Editors' Note
: Regarding "The contemporary spectrum of multiple sclerosis misdiagnosis: A multicenter study," Dalla Costa et al. express concerns about the interpretation of results because of the number of patients screened, the clinical and paraclinical characteristics of the misdiagnosed patients, and the lack of specification of fulfilled multiple sclerosis (MS) criteria through which the diagnosis was made. Solomon et al., authors of the study, explain that the study was not designed to assess the frequency of MS misdiagnosis or its specific causes. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Karam, C., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Recurrent belly dancer dyskinesia in pregnancy
A 37-year-old woman in her 34th week of pregnancy developed continual abdominal movements, which had complicated both her previous pregnancies (video at Neurology.org). Examination, routine bloodwork, and brain MRI were normal. Circumstances precluded prepartum thoracolumbar MRI; postpartum MRI was unrevealing. Clonazepam and levetiracetam suppressed the movements, which remitted postpartum. All babies were healthy. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Meyer, J. A., Desai, K. V., Geyer, H. L. Tags: Clinical neurology history, Clinical neurology examination, All Movement Disorders VIDEO NEUROIMAGES Source Type: research

Asymptomatic carotid stenosis: Medicine alone or combined with carotid revascularization
Two positive randomized trials established carotid endarterectomy (CEA) as a superior treatment to medical management alone for the treatment of asymptomatic carotid artery stenosis. However, advances in medical therapy have led to an active and spirited debate about the best treatment for asymptomatic carotid stenosis. The Carotid Revascularization and Medical Management for Asymptomatic Carotid Stenosis (CREST 2) trial aims to better define the best treatment for the average patient with severe asymptomatic carotid stenosis. Enrollment in the trial may be hampered by strong opinions on either side of the debate. It is im...
Source: Neurology - May 22, 2017 Category: Neurology Authors: Heck, D. V., Roubin, G. S., Rosenfield, K. G., Gray, W. A., White, C. J., Jovin, T. G., Matsumura, J. S., Lal, B. K., Katzen, B. T., Dabus, G., Jankowitz, B. T., Brott, T. G. Tags: Stroke prevention, Other cerebrovascular disease/ Stroke, All Cerebrovascular disease/Stroke VIEWS & amp;amp; REVIEWS Source Type: research

Body mass index and outcome after revascularization for symptomatic carotid artery stenosis
Objective: To determine whether the obesity paradox exists in patients who undergo carotid artery stenting (CAS) or carotid endarterectomy (CEA) for symptomatic carotid artery stenosis. Methods: We combined individual patient data from 2 randomized trials (Endarterectomy vs Angioplasty in Patients with Symptomatic Severe Carotid Stenosis and Stent-Protected Angioplasty vs Carotid Endarterectomy) and 3 centers in a third trial (International Carotid Stenting Study). Baseline body mass index (BMI) was available for 1,969 patients and classified into 4 groups:
Source: Neurology - May 22, 2017 Category: Neurology Authors: Volkers, E. J., Greving, J. P., Hendrikse, J., Algra, A., Kappelle, L. J., Becquemin, J.-P., Bonati, L. H., Brott, T. G., Bulbulia, R., Calvet, D., Eckstein, H.-H., Fraedrich, G., Gregson, J., Halliday, A., Howard, G., Jansen, O., Roubin, G. S., Brown, M. Tags: Stroke prevention, Clinical trials Systematic review/meta analysis, Risk factors in epidemiology, Infarction ARTICLE Source Type: research

Alcohol use and risk of intracerebral hemorrhage
Objective: To analyze the dose–risk relationship for alcohol consumption and intracerebral hemorrhage (ICH) in the Ethnic/Racial Variations of Intracerebral Hemorrhage (ERICH) study. Methods: ERICH is a multicenter, prospective, case-control study, designed to recruit 1,000 non-Hispanic white patients, 1,000 non-Hispanic black patients, and 1,000 Hispanic patients with ICH. Cases were matched 1:1 to ICH-free controls by age, sex, race/ethnicity, and geographic area. Comprehensive interviews included questions regarding alcohol consumption. Patterns of alcohol consumption were categorized as none, rare (2 drinks per day and
Source: Neurology - May 22, 2017 Category: Neurology Authors: Chen, C.-J., Brown, W. M., Moomaw, C. J., Langefeld, C. D., Osborne, J., Worrall, B. B., Woo, D., Koch, S., For the ERICH Investigators Tags: All Cerebrovascular disease/Stroke, Alcohol, Case control studies, Risk factors in epidemiology, Intracerebral hemorrhage ARTICLE Source Type: research

