Physical activity monitoring: A promising outcome measure in idiopathic inflammatory myopathies
Availability of reliable outcomes for idiopathic inflammatory myopathies (IIM) is critical to optimize treatment strategies.1 The current consensus for the assessment of IIM severity and treatment efficacy relies on the evaluation of core set measures comprising manual muscle testing, muscle enzymes, extramuscular disease activity assessment, physician and patient global disease activity assessment, and patient's physical function questionnaires.2 Muscle enzymes such as serum creatine kinase (CK) level are highly sensitive markers for detecting changes in disease activity though this does not correlate well with disease se...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Bachasson, D., Landon-Cardinal, O., Benveniste, O., Hogrel, J.-Y., Allenbach, Y. Tags: Outcome research, Autoimmune diseases, Muscle disease, Clinical trials Methodology/study design CLINICAL/SCIENTIFIC NOTES Source Type: research

Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium
The Dementia with Lewy Bodies (DLB) Consortium has refined its recommendations about the clinical and pathologic diagnosis of DLB, updating the previous report, which has been in widespread use for the last decade. The revised DLB consensus criteria now distinguish clearly between clinical features and diagnostic biomarkers, and give guidance about optimal methods to establish and interpret these. Substantial new information has been incorporated about previously reported aspects of DLB, with increased diagnostic weighting given to REM sleep behavior disorder and 123iodine-metaiodobenzylguanidine (MIBG) myocardial scintigr...
Source: Neurology - July 3, 2017 Category: Neurology Authors: McKeith, I. G., Boeve, B. F., Dickson, D. W., Halliday, G., Taylor, J.-P., Weintraub, D., Aarsland, D., Galvin, J., Attems, J., Ballard, C. G., Bayston, A., Beach, T. G., Blanc, F., Bohnen, N., Bonanni, L., Bras, J., Brundin, P., Burn, D., Chen-Plotkin, A Tags: Dementia with Lewy bodies, Parkinson's disease with dementia, Assessment of cognitive disorders/dementia VIEWS & amp;amp; REVIEWS Source Type: research

The botulinum toxin legend of Reinhard Heydrich's death: The end of "Himmler's brain"
The high-ranking German Nazi Reinhard Heydrich (1904–1942) was one of the main organizers of the mass murder of Jews during the Second World War. He died on June 4, 1942, in Prague after having been wounded in Operation Anthropoid planned by the British intelligence services. Since the 1970s and 1980s, Heydrich's death has been frequently presented in British, American, and French literature as the consequence of a bacteriologic attack. Botulinum toxin would have been used in the grenades or ammunition. We discuss the botulinum toxin hypothesis using the now declassified British archives of Operation Anthropoid and o...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Tatu, L., Jost, W., Bogousslavsky, J. Tags: Botulinum toxin, Bioterrorism HISTORICAL NEUROLOGY Source Type: research

Polyneuropathy relates to impairment in daily activities, worse gait, and fall-related injuries
Conclusions: Chronic polyneuropathy strongly associates with impairment in the ability to perform daily activities and relates to worse gait and an increased history of falling. (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Hanewinckel, R., Drenthen, J., Verlinden, V. J. A., Darweesh, S. K. L., van der Geest, J. N., Hofman, A., van Doorn, P. A., Ikram, M. A. Tags: Outcome research, Peripheral neuropathy, EMG, Cohort studies ARTICLE Source Type: research

Long-term unmet needs and associated factors in stroke or TIA survivors: An observational study
Conclusions: Survivors of stroke/TIA reported considerable unmet needs ≥2 years after discharge, particularly in secondary prevention. The factors associated with unmet needs could help guide policy decisions, particularly for tailoring care and support services provided after discharge. (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Olaiya, M. T., Cadilhac, D. A., Kim, J., Nelson, M. R., Srikanth, V. K., Andrew, N. E., Bladin, C. F., Gerraty, R. P., Fitzgerald, S. M., Phan, T., Frayne, J., Thrift, A. G., On behalf of the STANDFIRM (Shared Team Approach Between Nurses and Doctors for Tags: Outcome research, Medical care, Stroke prevention, All Cerebrovascular disease/Stroke ARTICLE Source Type: research

