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Editors' Note
: In response to "Acute Zika infection with concurrent onset of Guillain-Barré syndrome," Dr. Gérardin et al. suggest that Zika-related Guillain-Barré syndrome (GBS) may have a different mechanism than postviral GBS. (Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

If brains could talk
Rows of shelved brains Shuddered in blind horror as One was taken down for dissection. (Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Authors: Burton, R. A. Tags: REFLECTIONS: NEUROLOGY AND THE HUMANITIES Source Type: research

L-Dopa in dystonia: A modern perspective
"Every child exhibiting dystonia merits an l-dopa trial, lest the potentially treatable condition of dopa-responsive dystonia (DRD) is missed" has been a commonly cited and highly conserved adage in movement disorders literature stemming from the 1980s. We here provide a historical perspective on this statement, discuss the current diagnostic and therapeutic applications of l-dopa in everyday neurologic practice, contrast these with its approved indications, and finish with our view on both a diagnostic and therapeutic trial in children and adults with dystonia. In light of the relatively low prevalence of DRDs, ...
Source: Neurology - May 8, 2017 Category: Neurology Authors: Maas, R. P. P. W. M., Wassenberg, T., Lin, J.-P., van de Warrenburg, B. P. C., Willemsen, M. A. A. P. Tags: Dystonia, All Pediatric, All Genetics VIEWS & amp;amp; REVIEWS Source Type: research

Visual discrimination training improves Humphrey perimetry in chronic cortically induced blindness
Conclusions: In adults with chronic cortical visual impairment, the blind field border appears to have enhanced plastic potential, which can be recruited by gaze-controlled visual discrimination training to expand the visible field. Our findings underscore a critical need for future studies to measure the effects of vision restoration approaches on perimetry in larger cohorts of patients. (Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Authors: Cavanaugh, M. R., Huxlin, K. R. Tags: Visual loss, Visual fields, Visual processing, All Cerebrovascular disease/Stroke, All Rehabilitation ARTICLE Source Type: research

Probable REM sleep behavior disorder and risk of stroke: A prospective study
Conclusions: Presence of pRBD was associated with a higher risk of developing stroke, including both ischemic and hemorrhagic types. Future studies with clinically confirmed RBD and a longer follow-up would be appropriate to further investigate this association. (Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Authors: Ma, C., Pavlova, M., Liu, Y., Liu, Y., Huangfu, C., Wu, S., Gao, X. Tags: All Cerebrovascular disease/Stroke, All Sleep Disorders, Cohort studies ARTICLE Source Type: research

Racial differences in statin adherence following hospital discharge for ischemic stroke
Objective: To compare nonadherence to statins in older black and white adults following an ischemic stroke. Methods: We studied black and white adults ≥66 years of age with Medicare fee-for-service insurance coverage hospitalized for ischemic stroke from 2007 to 2012 who filled a statin prescription within 30 days following discharge. Nonadherence was defined as a proportion of days covered
Source: Neurology - May 8, 2017 Category: Neurology Authors: Albright, K. C., Zhao, H., Blackburn, J., Limdi, N. A., Beasley, T. M., Howard, G., Bittner, V., Howard, V. J., Muntner, P. Tags: All Cerebrovascular disease/Stroke ARTICLE Source Type: research

Meta-analysis of folic acid efficacy trials in stroke prevention: Insight into effect modifiers
Objective: To examine the efficacy and effect modifiers of folic acid supplementation in the prevention of stroke in regions without folic acid fortification based on relevant, up-to-date published randomized trials. Methods: Relative risk (RR) was used to measure the effect of folic acid supplementation on risk of stroke using a fixed effects model. Findings: Overall, folic acid supplementation significantly reduced the stroke risk by 11% (22 trials, n = 82,723; RR 0.89, 95% confidence interval [CI] 0.84–0.96). The effect was greater in low folate regions (2 trials, n = 24,020; Asia, 0.78, 0.67–0.90) compare...
Source: Neurology - May 8, 2017 Category: Neurology Authors: Zhao, M., Wu, G., Li, Y., Wang, X., Hou, F. F., Xu, X., Qin, X., Cai, Y. Tags: Stroke prevention, All Cerebrovascular disease/Stroke, Clinical trials Randomized controlled (CONSORT agreement), All epidemiology ARTICLE Source Type: research