Relationship between carotid arterial properties and cerebral white matter hyperintensities
Conclusions: Large carotid artery diameters are associated with greater burden of white matter hyperintensity (WMH) in this multiethnic population. The association between increased diameters, decreased STRAIN, and greater WMH burden is more pronounced among Hispanics. These associations suggest a potential important pathophysiologic role of extracranial large artery remodeling in the burden of WMH. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Rundek, T., Della-Morte, D., Gardener, H., Dong, C., Markert, M. S., Gutierrez, J., Roberts, E., Elkind, M. S. V., DeCarli, C., Sacco, R. L., Wright, C. B. Tags: fMRI, Other cerebrovascular disease/ Stroke, Assessment of cognitive disorders/dementia ARTICLE Source Type: research

Effect of a long-term intensive lifestyle intervention on prevalence of cognitive impairment
Conclusions: Ten years of behavioral weight loss intervention did not result in an overall difference in the prevalence of cognitive impairment among overweight or obese adults with type 2 diabetes. Clinicaltrials.gov identifier: NCT00017953 (Action for Health in Diabetes). Level of evidence: This study provides Class II evidence that for overweight adults with type 2 diabetes, a lifestyle intervention designed to reduce weight and increase physical activity does not lower the risk of cognitive impairment. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Espeland, M. A., Luchsinger, J. A., Baker, L. D., Neiberg, R., Kahn, S. E., Arnold, S. E., Wing, R. R., Blackburn, G. L., Bray, G., Evans, M., Hazuda, H. P., Jeffery, R. W., Wilson, V. M., Clark, J. M., Coday, M., Demos-McDermott, K., Foreyt, J. P., Green Tags: All Clinical trials, All Cognitive Disorders/Dementia ARTICLE Source Type: research

Pregabalin use early in pregnancy and the risk of major congenital malformations
Conclusions: Findings did not confirm the suggested teratogenic effects of pregabalin, although they cannot rule out the possibility of a small effect. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Patorno, E., Bateman, B. T., Huybrechts, K. F., MacDonald, S. C., Cohen, J. M., Desai, R. J., Panchaud, A., Mogun, H., Pennell, P. B., Hernandez-Diaz, S. Tags: Neonatal, Cohort studies, Antiepileptic drugs ARTICLE Source Type: research

How small can the epileptogenic region be?: A case in point
Conclusions: This case demonstrates that small areas of cortex may be highly epileptogenic, drive intractable epilepsy, and disrupt large-scale networks likely to be involved in core cognitive functions. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Jackson, G. D., Pedersen, M., Harvey, A. S. Tags: Epilepsy surgery, Epileptogenic zone, Cortical localization, Functional neuroimaging, Cortical dysplasia ARTICLE Source Type: research

Hypothalamus as a mediator of chronic migraine: Evidence from high-resolution fMRI
Conclusions: Our data corroborate the fact that the hypothalamus plays a crucial role in the pathophysiology of migraine chronification and acute pain stage of migraineurs. While the more posterior part of the hypothalamus seems to be important for the acute pain stage, the more anterior part seems to play an important role in attack generation and migraine chronification. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Schulte, L. H., Allers, A., May, A. Tags: All Headache, Migraine, fMRI ARTICLE Source Type: research

Randomized controlled trial of deutetrabenazine for tardive dyskinesia: The ARM-TD study
Conclusions: In patients with TD, deutetrabenazine was well tolerated and significantly reduced abnormal movements. Classification of evidence: This study provides Class I evidence that in patients with TD, deutetrabenazine reduces AIMS scores. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Fernandez, H. H., Factor, S. A., Hauser, R. A., Jimenez-Shahed, J., Ondo, W. G., Jarskog, L. F., Meltzer, H. Y., Woods, S. W., Bega, D., LeDoux, M. S., Shprecher, D. R., Davis, C., Davis, M. D., Stamler, D., Anderson, K. E. Tags: Clinical trials Randomized controlled (CONSORT agreement), Class I ARTICLE Source Type: research

