Neurology October 2012 Volume 79 Issue 18

Objective: This study aims to evaluate protective effects of brief repetitive bilateral arm ischemic preconditioning (BAIPC) on stroke recurrence in patients with symptomatic atherosclerotic intracranial arterial stenosis (IAS).Methods: A total of 68 consecutive cases with symptomatic IAS, diagnosed by imaging, were enrolled in this prospective and randomized study. All patients received standard medical management. Patients in the BAIPC group (n = 38) underwent 5 brief cycles consisting of bilateral upper limb ischemia followed by reperfusion. The BAIPC procedure was performed twice daily over 300 consecutive days. Incidence of recurrent stroke and cerebral perfusion status in BAIPC-treated patients were compared with the untreated control group (n = 30).Results: In the control group, incidence of recurrent stroke at 90 and 300 days were 23.3% and 26.7%, respectively. In the BAIPC group, incidence of recurrent stroke was reduced to 5% and 7.9% at 90 and 300 days (p < 0.01), respectively. The average time to recovery (modified Rankin Scale score 0-1) was also shortened by BAIPC. Cerebral perfusion status, measured by SPECT and transcranial Doppler sonography, improved remarkably in BAIPC-treated brain than in control (p < 0.01).Conclusion: This study provides a proof-of-concept that BAIPC may be an effective way to improve cerebral perfusion and reduce recurrent strokes in patients with IAS. Further investigation of this therapeutic approach is warranted as some patients were excluded after randomization.GLOSSARY: BAIPC: bilateral arm ischemic preconditioningCTA: CT angiographyDWI: diffusion-weighted imaging18F-FDG: fluorodeoxyglucoseIAS: intracranial arterial stenosisMRA: magnetic resonance angiographymRS: modified Rankin ScaleNIHSS: NIH Stroke ScalePSV: peak systolic velocity99mTc-ECD: Technetium-99m ethylene cysteine dimerTCD: transcranial Doppler sonographyTOAST: Trial of Org 10172 in Acute Stroke Treatment(C)2012 American Academy of Neurology

Objective: The primary objective of this study was to explore the potential influence of gender on recovery from optic neuritis (ON) by determining whether differences in retinal nerve fiber layer (RNFL) thickness can be detected between men and women 6 months after an ON event.Methods: In this prospective cohort study, 39 men and 105 women with acute ON underwent repeat visual and optical coherence tomography (OCT) testing. The main outcome measures were change in RNFL measurements for male and female patients 6 months after ON.Results: Men were older (mean age = 39 years) than women (35 years) (p = 0.05) in this study, and more men (62%) than women (41%) had a diagnosis of relapsing-remitting multiple sclerosis (MS) (p = 0.02). Because age and MS subtype were 2 significant covariates, both variables were controlled for in multiple regression analyses. Other covariates controlled for in the multivariate regression included disease duration (years), use of disease-modifying therapy (yes/no), and use of high-dose corticosteroids for acute ON (yes/no). After 6 months, mean RNFL values were lower in men (74 [mu]m) than women (91 [mu]m) (p < 0.001). Men showed more apparent change in RNFL thickness in their ON eyes from baseline to 6 months after ON than women (p = 0.003).Conclusions: There may be differences in recovery between men and women after ON, which can be difficult to detect with conventional visual testing. Our findings raise interesting questions about the potential influence of gender in MS, which may be explored in future studies.GLOSSARY: CIS: clinically isolated syndromeDMT: disease-modifying therapylogMAR: logarithm of the minimum angle of resolutionMS: multiple sclerosisOCT: optical coherence tomographyON: optic neuritisRNFL: retinal nerve fiber layerRRMS: relapsing-remitting multiple sclerosisVA: visual acuityVF: visual field(C)2012 American Academy of Neurology

Objectives: To conduct a population-based study describing school performance in children with episodic migraine (EM), chronic migraine (CM), and probable migraine (PM), relative to controls.Methods: Children (n = 5,671) from 87 cities and 18 Brazilian states were interviewed by their teachers (n = 124). First, teachers were asked to provide information on the performance of the students while at school, which consisted of the same information provided to the educational board, with measurements of the overall achievement of competencies for the school year. The MTA-SNAP-IV scale was then used to capture symptoms of attention-deficit/hyperactivity disorder, and to provide objective information on the performance of the students. Parents were interviewed using a validated headache questionnaire and the Strengths and Difficulties Questionnaire, which measures behavior in 5 domains. Multivariate models estimated determinants of school performance as a function of headache status.Results: EM occurred in 9% of the children, PM in 17.6%, and CM in 0.6%. Poor performance at school was significantly more likely in children with EM and CM, relative to children without headaches, and was significantly influenced by severity (p < 0.001) and duration (p < 0.001) of headache attacks, by abnormal scores of mental health (p < 0.001), and by nausea (p < 0.001), as well as by headache frequency, use of analgesics, and gender.Conclusion: Children with migraine are at an increased risk of having impairments in their school performance and factors associated with impairment have been mapped. Future studies should address the directionality of the association and putative mechanisms to explain it.GLOSSARY: CI: confidence intervalCM: chronic migraineDSM-IV: Diagnostic and Statistical Manual of Mental Disorders, 4th editionEM: episodic migraineETTH: episodic tension-type headacheICHD-2: Second Edition of the International Classification of Headache DisordersPM: probable migraineRR: relative riskSDQ: Strengths and Difficulties Questionnaire(C)2012 American Academy of Neurology