Alterations of functional connectivity of the motor cortex in Fabry disease: An RS-fMRI study
Objective: To evaluate the presence of functional connectivity (FC) alterations of the motor circuits in patients with Fabry disease (FD) and their possible correlation with clinical variables with a resting-state (RS) fMRI analysis. Methods: In our cross-sectional study, 32 patients with FD with genetically confirmed classic diagnosis of FD (12 men, mean age 43.3 ± 12.2 years) were enrolled along with 35 healthy controls (HCs) of comparable age and sex (14 men, mean age 42.1 ± 14.5 years). RS-fMRI data were analyzed with a seed-based approach, with 2 different seeds for right and left motor cortex. Patients...
Source: Neurology - May 8, 2017 Category: Neurology Authors: Cocozza, S., Pisani, A., Olivo, G., Sacca, F., Ugga, L., Riccio, E., Migliaccio, S., Brescia Morra, V., Brunetti, A., Quarantelli, M., Tedeschi, E. Tags: fMRI, Metabolic disease (inherited), Motor cortex ARTICLE Source Type: research

Neuropsychiatric symptoms predict hypometabolism in preclinical Alzheimer disease
Conclusions: The magnitude of NPS in preclinical cases, driven by sleep behavior and irritability domains, is linked to transitory metabolic dysfunctions within limbic networks vulnerable to the AD process and predicts subsequent PCC hypometabolism. These findings support an emerging conceptual framework in which NPS constitute an early clinical manifestation of AD pathophysiology. (Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Authors: Ng, K. P., Pascoal, T. A., Mathotaarachchi, S., Chung, C.-O., Benedet, A. L., Shin, M., Kang, M. S., Li, X., Ba, M., Kandiah, N., Rosa-Neto, P., Gauthier, S., For the Alzheimer's Disease Neuroimaging Initiative Tags: PET, Alzheimer's disease, Cognitive neuropsychology in dementia ARTICLE Source Type: research

Mutant IDH1 and seizures in patients with glioma
Objective: Because the d-2-hydroxyglutarate (D2HG) product of mutant isocitrate dehydrogenase 1 (IDH1mut) is released by tumor cells into the microenvironment and is structurally similar to the excitatory neurotransmitter glutamate, we sought to determine whether IDH1mut increases the risk of seizures in patients with glioma, and whether D2HG increases the electrical activity of neurons. Methods: Three WHO grade II-IV glioma cohorts from separate institutions (total N = 712) were retrospectively assessed for the presence of preoperative seizures and tumor location, WHO grade, 1p/19q codeletion, and IDH1mut status. Rat cor...
Source: Neurology - May 8, 2017 Category: Neurology Authors: Chen, H., Judkins, J., Thomas, C., Wu, M., Khoury, L., Benjamin, C. G., Pacione, D., Golfinos, J. G., Kumthekar, P., Ghamsari, F., Chen, L., Lein, P., Chetkovich, D. M., Snuderl, M., Horbinski, C. Tags: Primary brain tumor, Paraneoplastic syndrome, All Epilepsy/Seizures ARTICLE Source Type: research

Body composition status and the risk of migraine: A meta-analysis
Objective: To evaluate the association between migraine and body composition status as estimated based on body mass index and WHO physical status categories. Methods: Systematic electronic database searches were conducted for relevant studies. Two independent reviewers performed data extraction and quality appraisal. Odds ratios (OR) and confidence intervals (CI) were pooled using a random effects model. Significant values, weighted effect sizes, and tests of homogeneity of variance were calculated. Results: A total of 12 studies, encompassing data from 288,981 unique participants, were included. The age- and sex-adjuste...
Source: Neurology - May 8, 2017 Category: Neurology Authors: Gelaye, B., Sacco, S., Brown, W. J., Nitchie, H. L., Ornello, R., Peterlin, B. L. Tags: All Headache, Migraine ARTICLE Source Type: research

Serum neurofilament light as a biomarker for mild traumatic brain injury in contact sports
Objective: To evaluate whether the axonal protein neurofilament light (NFL) in serum is a sensitive biomarker to detect subtle brain injury or concussion in contact sports athletes. Methods: Two prospective cohort studies involving (1) 14 Swedish amateur boxers who underwent fluid biomarker assessments at 7–10 days after bout and after 3 months of rest from boxing and (2) 35 Swedish professional hockey players who underwent blood biomarker assessment at 1, 12, 36, and 144 hours after concussion and when the players returned to play were performed. Fourteen healthy nonathletic controls and 12 athletic controls were a...
Source: Neurology - May 8, 2017 Category: Neurology Authors: Shahim, P., Zetterberg, H., Tegner, Y., Blennow, K. Tags: Brain trauma ARTICLE Source Type: research