Vagotomy and Parkinson disease: A Swedish register-based matched-cohort study
Conclusions: Although overall vagotomy was not associated the risk of PD, we found suggestive evidence for a potential protective effect of truncal, but not selective, vagotomy against PD development. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Liu, B., Fang, F., Pedersen, N. L., Tillander, A., Ludvigsson, J. F., Ekbom, A., Svenningsson, P., Chen, H., Wirdefeldt, K. Tags: Parkinson's disease/Parkinsonism, Cohort studies ARTICLE Source Type: research

Lewis P. Rowland, MD (1925-2017)
Lewis P. Rowland, one of the most influential neurologists of our time, died on March 16 following a stroke. It was somehow fitting that his last days were in the neurologic intensive care unit at New York Presbyterian Hospital, one of the first in the country. Critical care neurology was a development that Bud had foreseen and strongly supported as essential for modern neurology and neurosurgery. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Pedley, T. A., Mayeux, R., Griggs, R. C. Tags: IN MEMORIAM Source Type: research

Asymptomatic carotid stenosis: Why a moratorium is needed on intervention outside clinical trials
In this issue of Neurology®, Heck et al.1 discuss the need for evidence regarding intervention for asymptomatic carotid stenosis (ACS). They say it is not proven that the risk of stroke in asymptomatic carotid stenosis is well below that of intervention. They inveigh against the suggestion that a moratorium on intervention in low-risk ACS outside of randomized trials be accepted in order to foster the development of the evidence we need to settle how to manage ACS. They assert further that only randomized trials can provide valid evidence. However, results in carefully vetted randomized trials do not reflect real-world...
Source: Neurology - May 22, 2017 Category: Neurology Authors: Spence, J. D. Tags: Stroke prevention, Other cerebrovascular disease/ Stroke, All Cerebrovascular disease/Stroke EDITORIALS Source Type: research

Optimal management of patients with asymptomatic carotid stenosis
Prior trials have shown that carotid endarterectomy (CEA) is superior to nonoperative management of asymptomatic carotid stenosis.1,2 More recent trials have shown surprisingly better outcomes for stroke patients managed with maximal medical therapy.3,4 As such, a number of experts have called for revisiting clinical trials of both CEA and carotid stenting for patients with symptomatic and asymptomatic carotid stenosis using modern medical therapy. Given the current substantial equipoise within the field, modern studies seem reasonable. A number of ongoing trials aim to address current areas of equipoise, but enrollment in...
Source: Neurology - May 22, 2017 Category: Neurology Authors: Starke, R. M. Tags: Stroke prevention, Other cerebrovascular disease/ Stroke, All Cerebrovascular disease/Stroke EDITORIALS Source Type: research

Nothing like a spirited debate!
In this issue of Neurology®, 3 opinion pieces provide differing views on the landscape around the management of asymptomatic carotid disease and raise a number of thorny issues.1–3 Heck et al.1 offer an overview; Spence2 and Starke3 offer editorial perspectives. This note introduces the topic as context for those articles. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Worrall, B. B. Tags: EDITOR ' S NOTE Source Type: research

Intensive intervention and cognitive impairment: Are lifestyle changes enough for a good brain?
The Look AHEAD (Action for Health in Diabetes) study examined how a lifestyle intervention (diet and exercise), compared to diabetes support and education, may help to improve function in a population at high risk for cognitive decline—people with excess weight or obesity and type 2 diabetes.1 Lifestyle is a complex construct that includes behaviors such as cognitive and social engagement as well as diet choices and exercise. There is no clear consensus regarding the nature of the ideal choices and who gets to benefit by adhering. Much data regarding lifestyle come from observational studies, which by design cannot a...
Source: Neurology - May 22, 2017 Category: Neurology Authors: Camicioli, R., Driscoll, I. Tags: All Clinical trials, All Cognitive Disorders/Dementia EDITORIALS Source Type: research

