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Show NEURO-OPHTHALMOLOGY AT LARGE The 60th Annual Meeting of the American Academy of Neurology Chicago, Illinois, April 12-19, 2008 The 60th Annual Meeting of the American Academy of Neurology took place in Chicago, Illinois, from April 12 to 19, 2008. Eleven educational courses in the field of neuro-ophthalmology and neuro-otology were given, ranging from breakfast seminars to the traditional all-day course. Faculty included Robert Baloh, MD, Valerie Biousse, MD, James Corbett, MD, Wayne Cornblath, MD, Kathleen Digre, MD, Eric Eggenberger DO, Scott Eggers, MD, Terry Fife, MD, Steven Galetta, MD, Timothy Hain MD, Janet Helminski, PhD, Aki Kawasaki, MD, R. John Leigh, MD, Mark Moster, MD, Nancy Newman, MD, Valerie Purvin, MD, Janet Rucker, MD, Barbara Scherokman, MD, Jade Schiffman, MD, Michael Strupp, MD, Rosa Tang, MD, Jonathan Trobe, MD, Ronald Tusa, MD, Michael Wall, MD, and David Zee, MD. Approximately 1,560 scientific platform papers and posters were presented at the meeting. The material of most interest to neuro-ophthalmologists is summarized here. NEURODEGENERATIVE DISEASES A flash electroretinogram (ERG) study of 16 patients with dementia with Lewy bodies (DLB) demonstrated phot-opic a-wave and photopic and scotopic b-wave abnormal-ities. Pathologic analysis in 6 retinas showed cytoskeletal disorganization of the cone photoreceptor layer, pale inclu-sions in the outer plexiform layer and abnormal distribution of synucleins. No similar findings were seen in patients with Parkinson disease (PD) with visual hallucinations or in normal control retinas. DLB appears to differ from PD in the higher frequency of visual hallucinations, which may be of retinal origin, given the structural abnormalities dis-covered in the inner retina in DLB (Devos D, Lille, France, P01.188). The Useful Field of View (UFOV) test has been predictive of the ability to perform activities of daily living, including driving. Patients with PD and age-matched con-trol subjects referred to a driving rehabilitation specialist underwent the UFOV test, which was correlated with driving outcomes. Patients with PD performed significantly worse than the control group in UFOV subtests 1 (visual processing speed) and 2 (divided attention). Lane mainte-nance errors and rating of crash risk were significantly and strongly correlated with UFOV subtests 1 and 2 (McCarthy DP, Gainesville, FL, P01.192). Abnormalities in contrast sensitivity and stereoacuity are common among patients with PD. Freezing of gait in patients with PD is usually refractory to medications but may respond tovisual cues. To see if there was a relationship between the visual deficits and freezing of gait, 96 con-secutive patients with PD were evaluated for contrast sen-sitivity and stereoacuity and with a self-report of freezing of gait, but no significant correlation between the symptoms and visual function was seen (Wielinski CL, St. Louis Park, MN, P02.020). In a retrospective study, 11 patients who met estab-lished diagnostic criteria for possible progressive supra-nuclear palsy (PSP) were compared with 8 patients with possible PD and 9 normal control subjects to identify findings that might help diagnose PSP. Neuro-ophthalmic findings that differentiated PSP from PD included slowed horizontal saccades (0.006), the presence of square wave jerks (SWJ) (0.001), apraxia of eye lid opening (0.029), abnormal vertical optokinetic nystagmus (OKN) (0.001), lid-retraction stare (0.001), blink rate (0.021), and light sensitivity (0.021). PSP could be distinguished from PD with 100% sensitivity and specificity by the presence of two of the following features: greater than 5 SWJ, abnormal downward OKN, and lid retraction. Only 1 subject with PSP had the diagnosis confirmed at autopsy. Although the results seem credible, the clinical diagnosis of PSP may be inaccurate and the findings in this study need to be verified with pathologic studies (Hills W, Salt Lake City, UT, P05.042). OPTIC NEURITIS AND MULTIPLE SCLEROSIS A survey assessing the evaluation and management of optic neuritis (ON) by neurologists and ophthalmologists in 7 countries was reported. In all countries, patients present more frequently to ophthalmologists and are subsequently referred to neurologists. Evaluation