Clinical pearls and scientific advancement: Reconciling tradition with new knowledge
"Every child exhibiting dystonia merits an l-dopa trial, lest the potentially treatable condition of dopa-responsive dystonia is missed."1 This is but one of many clinical pearls we as neurologists learn in training. Clinical pearls are rarely evidence-based, yet we remember them well, searching for them as trainees, then passing on these same pearls to new generations. Why are clinical pearls part of our education and practice traditions? These statements distill complex pieces of information, key concepts, and clinical wisdom into short, memorable phrases. Neurology® has a section dedicated to clinical pear...
Source: Neurology - May 8, 2017 Category: Neurology Authors: Augustine, E. F., Gilbert, D. L. Tags: All Health Services Research, All Clinical Neurology, Dystonia, All Pediatric, All Education EDITORIALS Source Type: research

Use of RS-fMRI in Fabry disease: Do we need it?
Clinical studies have shown involvement of motor circuits independently of cerebrovascular symptoms in Fabry disease.1 In this issue of Neurology®, Cocozza et al.2 report alteration of the cortico-striatal pathway in Fabry disease and confirm the involvement of motor circuits in this pathology without cerebrovascular events. Strengths of this case-control study include the use of resting-state (RS) fMRI analysis for functional connectivity of the motor cortex in Fabry disease. They reported a deregulation of the basal ganglia and cortical and right cerebellar hemispheric motor circuits in these patients. (Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Authors: Siniscalchi, A. Tags: EDITORIALS Source Type: research

Isocitrate dehydrogenase mutations: A biomarker for glioma-related excitability and seizures
Tumor-related epilepsy (TRE) is common in patients with gliomas (40%–70%) and carries a substantial degree of morbidity and mortality.1,2 The impetus to understand the pathophysiology of TRE has led to the identification of several tumor markers that are associated with increased risk of seizures. Tumor-mediated glutamate release is one proposed mechanism that has more recently gained traction.3,4 Another tumor marker that has attracted the attention of researchers is expression of mutated isocitrate dehydrogenase 1 (IDH1mut), an enzyme whose normal function is key to the Kreb cycle; the nonmutated enzyme catalyzes t...
Source: Neurology - May 8, 2017 Category: Neurology Authors: Wychowski, T., Kapur, J. Tags: All Clinical trials, Primary brain tumor, All Epilepsy/Seizures EDITORIALS Source Type: research

A blood test for concussion?
Concussion, pervasive in most sports, epidemic in contact contests, affects millions of individuals ranging from youth sports through professional athletes.1 Those who routinely see patients with sports concussion rely predominately on patient-reported symptoms and a thorough neurologic examination to ascertain the extent of injury and prognosis. Although neuropsychological assessments are helpful, no other routine testing, including conventional neuroimaging, is useful in answering many basic questions regarding the clinical evaluation and management of concussion, let alone determining the severity of CNS damage.2 (Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Authors: Roy, P. Tags: All Clinical Neurology, All Clinical trials, Brain trauma EDITORIALS Source Type: research

Spotlight on the May 9 issue
(Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Teaching NeuroImages: Traumatic blown pupil without herniation
A 29-year-old man was struck by a car and presented with coma and a fixed, dilated left pupil without oculomotor palsy (figure 1). A head CT revealed central midbrain hemorrhage in the absence of uncal herniation (figure 2). Intracranial pressure was normal. At 6 months, he had regained consciousness and reactivity of the left pupil, and was independent in activities of daily living. The syndrome of acute traumatic midbrain hemorrhage is a rare form of coma and abnormal pupils.1 The unreactive pupil and lack of oculomotor palsy in our patient reflect the somatotopic map within the midbrain, namely, that pupillomotor axons ...
Source: Neurology - May 8, 2017 Category: Neurology Authors: Chung, D. Y., Edlow, B. L. Tags: Coma, Pupils, Brain trauma, Critical care, Intracerebral hemorrhage RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A 45-year-old man with weakness and myalgia after orthopedic surgery
A 45-year-old man underwent rotator cuff surgery and developed fatigue and generalized myalgia postoperatively. After 4 weeks of mild symptoms, he experienced severe muscle aches and bilateral leg weakness after walking 1.5 miles, prompting him to seek medical attention. (Source: Neurology)
Source: Neurology - May 8, 2017 Category: Neurology Authors: Vazquez do Campo, R., Siegel, J., Goldstein, E., Dimberg, E. Tags: Muscle disease RESIDENT AND FELLOW SECTION Source Type: research