Tenecteplase in ischemic stroke offers improved recanalization: Analysis of 2 trials
Objective: To test whether patients with complete vessel occlusion show greater recanalization at 24 hours and have improved clinical outcomes at 24 hours and 90 days when treated with tenecteplase compared to alteplase. Methods: Pooled clinical and imaging data from 2 phase 2 randomized trials comparing tenecteplase with alteplase allowed CT angiography (CTA) scans to be assessed centrally for occlusion status at baseline and at 24 hours post thrombolysis using the modified thrombolysis in cerebral infarction (TICI) scale. Twenty-four-hour poststroke NIH Stroke Scale (NIHSS) and 90-day modified Rankin Scale (mRS) scores ...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Bivard, A., Huang, X., Levi, C. R., Spratt, N., Campbell, B. C. V., Cheripelli, B. K., Kalladka, D., Moreton, F. C., Ford, I., Bladin, C. F., Davis, S. M., Donnan, G. A., Muir, K. W., Parsons, M. W. Tags: CT, MRI, All Cerebrovascular disease/Stroke, Infarction ARTICLE Source Type: research

Rapid transitions in the epidemiology of stroke and its risk factors in China from 2002 to 2013
Objective: To estimate the current prevalence, temporal incidence trends, and contribution of risk factors for stroke in China. Methods: The China National Stroke Screening Survey (CNSSS) is an ongoing nationwide population-based program. A representative sample of 1,292,010 adults over 40 years old with 31,188 identified stroke cases from the 2013 and 2014 CNSSS database was analyzed to provide descriptive statistics of the prevalence and risk factors for stroke in 2014. In addition, a retrospective evaluation of 12,526 first-ever stroke cases in 2002–2013 and stroke mortality data from the 2002–2013 China Pu...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Guan, T., Ma, J., Li, M., Xue, T., Lan, Z., Guo, J., Shen, Y., Chao, B., Tian, G., Zhang, Q., Wang, L., Liu, Y. Tags: All Cerebrovascular disease/Stroke, Prevalence studies, Incidence studies, Risk factors in epidemiology ARTICLE Source Type: research

Intake of dairy foods and risk of Parkinson disease
Conclusions: Frequent consumption of dairy products appears to be associated with a modest increased risk of PD in women and men. (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Hughes, K. C., Gao, X., Kim, I. Y., Wang, M., Weisskopf, M. G., Schwarzschild, M. A., Ascherio, A. Tags: Parkinson's disease/Parkinsonism, Cohort studies, Risk factors in epidemiology ARTICLE Source Type: research

Disconnection as a mechanism for social cognition impairment in multiple sclerosis
Objective: To assess the contribution of microstructural normal-appearing white matter (NAWM) damage to social cognition impairment, specifically in the theory of mind (ToM), in multiple sclerosis (MS). Methods: We enrolled consecutively 60 patients with MS and 60 healthy controls (HC) matched on age, sex, and education level. All participants underwent ToM testing (Eyes Test, Videos Test) and 3T brain MRI including conventional and diffusion tensor imaging sequences. Tract-based spatial statistics (TBSS) were applied for whole-brain voxel-wise analysis of fractional anisotropy (FA) and mean diffusivity (MD) on NAWM. Res...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Batista, S., Alves, C., dAlmeida, O. C., Afonso, A., Felix-Morais, R., Pereira, J., Macario, C., Sousa, L., Castelo-Branco, M., Santana, I., Cunha, L. Tags: MRI, All Neuropsychology/Behavior, Neuropsychological assessment, Multiple sclerosis ARTICLE Source Type: research

Functional network integrity presages cognitive decline in preclinical Alzheimer disease
Conclusions: In clinically normal older adults, lower functional connectivity predicted more rapid decline in PACC scores over time, particularly when coupled with increased Aβ burden. Among examined networks, default, salience, and control networks were the strongest predictors of rate of change in PACC scores, with the inflection point of greatest decline beyond the fourth year of follow-up. These results suggest that rs-fcMRI may be a useful predictor of early, AD-related cognitive decline in clinical research settings. (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Buckley, R. F., Schultz, A. P., Hedden, T., Papp, K. V., Hanseeuw, B. J., Marshall, G., Sepulcre, J., Smith, E. E., Rentz, D. M., Johnson, K. A., Sperling, R. A., Chhatwal, J. P. Tags: fMRI, PET, Alzheimer's disease, Cognitive neuropsychology in dementia ARTICLE Source Type: research