Preventing Parkinson disease by vagotomy: Fact or fiction?
Aggregation of the neuronal protein α-synuclein into insoluble filamentous inclusions may be a determining factor in the development of Parkinson disease (PD). Converging evidence has demonstrated that α-synuclein inclusions have prion-like properties, including cell-to-cell transmission and the ability to induce native α-synuclein to misfold, thus setting the stage for domino-like spreading of cellular pathology.1 (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Borghammer, P., Hamani, C. Tags: Parkinson's disease/Parkinsonism, Cohort studies EDITORIALS Source Type: research

Spotlight on the May 23 issue
(Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Teaching NeuroImages: Intraspinal synovial cyst causing Brown-Sequard syndrome: Uncommon cause of a classic syndrome
A 65-year-old woman with osteoarthritis presented with 6 weeks of insidiously worsening numbness in the left hemibody and weakness in the right arm and leg. Examination revealed pyramidal weakness in the right arm and leg with hyperreflexia, right Babinski sign, left-sided C4 sensory level to pinprick and temperature, and reduced proprioception in the right extremities. Cervical spine MRI revealed a facet joint synovial cyst at C2-C3 compressing the right hemicord (figure). This was excised, with only mild numbness at 6 months postoperatively. Symptomatic intraspinal cervical synovial cysts are rare and an uncommon cause o...
Source: Neurology - May 22, 2017 Category: Neurology Authors: Beecher, G., Kotylak, T., Johnston, W. S. Tags: MRI, Clinical neurology history, Clinical neurology examination, All Spinal Cord RESIDENT AND FELLOW SECTION Source Type: research

Child Neurology: LAMA2 muscular dystrophy without contractures
We present an interesting case with a novel mutation in the laminin α2 (LAMA2) gene, which has not been reported previously; the patient has a mixed clinical phenotype without contractures. (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Dean, M., Rashid, S., Kupsky, W., Moore, S. A., Jiang, H. Tags: All Neuromuscular Disease, Muscle disease, All Pediatric RESIDENT AND FELLOW SECTION Source Type: research

Godless State
"Jonesy," I say. "It's Dr. Braunstein. He's here to see you." (Source: Neurology)
Source: Neurology - May 22, 2017 Category: Neurology Authors: Warnack, W. R. Tags: REFLECTIONS: NEUROLOGY AND THE HUMANITIES Source Type: research

Mystery Case: CSF-1R mutation is a cause of intracranial cerebral calcifications, cysts, and leukoencephalopathy
(Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research

Summary author response: Mystery Case: CSF-1R mutation is a cause of intracranial cerebral calcifications, cysts, and leukoencephalopathy
I thank Dr. Lynch and Prof. Houlden for the response to my editorial summary accompaniment to the Mystery Case,1 and for highlighting the distinction between CSF-1R-related disease and Labrune syndrome for both myself and our readers. This is a helpful learning point I hope to carry forward. (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Ganesh, A. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Mystery Case: CSF-1R mutation is a cause of intracranial cerebral calcifications, cysts, and leukoencephalopathy
I thank Dr. Lynch and Prof. Houlden for the important comment on our Mystery Case.1 Indeed, calcifications and cysts found in patients with SNORD118 mutations, formerly described in Labrune syndrome, are larger than those seen in patients harboring CSF-1R mutations. Nevertheless, the importance of calcifications in this disorder was recently emphasized.2 Notably, calcifications are small and may have a particular stepping-stone appearance in the frontal pericallosal regions.2 (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Ayrignac, X. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Mystery Case: CSF-1R mutation is a cause of intracranial cerebral calcifications, cysts, and leukoencephalopathy
Ayrignac et al.1 presented an interesting case that highlighted the importance of CSF-1R in adult-onset leukoencephalopathies. However, we are concerned that the discussion by Dr. Ganesh in the Mystery Case Responses section confused 2 different diseases as the same.1 This case clearly described a typical presentation of CSF-1R-related disease with apparent autosomal dominant inheritance. However, Dr. Ganesh described this as a case of Labrune syndrome (leukoencephalopathy with calcifications and cysts), an autosomal recessive disorder caused by mutations in SNORD118.2 While calcifications occur in both syndromes, they are...
Source: Neurology - May 15, 2017 Category: Neurology Authors: Lynch, D. S., Houlden, H. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In WriteClick this week, Drs. Lynch and Houlden point out an error in the Mystery Case Responses section of "Mystery Case: CSF-1R mutation is a cause of intracranial cerebral calcifications, cysts, and leukoencephalopathy," in which CSF-1R-related leukoencephalopathy is mistaken to be the same as Labrune syndrome. (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Alcauskas, M., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Pure sensory ganglionopathy as the first sign of relapse in non-Hodgkin lymphoma
A 34-year-old man with diffuse large B-cell lymphoma in complete remission presented with painful paresthesias of the thorax and proximal legs with loss of patellar reflexes bilaterally. Laboratory studies showed no evidence of autoimmune or infectious processes. CSF cytology was unremarkable. MRI showed enlarged, enhancing dorsal root ganglia (DRG) at L3-L4 bilaterally (figure, A). Whole-body PET showed avidity in these nerve roots and in T2 and T8 (figure, C). Abnormalities on nerve conduction studies were isolated to sensory nerves affecting the same roots. Sensory ganglionopathies can occur with lymphomatous infiltrati...
Source: Neurology - May 15, 2017 Category: Neurology Authors: Hossack, M., McClean, J. Tags: All Imaging, All Clinical Neurology, All Oncology, All clinical neurophysiology NEUROIMAGES Source Type: research