Objective: Fragile X-associated tremor ataxia syndrome (FXTAS) is defined by FMR1 premutation, cerebellar ataxia, intentional tremor, and middle cerebellar peduncle (MCP) hyperintensities. We delineate the clinical, neurophysiologic, and morphologic characteristics of FXTAS.Methods: Clinical, morphologic (brain MRI, 123I-ioflupane SPECT), and neurophysiologic (tremor recording, nerve conduction studies) study in 22 patients with FXTAS, including 4 women.Results: A total of 43% of patients had no family history of fragile X syndrome (FXS), which contrasts with previous FXTAS series. A total of 86% of patients had tremor and 81% peripheral neuropathy. We identified 3 electroclinical tremor patterns: essential-like (35%), cerebellar (29%), and parkinsonian (12%). Two electrophysiologic patterns evocative of non-length-dependent (56%) and length-dependent sensory neuropathy (25%) were identified. Corpus callosum splenium (CCS) hyperintensity was as frequent (68%) as MCP hyperintensities (64%). Sixty percent of patients had parkinsonism and 47% abnormal 123I-ioflupane SPECT. Unified Parkinson's Disease Rating Scale motor score was correlated to abnormal 123I-ioflupane SPECT (p = 0.02) and to CGG repeat number (p = 0.0004). Scale for the assessment and rating of ataxia correlated with dentate nuclei hyperintensities (p = 0.03) and CCS hyperintensity was a marker of severe disease progression (p = 0.04).Conclusions: We recommend to include in the FXTAS testing guidelines both CCS hyperintensity and peripheral neuropathy and to consider them as new major radiologic and minor clinical criterion, respectively, for the diagnosis of FXTAS. FXTAS should also be considered in women or when tremor, MCP hyperintensities, or family history of FXS are lacking. Our study broadens the spectrum of tremor, peripheral neuropathy, and MRI abnormalities in FXTAS, hence revealing the need for revised criteria.GLOSSARY: Acc: accelerometerCCS: corpus callosum spleniumCMAP: compound muscle action potentialDD: disease durationDL: distal latencyDRG: dorsal root gangliaET: essential tremorF-WL: F-wave latencyFAB: Frontal Assessment BatteryFLAIR: fluid-attenuated inversion recoveryFTMa: Fahn-Tolosa-Marin adapted tremor rating scale, part AFXS: fragile X syndromeFXTAS: fragile X-associated tremor ataxia syndromeLL: lower limbMCP: middle cerebellar peduncleMMSE: Mini-Mental State ExaminationMNCV: motor nerve conduction velocityNCS: nerve conduction studyPD: Parkinson diseasePOI: primary ovarian insufficiencySARA: Scale for the Assessment and Rating of AtaxiaSNAP: sensory nerve action potentialSNN: sensory neuronopathyUL: upper limbUPDRS-III: Unified Parkinson's Disease Rating Scale motor(C)2012 American Academy of Neurology

: Diffuse gliomas are a heterogenous group of neoplasms traditionally classified as grades II to IV based on histologic features, and with prognosis determined mainly by histologic grade and pretreatment clinical factors. Our understanding of the molecular basis of glioma initiation, tumor progression, and treatment failure is rapidly evolving. A molecular profile of diffuse gliomas is emerging. Studies evaluating gene expression and DNA methylation profile have found multiple glioma subtypes and an association between subtype and survival. The recent discovery of isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) mutations in glioma has provided reproducible prognostic biomarkers and novel therapeutic targets. Glioblastomas that exhibit CpG island hypermethylator phenotype, proneural gene expression, or IDH1 mutation identify a subset of patients with markedly improved prognosis. Accumulated evidence supports the stratification of both low-grade and anaplastic diffuse gliomas into prognostic groups using 1p/19q codeletion and IDH mutation status. A classification scheme incorporating clinical, pathologic, and molecular information may facilitate improved prognostication for patients treated in the clinic, the development of more effective clinical trials, and rational testing of targeted therapeutics.GLOSSARY: AA : anaplastic astrocytomaAG : anaplastic gliomaAML : acute myelogenous leukemiaAO : anaplastic oligodendrogliomaAOA : anaplastic mixed oligoastrocytomaEGFR : epidermal growth factor receptorEORTC : European Organisation for Research and Treatment of CancerG-CIMP : CpG island hypermethylator phenotypeGB : glioblastomaGBO : glioblastoma with oligodendroglial featuresGSC : glioma stem cellIHC : immunohistochemicalLGA : low-grade astrocytomaLGG : low-grade gliomaLGO : low-grade oligodendrogliomaLGOA: low-grade mixed oligoastrocytomaLOH : loss of heterozygosityPDGFR : platelet-derived growth factor receptorRb : retinoblastomaRPA : recursive partitioning analysisRTOG : Radiation Therapy Oncology GroupTCGA : the Cancer Genome Atlas(C)2012 American Academy of Neurology