and management of ON varies among countries. Of the patients with ON, 70%- 80% undergo brain MRI, and sometimes lumbar puncture (mostly in Europe and Thailand). Although most patients 352 J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 Neuro-Ophthalmology at Large J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 receive acute treatment with intravenous steroids, 17.5% of United States neurologists and 17.6% of ophthalmologists still prescribe oral prednisone (1 mg/kg/day) compared with 32% of neurologists and 26.7% of ophthalmologists outside the United States. In all countries, corticosteroids are often prescribed for reasons that are contrary to those in published studies: improving final visual acuity or decreasing the long-term risk of developing multiple sclerosis (MS). Disease-modifying agents are prescribed outside of local official recommendations by 20%-30% of physicians. The authors concluded that interpretation and application of evidence-based medicine generated by United States-based clinical trials vary among countries, guidelines are adapted to local health care systems and access to imaging and drugs, and the same mistakes and misinterpretations of studies are made in many countries (Biousse V, Atlanta, GA, S19.005) Pathologic and clinical studies of MS and ON have suggested that fibers of the papillomacular bundle, which lie temporally in the retinal nerve fiber layer (RNFL), are particularly vulnerable to axonal degeneration. Patients with MS and disease-free control subjects had ocular coherence tomography (OCT)-3 to measure RNFL thick-ness and tests of low-contrast letter acuity and high-contrast visual acuity. Among 352 patients, percent reductions in RNFL thickness were greater in the temporal (17%) than in the superior (13%), nasal (11%), or inferior (11%) quadrants (P # 0.0001). In eyes with a prior history of ON (n = 250), the temporal quadrant showed the greatest percent reductions (26% for temporal vs 15%-18% for other quadrants, P # 0.0001). This feature was not as notable among eyes without a history of ON (11% vs 8%- 9%). Correlations with visual function were greatest for the temporal quadrant. The investigators concluded that thinning of the RNFL in patients with MS is not uniform across retinal quadrants. The greatest degree of axonal loss occurs temporally, particularly in eyes with a prior history of ON. In addition, temporal RNFL thinning is most strongly associated with low-contrast acuity loss (Nemani, D, Philadelphia, PA, P05.046). RNFL thickness of 35 affected eyes with acute ON was studied with OCT and compared with that of unaffected fellow eyes and control eyes. The authors defined swelling as having 3 sectors with a ratio of RNFL thickness >1.1, comparing affected eyes either to the fellow unaffected eyes or to the upper 95th percentile of control eyes. RNFL loss was judged if >3 sectors were thinner than the 5% lower limit of controls or if 2 sectors were >10 mm thinner than same sectors of fellow eyes. Compared with control eyes, 11 (31%) of affected eyes but no fellow eyes had swelling. Compared with fellow eyes, 23 (85%) affected eyes were swollen. The number of swollen sectors did not correlate with MRI optic nerve lesion site or extent or baseline vision. At 1 month, swelling remained in 4 (14%) affected eyes compared with control eyes and in 10 (50%) affected eyes compared with fellow eyes. At 1 month, RNFL loss developed in 54% compared with fellow eyes and in 24% compared with control eyes, and RNFL loss occurred in 50% of eyes still swollen. GDx was also used in this study and showed findings similar to those with OCT (Kupersmith M, New York, NY, S19.004). A study reported RNFL thickness measured by OCT in 58 patients with MS (24 relapsing-remitting, 24 primary progressive, and 10 secondary progressive) with no history of ON. Patients with MS had thinner RNFLs in all four quadrants (mean: P # 0.001, temporal: P # 0.001, superior: P # 0.001, nasal: P = 0.003, and inferior: P # 0.001). Compared with control subjects, RNFL was reduced in relapsing-remitting MS (95 mm, P = 0.002), secondary progressive MS (84 mm, P # 0.001), and primary progressive MS (88 mm, P # 0.001). There was a significant reduction in the RNFL in secondary progressive MS compared with relapsing-remitting MS (P = 0.017). In