Reprogramming cells from Gulf War veterans into neurons to study Gulf War illness
Gulf War illness (GWI), which afflicts at least 25% of veterans who served in the 1990–1991 war in the Persian Gulf, is thought to be caused by deployment exposures to various neurotoxicants, including pesticides, anti–nerve gas pills, and low-level nerve agents including sarin/cyclosarin. GWI is a multisymptom disorder characterized by fatigue, joint pain, cognitive problems, and gastrointestinal complaints. The most prominent symptoms of GWI (memory problems, poor attention/concentration, chronic headaches, mood alterations, and impaired sleep) suggest that the disease primarily affects the CNS. Development o...
Source: Neurology - May 15, 2017 Category: Neurology Authors: Qiang, L., Rao, A. N., Mostoslavsky, G., James, M. F., Comfort, N., Sullivan, K., Baas, P. W. Tags: Other toxicology, Bioterrorism MEDICAL HYPOTHESIS Source Type: research

Pharmacotherapy for diabetic peripheral neuropathy pain and quality of life: A systematic review
Conclusions: For reducing pain, duloxetine and venlafaxine, pregabalin and oxcarbazepine, tricyclic antidepressants, atypical opioids, and botulinum toxin were more effective than placebo. However, quality of life was poorly reported, studies were short-term, drugs had substantial dropout rates, and opioids have significant risks. Future studies should evaluate longer-term outcomes, use methods and measures recommended by pain organizations, and assess patients' quality of life. (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Waldfogel, J. M., Nesbit, S. A., Dy, S. M., Sharma, R., Zhang, A., Wilson, L. M., Bennett, W. L., Yeh, H.-C., Chelladurai, Y., Feldman, D., Robinson, K. A. Tags: Quality of life, Peripheral neuropathy, Clinical trials Systematic review/meta analysis, All Pain, Neuropathic pain VIEWS & amp;amp; REVIEWS Source Type: research

Quality improvement in neurology: Dementia management quality measurement set update
Dementia is a neurologic condition manifested by a substantial decline in multiple cognitive abilities that collectively render a person unable to function at expected levels and progressively impede independent ability to perform everyday activities. For decades, public health officials have warned of the coming tsunami of Alzheimer disease (AD), and dementia has even been characterized as the dominant scourge of modern times, replacing cancer.1 Recently, hopeful signs have appeared, including reports from some longitudinal research studies that incidence of dementia is declining,2 and from the federal government that res...
Source: Neurology - May 15, 2017 Category: Neurology Authors: Sanders, A. E., Nininger, J., Absher, J., Bennett, A., Shugarman, S., Roca, R. Tags: All Health Services Research, All Cognitive Disorders/Dementia, All Practice Management SPECIAL ARTICLE Source Type: research