Familial aggregation of focal seizure semiology in the Epilepsy Phenome/Genome Project
Conclusion: Our results provide insight into genotype–phenotype correlation in the nonacquired focal epilepsies and a framework for identifying subgroups of patients likely to share susceptibility genes. (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Tobochnik, S., Fahlstrom, R., Shain, C., Winawer, M. R., For the EPGP Investigators Tags: All Epilepsy/Seizures, All Genetics ARTICLE Source Type: research

Outcome measures in the idiopathic inflammatory myopathies: On the search for the holy grail
The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of rare autoimmune disorders that share some similarities, such as proximal predominant symmetric muscle weakness, also known as myopathy pattern 1,1 except for myopathy pattern 4 (distal arm and proximal leg weakness), in inclusion body myositis (IBM).1,2 Common features in IBM, dermatomyositis, polymyositis, and necrotizing autoimmune myopathy, frequently include serum creatine kinase (CK) elevation and myopathy with muscle membrane irritability on EMG. Muscle histopathology findings vary depending on the underlying disease, and may include inflammato...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Dimachkie, M. M., Paganoni, S. Tags: Outcome research, Autoimmune diseases, Muscle disease, Clinical trials Methodology/study design EDITORIALS Source Type: research

Dementia with Lewy bodies advances: A new consensus report
Beginning in the 1980s, a number of studies defined a subset of patients with dementia with brainstem and cortical Lewy bodies and associated clinical characteristics. Based on the accumulated evidence, an international consortium proposed clinical and pathologic criteria for this distinct syndrome, dementia with Lewy bodies (DLB).1 Two subsequent consensus reports refined the diagnostic and pathologic criteria as additional evidence emerged. In this issue of Neurology®, the DLB Consortium publishes the fourth consensus report, further refining the clinical criteria for DLB by incorporating new evidence.2 (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Barrett, M. J., Armstrong, M. J. Tags: Dementia with Lewy bodies, Parkinson's disease with dementia, Assessment of cognitive disorders/dementia EDITORIALS Source Type: research

White matter changes and social cognitive function in MS: When all is no longer in the eyes
The mechanisms and consequences of cognitive impairment in people with multiple sclerosis (MS) are slowly being uncovered. Interest in this area has in part increased due to the lack of clear evidence for effective therapies.1 A separate, major motivator is that cognitive impairment is not just a predictor of unemployment but is the leading predictor of occupational disability.2 This increasing interest in cognitive function has been mirrored by a parallel increase in studies of social cognition: a collection of psychological processes that facilitate interpersonal communication and interaction by helping individuals under...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Cotter, J., Muhlert, N. Tags: MRI, All Neuropsychology/Behavior, Neuropsychological assessment, Multiple sclerosis EDITORIALS Source Type: research

Advancing the phenome alongside the genome in epilepsy studies
The development of high-throughput sequencing technologies has led to remarkable progress in understanding the genetic basis of human epilepsies. Over the last 5 years, more than 20 genes have been identified through genome-first approaches, identifying novel genes first and then working backwards to understand the associated phenotypes. This approach has been useful in epileptic encephalopathies, where family-based exome studies have identified a growing list of genes with causative de novo mutations.1 (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Helbig, I., Lindhout, D. Tags: Cohort studies, Epilepsy semiology, Partial seizures, All Genetics, Association studies in genetics EDITORIALS Source Type: research

Correcting honest pervasive errors in the scientific literature: Retractions without stigma
In the June 6 issue of Neurology®, we published a retraction of an article,1 a replacement article,2 and a copy of the original published version of the article with the changes highlighted. We had been alerted by a reader's submission to our WriteClick® Rapid Online Correspondence section that the authors had misdiagnosed a medical condition.3,4 Conversations with the authors revealed that the use of an erroneous term for the diagnosis was an honest mistake caused by an error in translation from another language. Issuing a retraction and a replacement version were vitally important to prevent misinforming readers ...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Baskin, P. K., Mink, J. W., Gross, R. A. Tags: EDITORIALS Source Type: research