addition, RNFL thinning was correlated with the Expanded Disability Status Score (EDSS) (r = 0.311, P = 0.018). Low-contrast letter acuity was significantly reduced in primary progressive MS compared with relapsing-remitting MS (P = 0.022) and was correlated with the decrease in RNFL thickness (r = 0.432, P # 0.001). These results suggest that RNFL is reduced in patients with MS without ON. OCT is a useful tool for monitoring axonal loss in the different forms of MS and could help in diagnosis of puzzling cases of MS, including the primary progressive variant (Meyniel C, Paris, France, P05.031). High-dose intravenous methylprednisolone (IVMP) decreases the severity and duration of clinical relapses of MS, and prior studies have shown that high-dose oral corticosteroids have equivalent bioavailability and clinical efficacy. Forty patients with MS within 2 weeks of a clinical relapse with at least one contrast-enhancing MRI lesion were randomly assigned to receive either 1 g/day oral methylprednisolone (OMP) or IVMP for 5 days. At 28 days, the two groups showed significant reductions in enhancing lesions and similar clinical improvement, with no difference in tolerability, suggesting that OMP is as effective as IVMP for acute relapses in patients with MS (Martinelli V, Milan, Italy, P02.135). A few studies dealt with neutralizing antibodies (NAbs) in patients with MS treated with interferon. In the BENEFIT (Betaseron in Newly Emerging Multiple Sclerosis for Initial Treatment) trial, NAb titers were measured using the in vitro MxA assay every 6 months in 277 patients. Data were available for all patients 3 years after randomization. Over 3 years, NAb titers >1:20 NY/mL were detected at least once in 88 (31.8%) patients; 41 (46.6%) patients reverted to NAb-negative status by the 353 J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 Wu-Chen and Moster third year of the trial. There was no significant relationship between NAb titer and time to develop clinically definite MS (CDMS) or time to disability progression (Freedman M, Berlin, Germany P02.148). Another study identified 41 patients under continuous treatment with interferon [IFN]-1b who were NAb-positive for at least 12 months and reverted to being NAb-negative for at least 12 months. These investigators studied 64 matching patients who had remained seronegative during 36 months. During the NAb-positive period, the annualized relapse rate was 0.77 compared with the annualized relapse rate of 0.43 in the permanently NAb-negative patients in matched periods of observation [P = 0.008]. Patients who reverted to a NAb-negative state regained a treatment effect with a relapse rate [0.43] similar to that observed in the permanently NAb-negative patients [0.4] in corresponding time periods [P = 0.67]. The investigators concluded that NAb positivity reduced the therapeutic effect of IFN-1b significantly. When NAb positivity disappeared during continued treatment, patients regained the full effect of INF-1b therapy [Sorensen P, Copenhagen, Denmark, P02.153]. A study investigated gray matter (GM) and white matter (WM) atrophy in MS. There were 73 patients (29 clinically isolated syndrome [CIS]; 33 relapsing-remitting MS [RRMS], and 11 secondary progressive MS [SPMS]) with a mean of 20.0 years from disease onset. Disability in patients was assessed by EDSS and MS Functional Score Composite score (MSFC). Three-dimensional T1 brain magnetic resonance images were obtained on a 1.5-T scanner and from these WM (WMF) and GM fraction volumes (GMF) were derived using SPM2 (statistical parametric mapping). GM and WM atrophy was signifi-cantly greater in patients with MS than in control subjects. There was significantly more GM atrophy in SPMS vs RRMS and in RRMS vs CIS. GMF (but not WMF) correlated with EDSS (rs = 0.48, P # 0.001) and MSFC (rs = 0.59; P # 0.001). The authors concluded that GM atrophy is greater than WM atrophy in patients with MS of relatively long disease duration (~20 years). In addition, GM atrophy seems to be more closely related to disability and disease subtype (Fisniku L, London, UK, S32.001). A report of 30 children younger than 10 years with MS showed that the mean onset age was 4.9 years, 60% were female, and the most common presentation was an acute disseminated encephalomyelitis (ADEM)-like