Nrf2, cellular redox regulation, and neurologic implications
Nuclear factor erythroid 2 p45-related factor 2 (Nrf2), encoded by the NFE2L2 gene, is a major regulator of cellular homeostasis. Nrf2 is a transcription factor that promotes the production of components of antioxidant systems, including the glutathione and thiol systems, enzymes of pathways that generate nicotinamide adenine dinucleotide phosphate, and proteins involved in iron metabolism, xenobiotic detoxification, proteostasis, and lipogenesis. Nrf2 protects mitochondrial function and promotes clearance of misfolded proteins, and thus prevents initiation of cell death programs. The regulation and effects of Nrf2 signali...
Source: Neurology - May 15, 2017 Category: Neurology Authors: Benarroch, E. E. Tags: CLINICAL IMPLICATIONS OF NEUROSCIENCE RESEARCH Source Type: research

Significance of circadian rhythms in severely brain-injured patients: A clue to consciousness?
Conclusion: The study provides evidence for an association between circadian body temperature rhythms and arousal as a necessary precondition for consciousness. Our findings also make a case for circadian rhythms as a target for treatment as well as the application of diagnostic and therapeutic means at times when cognitive performance is expected to peak. (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Blume, C., Lechinger, J., Santhi, N., Giudice, R. d., Gnjezda, M.-T., Pichler, G., Scarpatetti, M., Donis, J., Michitsch, G., Schabus, M. Tags: Neuropsychological assessment, Attention, Brain trauma ARTICLE Source Type: research

Trends in dementia prevalence, incidence, and survival rate in a Japanese community
Objective: To investigate secular trends in the prevalence, incidence, and survival rate of dementia in a Japanese elderly population in a comprehensive manner. Methods: Five cross-sectional surveys of dementia were conducted among residents of a Japanese community, aged ≥65 years, in 1985, 1992, 1998, 2005, and 2012. We also established 2 cohorts consisting of the residents of this age group without dementia in 1988 (n = 803) and 2002 (n = 1,231), and each was followed for 10 years. Results: The age-standardized prevalence of all-cause dementia and Alzheimer disease (AD) increased with time (for all-cause dementia: 6...
Source: Neurology - May 15, 2017 Category: Neurology Authors: Ohara, T., Hata, J., Yoshida, D., Mukai, N., Nagata, M., Iwaki, T., Kitazono, T., Kanba, S., Kiyohara, Y., Ninomiya, T. Tags: All Cognitive Disorders/Dementia, Alzheimer's disease, Vascular dementia, Prevalence studies, Incidence studies ARTICLE Source Type: research

Phenotype of GABA-transaminase deficiency
Conclusions: GABA-transaminase deficiency presents with neonatal or infantile-onset encephalopathy including hypersomnolence and choreoathetosis. A widened phenotypic spectrum is reported as opposed to lethality by 2 years of age. The GABA-A benzodiazepine receptor antagonist flumazenil may represent a therapeutic strategy. (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Koenig, M. K., Hodgeman, R., Riviello, J. J., Chung, W., Bain, J., Chiriboga, C. A., Ichikawa, K., Osaka, H., Tsuji, M., Gibson, K. M., Bonnen, P. E., Pearl, P. L. Tags: Metabolic disease (inherited), Chorea, All Epilepsy/Seizures ARTICLE Source Type: research

Certainty of genuine treatment increases drug responses among intellectually disabled patients
Conclusions: Our data suggest that clinical trials in patients with severe cognitive deficits are influenced by the certainty of receiving genuine medication, and open-label design should thus not be used to evaluate the effect of pharmacologic treatments in ID, as the results will be biased by an enhanced placebo component. (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Jensen, K. B., Kirsch, I., Ponten, M., Rosen, A., Yang, K., Gollub, R. L., des Portes, V., Kaptchuk, T. J., Curie, A. Tags: Intelligence, Executive function, Clinical trials Systematic review/meta analysis, Developmental disorders, Mental retardation ARTICLE Source Type: research

Risks and benefits of clopidogrel-aspirin in minor stroke or TIA: Time course analysis of CHANCE
Conclusions: Clopidogrel–aspirin treatment may have a benefit of reducing stroke risk outweighing the potential risk of increased bleeding especially within the first 2 weeks compared with aspirin alone in patients with minor stroke or TIA. Clinicaltrials.gov identifier: NCT00979589. Classification of evidence: This study provides Class II evidence that for patients with minor stroke or TIA, the reduction of stroke risk from clopidogrel plus aspirin within the first 2 weeks outweighs the risk of bleeding compared with aspirin alone. (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Pan, Y., Jing, J., Chen, W., Meng, X., Li, H., Zhao, X., Liu, L., Wang, D., Johnston, S. C., Wang, Y., Wang, Y., On behalf of the CHANCE investigators Tags: All Cerebrovascular disease/Stroke, Clinical trials Randomized controlled (CONSORT agreement) ARTICLE Source Type: research