Author response: Comorbidity between central disorders of hypersomnolence and immune-based disorders
We thank Dr. Peraita-Adrados for the comment on our article.1 Our finding that narcolepsy type 1 (NT1) was not associated with increased risk of comorbid immune disorders was previously stated,2 which gave us additional confidence in our results and can be explained by a unique NT1 pathophysiology. We agree that the background in Dr. Peraita-Adrados's research was the same3; however, the methodology was different: our cohort was much larger, with patients included prospectively; the study was multicentric; adult and children populations were analyzed separately; and results were controlled for potential confounders.1 Impor...
Source: Neurology - May 1, 2017 Category: Neurology Authors: Dauvilliers, Y., Barateau, L. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Comorbidity between central disorders of hypersomnolence and immune-based disorders
I read with interest the article by Barateau et al.1 The authors' research is similar to the research of our group in Madrid, but our article was not cited.2 However, the authors do mention, compare, and criticize an article published 2 years ago by our group.3 The fact that "Our results contrast with the retrospective Spanish study showing a high frequency of immunopathologic diseases and allergic comorbidities (16.6%) in NT1" was stressed by the authors,1 but why? Did the authors' relevant data not match our findings or were inconsistencies because of an incorrect comparison? We demonstrated a higher frequency ...
Source: Neurology - May 1, 2017 Category: Neurology Authors: Peraita-Adrados, R. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Cathepsin A-related arteriopathy with strokes and leukoencephalopathy (CARASAL)
We thank Fussiger et al. for the interest in our article and for the comments. We agree that the nomenclature of genetically determined cerebral small vessel disease is confusing and that using similar acronyms for genetically different variants may increase the confusion. The focus of our article was on adding a gene that is associated with cerebral small vessel disease,1 not on a new classification system. An expert in the field of cerebral small vessel disease or a consortium should consider an overall classification. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: van der Knaap, M. S., Bugiani, M., Kevelam, S. H. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Cathepsin A-related arteriopathy with strokes and leukoencephalopathy (CARASAL)
A novel autosomal dominant cerebral small vessel disease (SVD) due to mutations in CTSA was described by Bugiani et al.1 The authors named this condition cathepsin A–related arteriopathy with strokes and leukoencephalopathy (CARASAL). (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Fussiger, H., Jardim, L. B., Saute, J. A. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In WriteClick this week, in reference to "Cathepsin A–related arteriopathy with strokes and leukoencephalopathy (CARASAL)," Drs. Fussiger et al. argue for a more logical method of naming autosomal dominant small vessel disease disorders; for example, by utilizing a combination of main phenotype, inheritance pattern, and implicated genes. Authors van der Knaap et al. agree that a consensus on nomenclature would be helpful in avoiding confusion. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Alcauskas, M., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

A phase 3 trial of IV immunoglobulin for Alzheimer disease
Conclusions: Participants with mild to moderate AD showed good tolerability of treatment with low-dose human IVIg for 18 months but did not show beneficial effects on cognition or function relative to participants who received placebo. Clinicaltrials.gov identifier: NCT00818662. Classification of evidence: This study provides Class II evidence that IVIg infusions performed every 2 weeks do not improve cognition or function at 18 months in patients with mild to moderate AD. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Relkin, N. R., Thomas, R. G., Rissman, R. A., Brewer, J. B., Rafii, M. S., van Dyck, C. H., Jack, C. R., Sano, M., Knopman, D. S., Raman, R., Szabo, P., Gelmont, D. M., Fritsch, S., Aisen, P. S., For the Alzheimer's Disease Cooperative Study Tags: Clinical trials Randomized controlled (CONSORT agreement), Alzheimer's disease ARTICLE Source Type: research