pic-ture. Fifteen children presented with a CIS: brainstem-cerebellar 20%, transverse myelitis 13%, ON 10%, and motor syndrome 10%. After a mean follow-up of 8.3 years, 67% of children had RRMS, 30% had SPMS, and 3% had the Marburg variant. The mean number of relapses was 2.15 in the first year and 3 in the second year of disease. The median time from onset to disability scores of 3, 4, and 6 were 2.8, 3.3, and 5 years, respectively. This group 354 showed a high relapse rate and rapid onset of disability (Tenenbaum S, Buenos Aires, Argentina, P03.024). NEUROMYELITIS OPTICA A pathologic study sought to determine whether neuromyelitis optica (NMO) hemispheric cerebral lesions demonstrate similar complement and aquaporin 4 (AQP4) expression as has been reported for optic nerve and spinal cord NMO lesions. Cerebral lesions from 2 patients with NMO (n = 4 lesions) and 13 patients with MS (n = 57 lesions) were analyzed via routine neuropathologic stains and immunohistochemical analysis against myelin, immune complexes, and AQP4 and compared with optic nerve and spinal cord lesions in 9 patients with NMO. All patients with NMO were seropositive for NMO IgG antibody. NMO cerebral lesions resembled NMO optic nerve and spinal cord lesions, including the presence of active demyelination, eosinophilia, and cavitation. Vasculocentric, periependymal immune complex deposition and AQP4 loss were noted as well. These characteristics were in contrast to those of cerebral and periventricular MS lesions, in which AQP4 expression was increased or retained in the periventricular ependymal lining (Roemer S, Rochester, MN, S27.003). NMO has shown mechanisms in common with myasthenia gravis (MG) in that IgG in both disorders triggers antigenic modulation and complement activation. Sera submitted to the Mayo Clinic Neuroimmunology Laboratory (1981-2007) in 16 patients who met clinical diagnostic criteria for both NMO and MG were identified. All were women and were seropositive for NMO IgG and muscle acetylcholine receptor (AChR) antibody (13 of 14 tested were confirmed for AQP4-specific IgG). Fifteen patients (94%) had generalized MG; in 1 patient MG was restricted clinically to extraocular muscles. None had chest CT evidence of thymoma. Eleven patients (69%) had undergone thymectomy (9 with hyperplasia and 2 normal). All benefited from conventional therapy for MG; 12 had a remission. In 15 patients (94%) MG onset preceded NMO (mean interval 11.6 years; range 0.5-30). Fourteen patients had NMO and 2 had recurrent longitudinally extensive transverse myelitis without ON. Three patients (19%) had MRI brain abnormalities, predominantly diencephalic and brainstem. This study suggests a link between NMO and MG (Chan K, Rochester, MN, S27.006). CEREBROVASCULAR DISEASE Twenty-one patients with superior sagittal sinus thrombosis were reviewed to identify radiologic factors associated with hemorrhagic infarcts seen in 11 patients (52.4%). Nine patients (81.8%) in the hemorrhagic infarct group did not have evidence of collateral venous drainage compared with 3 (30%) in the nonhemorrhagic infarct q 2008 Lippincott Williams & Wilkins Neuro-Ophthalmology at Large J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 group (relative risk [RR] 2.7; 95% confidence interval [CI] 1.0-7.3, P = 0.019). Thrombus volume was not associated with hemorrhagic infarct (Muppidi, S, Wayne, PA, P03.123). Prior studies suggest a higher mortality in patients with ischemic stroke admitted to hospitals on weekends and treated with intravenous tissue plasminogen activator (tPA) (‘‘weekend effect''). A study of weekend vs weekday mortality in 286 patients at 2 university comprehensive stroke centers showed that weekend patients had 12.1% (13 of 107) in-hospital mortality compared with 10.1% (18 of 179) in the weekday group (P = 0.68). This negligible difference suggests that a comprehensive stroke center may erase the weekend effect (Albright K, La Jolla, CA, P01.043) Of 432 consecutive patients with stroke, 17 were identified as having smoked cannabis within 30 minutes of symptom onset and had positive urine drug screen results. The mean age was 41 years. Two patients (both adolescents) died, and pathologic examination revealed hemorrhagic cerebellar infarction without embolus or vasculitis. Although not a