 29 with study- onset hallucinations, only four ( 14%) showed resolution. Among the 60 patients who did not have visual hallucinations at study onset, 50% developed them within 4 years. Neuroleptics rarely helped in resolving the visual hallucinations ( Goetz CG, Chicago, IL, P03.147). MIGRAINE Late- life migraine equivalents, such as the scintillating scotoma, showed excellent response to topiramate. Five patients over age 65 with attacks lasting from 15 minutes to 1 hour, occurring five to 14 times per week, had complete resolution within 12 weeks of treatment with an average dose of 78 mg/ d ( Roberson S, Nashville, TN, P01.128). STROKE 1. The results of the multicenter Warfarin- Aspirin Recurrent Stroke Study ( WARSS), comparing long- term anticoagulation to aspirin in 2,206 patients with minor stroke, showed no difference between treatment groups ( warfarin only, aspirin only) in the two outcomes of recurrent stroke and death. Both treatments were equally safe ( PL01.002). 2. A study of patients with spontaneous cervical artery dissections found that risk factors included recent infection, migraine, and minor trauma or exercise ( Guillon B, Nantes, France, P03.002). 3. Thirty-two of 110 consecutive patients with carotid artery occlusion had ocular ischemic findings on examination and four of these patients developed severe signs of clinical ocular ischemia ( Klijn CJM, Utrecht, The Netherlands, P04.113). 4. Anticoagulation for radiation optic neuropathy showed improvement in three patients, but visual details were not provided in enough detail to draw much meaning from this small series ( Venketasubramanian N, Singapore, P01.051). EYELID A study of Botox treatment of apraxia of lid opening provided further evidence that the benefits of Botox are explained by diminishing activity of the hyperactive orbicularis oculi. The treatment was generally effective ( Boghen D, Montreal, Canada, P01.053). TRANSPLANTATION Based on evidence from animal models of Parkinson's disease that intrastriatal implantation of retinal pigment epithelial cells produces benefit, a pilot study of six patients was performed. Surgery was tolerated well and initial efficacy evaluation showed promise ( Watts RL, Atlanta, GA, P04.102). / Neuro- Ophthalmol, Vol. 21, No. 3, 2001 |