Hippocampal hypometabolism in older adults with memory complaints and increased amyloid burden
Conclusions: These data show the relevant role of posterior and anterior midline regions in SMCs in older individuals. Decreased hippocampal metabolism may be a specific marker of subclinical changes in cognition due to amyloid pathology. However, longitudinal studies are needed to determine whether our findings foreshadow clinical decline. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Vannini, P., Hanseeuw, B., Munro, C. E., Amariglio, R. E., Marshall, G. A., Rentz, D. M., Pascual-Leone, A., Johnson, K. A., Sperling, R. A. Tags: PET, Alzheimer's disease, Cognitive aging ARTICLE Source Type: research

Comment: MCI trials--Categorical "square pegs" in dimensional "round holes"?
(Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Breitner, J. C. S. Tags: All Clinical trials, Clinical trials Methodology/study design, Alzheimer's disease, MCI (mild cognitive impairment) ARTICLE Source Type: research

Randomized controlled trials in mild cognitive impairment: Sources of variability
Conclusions: In spite of apparently similar criteria for MCI being adopted by the 7 studies, the implementation of the criteria varied a great deal. Several explanations including instruments used to characterize participants and variability among study populations contributed to the findings. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Petersen, R. C., Thomas, R. G., Aisen, P. S., Mohs, R. C., Carrillo, M. C., Albert, M. S., For the Alzheimer's Disease Neuroimaging Initiative (ADNI) and Foundation for NIH (FNIH) Biomarkers Consortium AD MCI Placebo Data Analysis Project Team Tags: All Clinical trials, Clinical trials Methodology/study design, Alzheimer's disease, MCI (mild cognitive impairment) ARTICLE Source Type: research

Monophasic demyelination reduces brain growth in children
Conclusions: Brain volume, and more importantly age-expected brain growth, is negatively affected by acquired demyelination, even in the absence of chronicity, implicating factors other than active inflammation as operative in this process. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Aubert-Broche, B., Weier, K., Longoni, G., Fonov, V. S., Bar-Or, A., Marrie, R. A., Yeh, E. A., Narayanan, S., Arnold, D. L., Verhey, L. H., Banwell, B., Collins, D. L., On behalf of the Canadian Pediatric Demyelinating Disease Network Tags: Volumetric MRI, All Pediatric, Optic neuritis; see Neuro-ophthalmology/Optic Nerve, Acute disseminated encephalomyelitis, Transverse myelitis ARTICLE Source Type: research

Clinical manifestations of the anti-IgLON5 disease
Conclusions: Patients with IgLON5 antibodies develop a characteristic sleep disorder preceded or accompanied by bulbar symptoms, gait abnormalities, oculomotor problems, and, less frequently, cognitive decline. IgG4 subclass antibodies predominate over IgG1; we confirm a strong association with the HLA-DRB1*10:01 allele. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Gaig, C., Graus, F., Compta, Y., Högl, B., Bataller, L., Brüggemann, N., Giordana, C., Heidbreder, A., Kotschet, K., Lewerenz, J., Macher, S., Marti, M. J., Montojo, T., Perez-Perez, J., Puertas, I., Seitz, C., Simabukuro, M., Tellez, N., Tags: Autoimmune diseases, Gait disorders/ataxia, Progressive supranuclear palsy, Parasomnias ARTICLE Source Type: research

Burnout, psychological morbidity, job stress, and job satisfaction in Chinese neurologists
Conclusions: Burnout and psychological morbidity are common in Chinese neurologists. Burnout is the single greatest predictor of neurologists' psychological morbidity, high job stress, and low job satisfaction. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Zhou, X., Pu, J., Zhong, X., Zhu, D., Yin, D., Yang, L., Zhang, Y., Fu, Y., Wang, H., Xie, P., On behalf of the China Neurologist Association Tags: All epidemiology, Prevalence studies, Risk factors in epidemiology ARTICLE Source Type: research

Physical activity, but not body mass index, predicts less disability before and after stroke
Conclusions: Physical inactivity predicts a higher risk of being dependent both before and after stroke. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Rist, P. M., Capistrant, B. D., Mayeda, E. R., Liu, S. Y., Glymour, M. M. Tags: All Cerebrovascular disease/Stroke, Cohort studies, Risk factors in epidemiology ARTICLE Source Type: research

Fine particulate matter exposure and incidence of stroke: A cohort study in Hong Kong
Conclusion: Long-term PM2.5 exposure was associated with higher risk of incident ischemic stroke, but the association with incident hemorrhagic stroke was less clear. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Qiu, H., Sun, S., Tsang, H., Wong, C.-M., Lee, R. S.-y., Schooling, C. M., Tian, L. Tags: All Cerebrovascular disease/Stroke, Cohort studies, Risk factors in epidemiology ARTICLE Source Type: research