controlled study, this report raises the possibility of a link between smoking cannabis and stroke (Singh NN, St. Louis, MO, P01.053). A retrospective study compared emergency carotid endarterectomy (CEA) with intravenous thrombolysis (IVT) in acute internal carotid occlusion. The internal carotid artery recanalization rate was significantly higher in patients undergoing CEA (86.2%) than in those undergoing IVT (15.0%) (P # 0.0001). Moreover, a favorable 1-year clinical outcome was significantly more frequent in patients undergoing CEA (48.3%) than in those undergoing IVT (20.0%) (P = 0.044). The patients undergoing surgery were younger and had less deficit. However, when the analysis was restricted to the subgroups of patients with admission NIH Stroke Scale (NIHSS) scores of 10-20, no significant difference in age (P = 0.07) and sex distribution (P = 0.43) was found; good 1-year clinical outcome was seen in 35.7% of patients undergoing CEA versus 14.3% of those receiving IVT in this subgroup (P = 0.19). CEA may be a better treatment option, but because this study was retrospective, it is difficult to sort out the features that directed the treatment decisions (Herzig R, Lausanne, Switzerland, P01.164). A retrospective study of 100 autopsy-verified cases of severe stroke showed a high percentage of pulmonary embolism in 38% of 76 patients with hemispheric infarcts and in 46% of 24 patients with brainstem infarcts. Pul-monary embolism was the cause of death in 5% of patients with hemispheric infarcts and in 29% of those with brainstem infarcts (P # 0.01) (Piradov M, Moscow, Russia, P02.005). The frequency of cerebral venous and dural sinus thrombosis occurring during the fasting month of Ramadan was compared with that of such events occurring at other times. In 3 neurologic centers over a 5-year period 33 patients with venous strokes were evaluated during Ramadan and 129 during other months. The mean number of new cases during the month of Ramadan was 5.5 compared with 1.95 in other months. The authors concluded that fasting increases the frequency of venous thrombosis (Saadatnia M, Isfahan, Iran, P02.007). A single institutional study reported 131 consecutive cases of cerebral artery dissection not associated with major trauma. In this cohort, 64% were men and 36% were women, with a mean age of 44. Risk factors included hypertension (29%), smoking (28%), migraine (12%), and minor trauma (29%). Presentations included 56% stroke, 15% transient ischemic attack (TIA), and 5% subarachnoid hemorrhage. No cerebrovascular events affected 24% of patients, who presented with headache (52%), Horner syndrome (42%), cervical pain (22%), tinnitus (8%), and cranial nerve palsy (8%). Extracranial dissection occurred in 56%, intracranial dissection in 28%, and both extra- and intracranial dissection in 16%. The carotid artery was involved in 75%, the vertebral artery in 17%, the basilar artery in 2%, and the posterior inferior cerebellar artery in 2%. Treatment consisted of anticoagulation in 55%, antiplatelet agents in 42%, and endovascular procedures in 3%. At follow-up, 87% had no or mild disability, 11% had moderate to severe disability, and 2% died (Falcone GJ, Buenos Aires, Argentina, P02.010). IDIOPATHIC INTRACRANIAL HYPERTENSION A study from three institutions compared the clinical characteristics in men and women in 721 consecutive cases of idiopathic intracranial hypertension (IIH). Men com-prised 9%. As their first symptom of IIH, men were less likely to report headache (55% vs 75%, P # 0.001) but more likely to report visual loss (35% vs 20%, P = 0.005). Fewer men developed headache (79% vs 89%, P = 0.01) and fewer reported tinnitus (26% vs 38%). Visual acuity (VA) and visual fields (VF) at presentation and last follow-up were significantly worse among men. The relative risk of severe visual loss (defined as visual acuity poorer than 20/200 or a visual field perimeter of >20 degrees) for men compared with women was 2.1 for at least one eye (95% CI 1.4-3.3, P = 0.002) and 2.1 (95% CI 1.1-3.7, P = 0.03) for both eyes. Men were also more likely to have sleep apnea (24% vs 4%, P # 0.001) (Bruce B, Atlanta, GA, S19.003). A retrospective chart review of 23 adult patients with IIH sought to determine whether weight gain correlated with clinical recurrence. Body mass