Teaching NeuroImages: Abnormal cervical and cerebral vasculature in 22q11 deletion syndrome
A 12-day-old girl with a postnatal microarray diagnosis of 22q11.2 deletion syndrome was transferred for surgical repair of truncus arteriosus. Neurologic examination at the time of transfer was unremarkable. Brain MRI on day of life 9 demonstrated an absent left internal carotid flow void. Magnetic resonance angiography of the head (figure, A) and neck (figure, B) was subsequently obtained prior to cardiac repair. Patients with 22q11 deletion syndromes may have abnormal cervical vessel development.1 Children with congenital heart disease have an elevated stroke risk, particularly for periprocedural stroke.2 Understanding ...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Mithal, D. S., Kurz, J. E. Tags: All Cerebrovascular disease/Stroke, Pediatric stroke; see Cerebrovascular Disease/ Childhood stroke, All Genetics RESIDENT AND FELLOW SECTION Source Type: research

Message from the Editors to our Reviewers
We cannot adequately convey our gratitude for the dedication of Neurology's peer reviewers. Peer review is an essential mechanism for assessing quality of research and conferring credibility to the content we publish, and authors benefit greatly by receiving your feedback on their work. Listing your names in this message cannot fully reflect our appreciation for your efforts in reviewing papers. (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Gross, R. A., Worrall, B. B., Amato, A. A., Cascino, G. D., Ciccarelli, O., Corboy, J. R., Dalmau, J. O., Gottesman, R. F., Grossman, M., Millichap, J. J., Mink, J. W., Pulst, S. M., Uitti, R. J. Tags: ACKNOWLEDGMENT TO REVIEWERS Source Type: research

Clinical Reasoning: A 58-year-old man with progressive ptosis and walking difficulty
A 58-year-old man developed progressive walking difficulty over 10 years. He had normal development and an unremarkable birth history. At age 30, he developed bilateral drooping eyelids with double vision. At age 48, he developed progressive gait unsteadiness, slurred speech, and swallowing difficulty. At age 55, he needed to walk with a cane. He did not have seizures, hearing loss, or memory impairment. He did not have any history of alcohol abuse or exposures to anticonvulsants. His mother has congenital cardiac disease and diabetes mellitus, and his sister has had epilepsy since childhood. His daughter is healthy. (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Kuo, P.-H., Lo, R. Y., Tanji, K., Kuo, S.-H. Tags: Gait disorders/ataxia, Parkinson's disease/Parkinsonism, Eyelids, Diplopia (double vision), Mitochondrial disorders RESIDENT AND FELLOW SECTION Source Type: research

Spotlight on the July 4 issue
(Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Author response: Gram-negative bacterial molecules associate with Alzheimer disease pathology
The comment by Dr. Mente raises critical issues for any postmortem microbial study. We agree that short postmortem intervals (PMI) and precautions against contamination before dissection procedures, coupled with negative blood and organ cultures, would help exclude possible contamination. In our study,1 PMIs were similar in Alzheimer disease (AD) compared to controls, brain procurement was performed before general autopsy, and identical procedures were used for removing and processing samples from AD and control brains. Therefore, contamination is unlikely to explain the markedly increased levels and differing cellular loc...
Source: Neurology - June 12, 2017 Category: Neurology Authors: Zhan, X. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Gram-negative bacterial molecules associate with Alzheimer disease pathology
The findings of Zhan et al.1 are intriguing and further the understanding of Alzheimer disease pathogenesis. Although the investigators meticulously described the brain tissue handling procedures, there are concerns about contamination. In forensic pathology, postmortem microbial translocation from the gastrointestinal tract to other organs, especially with longer postmortem intervals, may interfere with the interpretation of microbiology cultures.2 A general autopsy performed before brain procurement could be another source of contamination from the gastrointestinal tract.3 Nevertheless, contamination can be detected by 1...
Source: Neurology - June 12, 2017 Category: Neurology Authors: Mente, K. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Practice guideline summary: Treatment of restless legs syndrome in adults: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology
We appreciate Dr. Sethi's emphasis on the often overlooked iron treatment of restless legs syndrome (RLS) in the comment on our article.1 The recommendation to treat patients with serum ferritin less than or equal to 75 μg/L was based on the reviewed placebo-controlled study showing significant RLS benefit and ferritin increase with ferrous sulfate 325 mg (65 mg iron) with vitamin C 100 mg dosed twice daily.2 As described in the full-length guideline,1 only oral iron studies where patients had evidence of iron deficiency were considered for the guideline, and oral iron is absorbed only in the context of low iron levels....
Source: Neurology - June 12, 2017 Category: Neurology Authors: Allen, R. P., Armstrong, M. J., Trenkwalder, C., Zee, P. C., Winkelman, J. W. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Practice guideline summary: Treatment of restless legs syndrome in adults: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology
I read with interest the practice guideline summary report for treatment of restless leg syndrome (RLS) in adults by the Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology.1 Studies have shown a strong correlation between body iron stores, as determined by serum ferritin level, and the severity of RLS symptoms.2,3 Typically, in patients with RLS with ferritin level
Source: Neurology - June 12, 2017 Category: Neurology Authors: Sethi, N. K. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In WriteClick this week, Dr. Sethi asks for additional clarification about ferritin levels and iron supplementation beyond that found in "Practice guideline summary: Treatment of restless legs syndrome in adults." Authors Allen et al. review the pertinent evidence. (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: Alcauskas, M., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