Integrity of normal-appearing white matter and functional outcomes after acute ischemic stroke
Objective: To characterize the effect of white matter microstructural integrity on cerebral tissue and long-term functional outcomes after acute ischemic stroke (AIS). Methods: Consecutive AIS patients with brain MRI acquired within 48 hours of symptom onset and 90-day modified Rankin Scale (mRS) score were included. Acute infarct volume on diffusion-weighted imaging (DWIv) and white matter hyperintensity volume (WMHv) on T2 fluid-attenuated inversion recovery MRI were measured. Median fractional anisotropy (FA), mean diffusivity, radial diffusivity, and axial diffusivity values were calculated within normal-appearing whi...
Source: Neurology - May 1, 2017 Category: Neurology Authors: Etherton, M. R., Wu, O., Cougo, P., Giese, A.-K., Cloonan, L., Fitzpatrick, K. M., Kanakis, A. S., Boulouis, G., Karadeli, H. H., Lauer, A., Rosand, J., Furie, K. L., Rost, N. S. Tags: MRI, Other cerebrovascular disease/ Stroke, Volumetric MRI, Metabolic disease (inherited) ARTICLE Source Type: research

Outcome of intracerebral hemorrhage associated with different oral anticoagulants
Conclusions: In our international collaborative multicenter pooled analysis, baseline ICH volume, hematoma expansion, 90-day mortality, and functional outcome were similar following NOAC-ICH and VKA-ICH. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Wilson, D., Seiffge, D. J., Traenka, C., Basir, G., Purrucker, J. C., Rizos, T., Sobowale, O. A., Sallinen, H., Yeh, S.-J., Wu, T. Y., Ferrigno, M., Houben, R., Schreuder, F. H. B. M., Perry, L. A., Tanaka, J., Boulanger, M., Al-Shahi Salman, R., Ja0 Tags: Outcome research, CT, Prognosis, Clinical trials Observational study (Cohort, Case control), Intracerebral hemorrhage ARTICLE Source Type: research

A single demyelinating attack is enough to limit brain growth in children
Acquired demyelinating syndromes (ADS) represent acute neurologic illnesses characterized by deficits persisting for at least 24 hours and involving the optic nerve, brain, or spinal cord, associated with regional areas of increased T2 signal on conventional MRI. In contrast to the 75% of adult patients presenting with clinically isolated syndrome (CIS) who are eventually diagnosed with clinically defined multiple sclerosis (MS),1 the majority of children presenting with ADS have a monophasic illness with a good overall prognosis: more than 90% of children achieve a full neurologic recovery.2 Over the last decade, neuroima...
Source: Neurology - May 1, 2017 Category: Neurology Authors: Hacohen, Y., Patel, C. B., Hintzen, R. Tags: All Demyelinating disease (CNS), Acute disseminated encephalomyelitis EDITORIALS Source Type: research

Cell surface antibody-associated neurodegeneration: The case of anti-IgLON5 antibodies
Cell surface autoantibodies have become important biomarkers of acquired autoimmune CNS disorders, and typically define potentially severe but treatable acquired autoimmune encephalitis syndromes. However, the discovery of anti-IgLON5 antibodies has forced a reappraisal of the accepted paradigms regarding cell surface antibodies and their associated clinical manifestations.1 Although anti-IgLON5 antibody is a cell surface antibody, the clinical syndrome is not an encephalitis (there is no lymphocytic inflammatory infiltration), disease onset is more chronic rather than acute, and the neurologic sequelae are not reversible ...
Source: Neurology - May 1, 2017 Category: Neurology Authors: Dale, R. C., Ramanathan, S. Tags: EDITORIALS Source Type: research

The changing landscape of anticoagulant-related intracerebral hemorrhage
Hailed as a milestone in the management of patients with nonvalvular atrial fibrillation, new oral anticoagulants (NOACs) demonstrated comparable efficacy and similar or improved safety to vitamin K antagonists (VKAs) in randomized clinical trials.1 Their ensuing approvals by central regulatory agencies provoked reservations and caution by many in the medical community. Despite the consistent observation of a lower incidence of intracerebral hemorrhage (ICH) among users of various NOACs,1 the unexplored questions regarding the natural course and prognosis of NOAC-associated ICH, combined with an absence of specific treatme...
Source: Neurology - May 1, 2017 Category: Neurology Authors: Arsava, E. M., Hall, C. B. Tags: Outcome research, CT, Prognosis, Clinical trials Observational study (Cohort, Case control), Intracerebral hemorrhage EDITORIALS Source Type: research