index (BMI) was calculated at presentation, at the time of resolution of signs/symptoms, and at recurrence. BMIs for patients with IIH (n = 23; 92% female; age 35 6 9 years) at presentation, 355 J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 Wu-Chen and Moster FIG. 1. Faculty for the American Academy of Neurology 2008 full-day course in Neuro-Ophthalmology. Left to right: Jonathan Trobe, Kathleen Digre, Mark Moster, Valerie Biousse, Nancy Newman, and Steven Galetta. initial resolution, and recurrence were 33 6 6, 33 6 7, and 36 6 7 kg/m2, respectively. Mean difference between presentation and initial resolution BMI was 6.3 kg/m2, whereas the difference between initial resolution and recurrence BMI was +7.4 kg/m2 (consistent with weight gain). BMIs at recurrence were significantly higher than those at initial resolution (P = 0.01, paired t test) and those at presentation (P = 0.01). No significant difference was noted between BMIs at presentation and initial resolution (P = 0.81). Average rate of BMI gain between initial resolution and recurrence was 4.4 kg/m2/year (range 2.2 to 15.8). The investigators concluded that IIH recurrence is associated with a significant BMI increase compared with BMIs at presentation and initial resolution. Patients with resolved IIH should be advised that weight gain increases the risk of recurrence (Ko M, Philadelphia, PA, P05.025). LEBER HEREDITARY OPTIC NEUROPATHY A small subset of patients with Leber hereditary optic neuropathy (LHON) have a negative test for the three primary mitochondrial DNA (mtDNA) point mutations (11778/ND4, 3460/ND1, and 14484/ND6). Eight such patients were investigated with complete mtDNA sequence analysis. Conservation analysis was also performed using a global alignment of 143 sequences from different species. All cases were consistent with a rare mtDNA mutation fulfilling the criteria for being pathogenic. Three patients were found to have the 14568/ND6 mutation, previously reported in a few patients with LHON. The 14459/ND6 mutation (haplogroup J1c), which was previously associ-ated with LHON/dystonia, was found once. In addition, single occurrences of the 3700/ND1 (haplogroup H), 4171/ND1 (haplogroup J2b), 10663/ND4L (haplogroup L2a), and 14495/ND6 (haplogroup H) mutations were found. They were all reported previously in single (3700/ND1) patients or in very few patients. In a few instances, heteroplasmy was identified in maternal rela-tives, indicating a recent mutational event. This study confirms that about 10% of patients with LHON have rare primary mtDNA mutations (Amati-Bonneau, P, Strasbourg, France, P05.026). PARANEOPLASTIC DISEASE Two patients who had presented with narcolepsy and cataplexy and were found to have neuroblastomas were reported. One was a 3-year-old girl who subsequently developed opsoclonus/myoclonus. The second was a 2-year-old girl who also presented with weakness caused by Lambert-Eaton myasthenic syndrome (Sinsioco C, Kiev, Ukraine, P03.005). VESTIBULAR DISORDERS A cross-sectional study of emergency department (ED) visits from the National Hospital Ambulatory Medical Care Survey reviewed patients with the reason-for-visit code of vertigo/dizziness. In this study, 7,925 patients with dizziness comprised 3% of ED visits over 12 years. These patients were often polysymptomatic with other complaints being nausea/vomiting (19%), craniocervical pain (13%), malaise/fatigue (12%), and neurologic symptoms (9%). Chest pain, dyspnea, respiratory symptoms, abdominal pain, syncope, bleeding, palpitations, and fever/chills were 356 q 2008 Lippincott Williams & Wilkins Neuro-Ophthalmology at Large J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 common, with one or more symptom affecting 25%. Auditory/otologic (2.3%) and psychiatric (2.0%) com-plaints were uncommon. Diagnoses fell into the category of otologic/vestibular (27%), cardiovascular (21%), respira-tory (12%), metabolic (11%), neurologic (11%, including 4% cerebrovascular), injury/poisoning (11%), psychiatric (7.5%), digestive (7.4%), and genitourinary (5%). Danger-ous cardiovascular causes were diagnosed with comparable frequency among patients with dizziness as among patients with syncope (angina/myocardial infarction, 2.2% vs 2.8%; arrhythmia, 3.7% vs 4.5%). The authors concluded that among patients with