A prepontine subarachnoid abscess by Escherichia coli
A 53-year-old man had 6 months of intermittent fever and worsening headache, with 4/5 left hemiparesis and new unsteady gait, without other deficits. His immune status was normal, and he had self-treated with paracetamol-caffeine-aspirin powder. Two years prior he had purulent meningitis diagnosed by lumbar puncture with negative CSF culture, treated with ceftriaxone/vancomycin for 2 weeks. (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: Ren, Y., You, C., Liu, X. Tags: Abscess, Meningitis, Bacterial infections NEUROIMAGES Source Type: research

Square patches of light remember you
(Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: Ficek, B. N. Tags: Parkinson's disease/Parkinsonism, Progressive supranuclear palsy, Aphasia, All Cognitive Disorders/Dementia REFLECTIONS: NEUROLOGY AND THE HUMANITIES Source Type: research

Zika virus infection-associated acute transient polyneuritis
We present 3 cases of a newly described syndrome of ZIKV-associated acute transient polyneuritis. (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: Nascimento, O. J. M., Frontera, J. A., Amitrano, D. A., Bispo de Filippis, A. M., Da Silva, I. R. F., On behalf of the RIO-GBS-ZIKV Research Group Tags: Ultrasound, All Immunology, All Infections, Peripheral neuropathy, EMG CLINICAL/SCIENTIFIC NOTES Source Type: research

Benefits, pitfalls, and future design of population-based registers in neurodegenerative disease
Population-based disease registers identify and characterize all cases of disease, including those that might otherwise be neglected. Prospective population-based registers in neurodegeneration are necessary to provide comprehensive data on the whole phenotypic spectrum and can guide planning of health services. With the exception of the rare disease amyotrophic lateral sclerosis, few complete population-based registers exist for neurodegenerative conditions. Incomplete ascertainment, limitations and uncertainty in diagnostic categorization, and failure to recognize sources of bias reduce the accuracy and usefulness of man...
Source: Neurology - June 12, 2017 Category: Neurology Authors: Rooney, J. P. K., Brayne, C., Tobin, K., Logroscino, G., Glymour, M. M., Hardiman, O. Tags: All epidemiology, Incidence studies VIEWS & amp;amp; REVIEWS Source Type: research

Evaluating the safety of {beta}-interferons in MS: A series of nested case-control studies
Conclusions: Among patients with RRMS, IFN-β was associated with a 1.8- and 1.6-fold increase in the risk of stroke and migraine and 1.3-fold increases in depression and hematologic abnormalities. (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: de Jong, H. J. I., Kingwell, E., Shirani, A., Cohen Tervaert, J. W., Hupperts, R., Zhao, Y., Zhu, F., Evans, C., van der Kop, M. L., Traboulsee, A., Gustafson, P., Petkau, J., Marrie, R. A., Tremlett, H., On behalf of the British Columbia Multiple Scleros Tags: Patient safety, Multiple sclerosis, All epidemiology, Case control studies ARTICLE Source Type: research

Neurofilament markers for ALS correlate with extent of upper and lower motor neuron disease
Conclusions: In particular, CSF pNfH concentrations show an added value as diagnostic biomarkers for ALS, whereas the prognostic value of pNfH and NfL warrants further investigation. Both pNfH and NfL correlated with the extent of motor neuron degeneration. Classification of evidence: This study provides Class II evidence that elevated concentrations of CSF pNfH and NfL can accurately identify patients with ALS. (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: Poesen, K., De Schaepdryver, M., Stubendorff, B., Gille, B., Muckova, P., Wendler, S., Prell, T., Ringer, T. M., Rhode, H., Stevens, O., Claeys, K. G., Couwelier, G., D'Hondt, A., Lamaire, N., Tilkin, P., Van Reijen, D., Gourmaud, S., Fedtke, N., Heiling, Tags: Amyotrophic lateral sclerosis, Cerebrospinal Fluid ARTICLE Source Type: research