Spotlight on the May 2 issue
(Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Teaching Video NeuroImages: Lithium-induced reversible Pisa syndrome
A 49-year-old man on chronic lithium therapy (1,500 mg/d) for bipolar disorder presented with a 2-year history of progressive left-lateral truncal flexion dystonia disappearing on lying supine (video 1 at Neurology.org), known as Pisa syndrome (PS), along with anterocollis and parkinsonism. Lithium serum level was normal (1.1 mmol/L). PS improved substantially 3 months after stopping lithium (video 2), supporting the diagnosis of lithium-induced PS (figure). PS has been reported with chronic use of a single or a combination of antipsychotics, but never with lithium monotherapy.1,2 Parkinsonism and PS may result from lithiu...
Source: Neurology - May 1, 2017 Category: Neurology Authors: Kumar, N., Mendonca, D. A., Jog, M. Tags: RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Balamuthia mandrillaris amebic encephalitis: Clinical-radiologic-pathologic correlation
A 32-year-old immunocompetent man presented with new-onset generalized tonic-clonic seizures. On examination, he had mild right hand weakness, with right-sided Hoffman sign, ankle clonus, and Babinski reflex present. MRI of the brain demonstrated vasogenic edema surrounding a ring-enhancing lesion in the left parietal lobe (figure, A). Repeat brain MRIs showed rapid progression of the lesion, with new lesions appearing in the contralateral hemisphere (figure, B and C). Pathology showed necrotizing meningoencephalitis with organisms consistent with ameba (figure, D). Indirect immunofluorescence and PCR was positive for Bala...
Source: Neurology - May 1, 2017 Category: Neurology Authors: Mittal, S. O., Alsinaidi, O. Tags: MRI, Abscess, Encephalitis, Parasitic infections, All Epilepsy/Seizures RESIDENT AND FELLOW SECTION Source Type: research

Media and Book Reviews: Clinical Neurology and Neuroanatomy: A Localization-Based Approach
Dr. Berkowitz, a clinical neurologist and gifted teacher, sought to write a clinical neurology textbook that could condense clinical neuroanatomy and clinical neurology into a concise, easily digestible format, a feat in which he has succeeded. Dr Berkowitz's approach to summarizing clinical neurology in just over 300 pages is unique compared to other similar-sized texts in that it is both pragmatic and of sufficient detail to serve as a primary reference text for the neurology resident, enthusiastic medical student, neurologist reviewing for the recertification examination, or interested general internist. (Source: Neurology)
Source: Neurology - May 1, 2017 Category: Neurology Authors: McGrath, E. R. Tags: All Clinical Neurology, Clinical neurology examination, All Education, Textbooks RESIDENT AND FELLOW SECTION Source Type: research