dizziness who present to an ED, dizziness is rarely monosymptomatic, is rarely attributable to a vestibular disorder, and is more likely to be associated with cardiovascular and medical causes, including danger-ous ones such as myocardial infarction and arrhythmia (Newman-Toker DE, Baltimore, MD, S19.001). FACIAL NERVE DISORDERS A retrospective review of 26 children with facial palsy examined over a 10-year period (11 boys and 15 girls) showed that 6 had symptoms of a preceding upper respiratory infection. Their ages ranged from 2 months to 18 years (mean 12 years), and 76% were older than 12 years. One child had an elevated Lyme titer. Neuroimaging was performed in 14, and revealed 1 case each of pontine glioma, epidural abscess, and cholesteatoma. In this group, 17 (65%) of 26 were treated, 5 with oral corticosteroids, 10 with corticosteroids and acyclovir, and 2 with acyclovir alone. Treatment was started within 72 hours in 12 of the 17 children. The time to recovery was no different in the treated and untreated groups. The extent of recovery was not reported. Although the authors conclude that treatment is not useful in children with Bell's palsy, it would seem that this study is much too small to draw such a definitive conclusion (Thomas B, Cleveland, OH, P03013). A report described a family with 5 cases of hemifacial spasm in 4 successive generations. All were women, 3 with left and 2 with right spasms, in an apparently autosomal dominant pedigree with incomplete penetrance. Onset was in the sixth decade in 4 and in the fourth decade in 1. Among the 4 who underwent brain MRI, only 1 showed abnormal results (a tortuous basilar artery that did not appear to compress the facial nerve) (Nestrasil I, Olomomouc, Czech Republic, P03.057). INFECTIONS A report described the first case of extracranial involvement of the twelfth nerve and the recurrent laryngeal branch of the tenth nerve (ipsilateral paralysis of the vocal cord, soft palate, and tongue, or Tapia's syndrome) caused by cat scratch disease. In this patient, a cervical lymph node biopsy revealed chronic caseating granulomas with neutrophils. Oral erythromycin treatment was associated with partial recovery. Traditional causes of Tapia's syndrome include injuries of the high neck, complications during airway access, arterial dissection, infections or tumors of the parotid gland, and other neck tumors (Cardoso FM, Rio de Janeiro, Brazil, P01.115). A case report raised the issue of using corticosteroids in the treatment of Lyme disease. A woman with right peripheral facial palsy and a disseminated macular ring-like rash with positive Lyme IgM antibody and normal results on a CSF examination, did not improve after a 5-day course of ceftriaxone and doxycycline. With the addition of 80 mg of prednisone daily, the facial palsy and rash disappeared almost completely within 1 day (Ahmed SN, Elizabeth, NJ, P01.116). PRION DISEASES To assess the value of MRI in distinguishing Jakob- Creutzfeldt disease (CJD) from rapidly progressive dementia (RPD) of other causes, 90 subjects referred for CJD had brain MRI read by 2 neuroradiologists masked to the clinical diagnosis. All CJD patients had GM hyper-intensity (diffusion imaging > FLAIR) in neocortical, limbic, or subcortical regions but never in limbic regions alone. Apparent diffusion coefficient (ADC) maps con-firmed diffusion restriction in all subcortical regions only in patients with CJD. In the non-CJD group, the GM abnormalities were commonly limbic alone (FLAIR > diffusion imaging); none of the 10% of patients with subcortical hyperintensities had subcortical diffusion restriction on the ADC map. Sensitivity and specificity for CJD were 93.8% and 72.4% (reader 1) and 95.8% and 100% (reader 2), respectively. After consensus review, sensitivity and specificity for CJD were 97.9% and 100%. The pattern of FLAIR/diffusion imaging hyperintensity can differentiate CJD from other RPDs with high sensitivity and specificity, and the authors make the point that MRI should be included among the diagnostic criteria for CJD (Vitali P, Milano, Italy, IN2-2.005). Wen-Ying Wu-Chen, MD Temple University School of Medicine Mark L. Moster, MD Albert Einstein Medical Center and Wills Eye Institute Thomas Jefferson University School of Medicine Philadelphia, Pennsylvania 357 |