Copeptin and NT-proBNP for prediction of all-cause and cardiovascular death in ischemic stroke
Conclusions: Copeptin and NT-proBNP may be useful independent prognostic markers of all-cause or CVD mortality in Chinese patients with AIS. (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Tu, W.-J., Ma, G.-Z., Ni, Y., Hu, X.-S., Luo, D.-Z., Zeng, X.-W., Liu, Q., Xu, T., Yu, L., Wu, B. Tags: Prognosis, All Cerebrovascular disease/Stroke, Cardiac ARTICLE Source Type: research

Diagnostic value of prehospital ECG in acute stroke patients
Conclusions: In a selected cohort of patients with stroke, the in-field recordings of the ECG detected a relevant rate of cardiac arrhythmia. The results can add to the in-hospital evaluation and should be considered in prehospital care of acute stroke. (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Bobinger, T., Kallmünzer, B., Kopp, M., Kurka, N., Arnold, M., Heider, S., Schwab, S., Köhrmann, M. Tags: All Cerebrovascular disease/Stroke, Cardiac, Critical care ARTICLE Source Type: research

Anticoagulant and antiplatelet use in seniors with chronic subdural hematoma: Systematic review
Conclusions: Anticoagulant medication was associated with increased rebleeding risk in older adults with cSDH. However, antiplatelet medication was not associated with increased risk of rebleeding. (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Nathan, S., Goodarzi, Z., Jette, N., Gallagher, C., Holroyd-Leduc, J. Tags: Brain trauma, Patient safety ARTICLE Source Type: research

Outcomes after intracerebral hemorrhage from arteriovenous malformations
Conclusions: Patients with ICH due to ruptured AVM have more favorable outcomes than patients with ICH from other causes. (Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Murthy, S. B., Merkler, A. E., Omran, S. S., Gialdini, G., Gusdon, A., Hartley, B., Roh, D., Mangat, H. S., Iadecola, C., Navi, B. B., Kamel, H. Tags: Arteriovenous malformation, Intracerebral hemorrhage ARTICLE Source Type: research

Thromboembolism prevention after chronic subdural hematoma in the elderly: A leap in the dark
The incidence of chronic subdural hematomas (cSDH) increases with age.1,2 Risk factors that contribute to the occurrence of cSDH, apart from advancing age, include a history of falls, minor head injury, use of anticoagulant or antiplatelet medications, bleeding disorders, heavy alcohol use, epilepsy, low intracranial pressure conditions, and hemodialysis.1 The aged population also endures the highest risk of atrial fibrillation and other conditions that predispose them to devastating ischemic cardiac and cerebral ischemic events.3 Antithrombotic therapy constitutes evidence-based therapy to prevent these ischemic events, b...
Source: Neurology - May 15, 2017 Category: Neurology Authors: Suarez, J. I., Kapinos, G. Tags: Brain trauma, Patient safety EDITORIALS Source Type: research

Favorable outcome in patients with intracranial hemorrhage due to ruptured brain AVM
Despite all efforts, intracranial hemorrhage still has a dismal prognosis, with up to 61% mortality after 2 years.1 Several authors have concluded that intracranial hemorrhage due to a brain arteriovenous malformation (AVM) has a better clinical outcome.1–3 Only one study specifically addressed this issue and used a relatively small sample, comparing the outcome of 90 patients with a ruptured AVM from the Scottish Intracranial Vascular Malformation Study to the outcome of 60 patients with a spontaneous intracranial bleeding included in the Oxford Vascular Study.1 Those investigators found better outcomes after AVM-re...
Source: Neurology - May 15, 2017 Category: Neurology Authors: Buis, D. R. Tags: Arteriovenous malformation, Intracerebral hemorrhage EDITORIALS Source Type: research