Author response: Long-term improvement in obsessions and compulsions with subthalamic stimulation
We thank Prof. Hariz for the interest in our illustrative single case report of a patient with treatment-resistant obsessive-compulsive disorder (OCD) with a good outcome of deep brain stimulation (DBS) of the subthalamic nucleus (STN) that remained stable over 10 years.1 We fully agree with the importance of systematically publishing long-term data and not just cherry picking, thereby introducing publication bias. The open data from the 5-year follow-up of the STOC study are being analyzed by the lead author of the main study,2 and should be available soon. However, a treatment has never proven its efficacy with a single ...
Source: Neurology - April 24, 2017 Category: Neurology Authors: Krack, P., Polosan, M., Chabardes, S., Bougerol, T., Ardouin, C., Pollak, P., Benabid, A. L. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Long-term improvement in obsessions and compulsions with subthalamic stimulation
The clinical note by Polosan et al.1 described a patient with severe obsessive-compulsive disorder (OCD) with sustained improvement 10 years after deep brain stimulation (DBS) of the subthalamic nucleus (STN). The authors wrote, "Little is known about long-term outcome of STN-DBS in OCD," and they concluded, "Future studies will have to confirm the long-term efficacy of STN-DBS in OCD illustrated in this single case report."1 These are remarkable statements coming from the authors of the pioneering French multicenter study on STN-DBS for OCD published in 2008.2 In that study, 17 patients participated in...
Source: Neurology - April 24, 2017 Category: Neurology Authors: Hariz, M. I. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Hand postures in primary and secondary generalized tonic-clonic seizures
I thank Dr. Lanska for the comments and interest in our article.1 (Source: Neurology)
Source: Neurology - April 24, 2017 Category: Neurology Authors: Siegel, J. L. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Hand postures in primary and secondary generalized tonic-clonic seizures
In an interesting and clinically useful report, Drs. Siegel and Tatum1 reported the characteristics and frequencies of different hand postures in primary and secondary generalized tonic-clonic seizures and nonepileptic attacks. Collectively, there were 98 convulsions studied from 62 patients.1 (Source: Neurology)
Source: Neurology - April 24, 2017 Category: Neurology Authors: Lanska, D. J. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In response to "Hand postures in primary and secondary generalized tonic-clonic seizures," Dr. Lanska asks the authors whether hand postures were uniform across different convulsive events in individual patients and comments on the study methods, which could have distorted the results and conclusions. (Source: Neurology)
Source: Neurology - April 24, 2017 Category: Neurology Authors: Karam, C., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Superficial brain stimulation to overcome freezing of gait in Parkinson disease
Gait impairments are common and debilitating in patients with Parkinson disease (PD). One particularly debilitating type of gait impairment is freezing of gait (FOG), which is characterized by sudden episodes during which patients feel as if their feet "are being glued to the floor."1 Presumed mechanisms underlying gait impairment in PD involve dysfunction of the basal ganglia, which enable automatic performance of overlearned movements such as gait. In PD, basal ganglia dysfunction results in reduced automaticity and a reduced ability to internally generate movements (i.e., patients experience more difficulty pr...
Source: Neurology - April 24, 2017 Category: Neurology Authors: Nonnekes, J., Janssen, S., Bloem, B. R. Tags: Parkinson's disease/Parkinsonism, Motor Control, Basal ganglia CLINICAL/SCIENTIFIC NOTES Source Type: research

Practice guideline summary: Sudden unexpected death in epilepsy incidence rates and risk factors: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the American Epilepsy Society
Objective: To determine the incidence rates of sudden unexpected death in epilepsy (SUDEP) in different epilepsy populations and address the question of whether risk factors for SUDEP have been identified. Methods: Systematic review of evidence; modified Grading Recommendations Assessment, Development, and Evaluation process for developing conclusions; recommendations developed by consensus. Results: Findings for incidence rates based on 12 Class I studies include the following: SUDEP risk in children with epilepsy (aged 0–17 years) is 0.22/1,000 patient-years (95% confidence interval [CI] 0.16–0.31) (moderat...
Source: Neurology - April 24, 2017 Category: Neurology Authors: Harden, C., Tomson, T., Gloss, D., Buchhalter, J., Cross, J. H., Donner, E., French, J. A., Gil-Nagel, A., Hesdorffer, D. C., Smithson, W. H., Spitz, M. C., Walczak, T. S., Sander, J. W., Ryvlin, P. Tags: All Pediatric, Incidence studies, Risk factors in epidemiology, All Epilepsy/Seizures SPECIAL ARTICLE Source Type: research

Paroxysmal eye-head movements in Glut1 deficiency syndrome
Objective: To describe a characteristic paroxysmal eye–head movement disorder that occurs in infants with Glut1 deficiency syndrome (Glut1 DS). Methods: We retrospectively reviewed the medical charts of 101 patients with Glut1 DS to obtain clinical data about episodic abnormal eye movements and analyzed video recordings of 18 eye movement episodes from 10 patients. Results: A documented history of paroxysmal abnormal eye movements was found in 32/101 patients (32%), and a detailed description was available in 18 patients, presented here. Episodes started before age 6 months in 15/18 patients (83%), and preceded the...
Source: Neurology - April 24, 2017 Category: Neurology Authors: Pearson, T. S., Pons, R., Engelstad, K., Kane, S. A., Goldberg, M. E., De Vivo, D. C. Tags: Metabolic disease (inherited), Ocular motility, All Pediatric, Developmental disorders, All Epilepsy/Seizures ARTICLE Source Type: research