Spotlight on the May 16 issue
(Source: Neurology)
Source: Neurology - May 15, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Teaching NeuroImages: Prominent spinal cord atrophy and white matter changes in adult polyglucosan body disease
A 48-year-old man of Ashkenazi Jewish descent developed neurogenic bladder and mild cognitive dysfunction. Neurologic examination and CSF analysis were unremarkable. Brain MRI demonstrated white matter changes in the internal capsule, optic radiations, mesencephalon, superior cerebellar peduncles, pons, and medulla (figure 1) and prominent medullary and spinal cord atrophy (figure 2). These findings are classically found in adult polyglucosan body disease (APBD).1 Glycogen branching enzyme GBE1 gene sequencing revealed that the patient was compound heterozygous (c.691+2T>C and c.986 A>C). APBD should be considered in...
Source: Neurology - May 15, 2017 Category: Neurology Authors: Lopez Chiriboga, A. S. Tags: Leukodystrophies, Muscle disease, Multiple sclerosis RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Takayasu arteritis: Neuroimaging progression after immunosuppressant treatment
A 29-year-old woman presented with a 2-year history of heel pain, constitutional symptoms, and increased acute phase reactants. CT and magnetic resonance (MR) angiography revealed a thickening of aortic walls and a thread-like appearance of bilateral subclavian and common carotid arteries. The findings were consistent with Takayasu arteritis (TA)1 and the patient was prescribed methylprednisolone, followed by azathioprine. Follow-up MR angiography, 6 years later, showed an important improvement with only a mild luminal narrowing of both common carotid arteries (figures 1 and 2, A and B). TA is a large-artery inflammatory d...
Source: Neurology - May 15, 2017 Category: Neurology Authors: Martinez Rodriguez, L., Caminal Montero, L., Pena Suarez, J., Garcia-Cabo Fernandez, C., Calleja Puerta, S. Tags: MRI, Vasculitis, All Cerebrovascular disease/Stroke RESIDENT AND FELLOW SECTION Source Type: research

Author response: Gray matter MRI differentiates neuromyelitis optica from multiple sclerosis using random forest
We thank Dr. Avasarala for the comment on our article.1 Since the majority of patients with neuromyelitis optica (NMO) were positive for anti-aquaporin-4 autoantibody, the classification results (table 2)1 are expected to remain similar after excluding seronegative patients (negative predictive value 76%). This is expected as patients with multiple sclerosis (MS) and NMO are heterogeneous in clinical and MRI assessments, and volumes in different brain regions may overlap (figure 1).1 Including other features, such as spinal cord atrophy, may increase the accuracy.2 (Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Authors: Eshaghi, A., Ciccarelli, O. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Gray matter MRI differentiates neuromyelitis optica from multiple sclerosis using random forest
The article by Eshaghi et al.1 gave a glimpse of where neuroradiology is headed. Pertinent questions include the following: (Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Authors: Avasarala, J. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Acute Zika infection with concurrent onset of Guillain-BarrE syndrome
We thank Gérardin et al. for the comments on our Clinical/Scientific Note on Zika virus (ZIKV).1 Our patient was of particular interest because he had an illness clinically and electrophysiologically indistinguishable from Guillain-Barré syndrome (GBS) that developed within a few days of acute ZIKV infection, in the context of contemporaneous Zika viremia.1 Concurrently obtained CSF was negative for ZIKV. Brain and spine MRI were also normal. Importantly, there was no serologic evidence of priming by previous dengue or other flaviviruses. (Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Authors: Timmings, P. L., Siu, R., Bukhari, W., Todd, A., Gunn, W. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Acute Zika infection with concurrent onset of Guillain-BarrE syndrome
Siu et al.1 reported the concurrent onset of polyradiculoneuritis and acute Zika virus (ZIKV) infection, while the virus was not cleared from the serum. Guillain-Barré syndrome (GBS) is usually described as a postinfectious disease during which progressive flaccid paralysis develops after a phase of latency following infection. In the most common pathogenetic framework, this free interval permits the generation of sufficient levels of antibodies that cross-react by molecular mimicry with specific components of peripheral nerves, causing myelin or axonal injury, as previously documented with ZIKV infection.2 However,...
Source: Neurology - May 8, 2017 Category: Neurology Authors: Gerardin, P., Cao-Lormeau, V.-M., Tournebize, P., Cerny, T. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research