Effect of omega-3 supplementation on neuropathy in type 1 diabetes: A 12-month pilot trial
Conclusions: Twelve months of -3 supplementation was associated with increase in CNFL in T1DM. ClinicalTrials.govidentifier: NCT02034266. Classification of evidence: This study provides Class IV evidence that for patients with T1DM and evidence of DSP, 12 months of seal oil omega-3 supplementation increases CNFL. (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: Lewis, E. J. H., Perkins, B. A., Lovblom, L. E., Bazinet, R. P., Wolever, T. M. S., Bril, V. Tags: All Neuromuscular Disease, Peripheral neuropathy ARTICLE Source Type: research

Presurgical thalamic "hubness" predicts surgical outcome in temporal lobe epilepsy
Conclusions: A thalamic network associated with seizure recurrence may already be established presurgically. Thalamic hubness can serve as a potential biomarker of surgical outcome, outperforming the clinical characteristics commonly used in epilepsy surgery centers. (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: He, X., Doucet, G. E., Pustina, D., Sperling, M. R., Sharan, A. D., Tracy, J. I. Tags: Outcome research, fMRI, Epilepsy surgery, Functional neuroimaging ARTICLE Source Type: research

Clinical marker for Alzheimer disease pathology in logopenic primary progressive aphasia
Objective: To determine whether logopenic features of phonologic loop dysfunction reflect Alzheimer disease (AD) neuropathology in primary progressive aphasia (PPA). Methods: We performed a retrospective case-control study of 34 patients with PPA with available autopsy tissue. We compared baseline and longitudinal clinical features in patients with primary AD neuropathology to those with primary non-AD pathologies. We analyzed regional neuroanatomic disease burden in pathology-defined groups using postmortem neuropathologic data. Results: A total of 19/34 patients had primary AD pathology and 15/34 had non-AD pathology (...
Source: Neurology - June 12, 2017 Category: Neurology Authors: Giannini, L. A. A., Irwin, D. J., McMillan, C. T., Ash, S., Rascovsky, K., Wolk, D. A., Van Deerlin, V. M., Lee, E. B., Trojanowski, J. Q., Grossman, M. Tags: Alzheimer's disease, Dementia aphasia, Assessment of cognitive disorders/dementia ARTICLE Source Type: research

Racial disparities in neurologic health care access and utilization in the United States
Conclusions: Our findings highlight racial and ethnic inequalities in the utilization of neurologic care in the United States. (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: Saadi, A., Himmelstein, D. U., Woolhandler, S., Mejia, N. I. Tags: All Health Services Research, Medical care, All epidemiology ARTICLE Source Type: research

Total small vessel disease score and risk of recurrent stroke: Validation in 2 large cohorts
Objective: In patients with TIA and ischemic stroke, we validated the total small vessel disease (SVD) score by determining its prognostic value for recurrent stroke. Methods: Two independent prospective studies were conducted, one comprising predominantly Caucasian patients with TIA/ischemic stroke (Oxford Vascular Study [OXVASC]) and one predominantly Chinese patients with ischemic stroke (University of Hong Kong [HKU]). Cerebral MRI was performed and assessed for lacunes, microbleeds, white matter hyperintensities (WMH), and perivascular spaces (PVS). Predictive value of total SVD score for risk of recurrent stroke was...
Source: Neurology - June 12, 2017 Category: Neurology Authors: Lau, K. K., Li, L., Schulz, U., Simoni, M., Chan, K. H., Ho, S. L., Cheung, R. T. F., Küker, W., Mak, H. K. F., Rothwell, P. M. Tags: MRI, Prognosis, All Cerebrovascular disease/Stroke, Infarction, Intracerebral hemorrhage ARTICLE Source Type: research

MR perfusion lesions after TIA or minor stroke are associated with new infarction at 7 days
Objective: To investigate the relationship between acute perfusion-weighted imaging (PWI) lesions occurring within the first hours after a TIA or a minor brain infarction (BI) and the incidence of new BI detected on a systematic MRI at 1 week. Methods: Consecutive patients who experienced a TIA or BI with a neurologic deficit that lasted
Source: Neurology - June 12, 2017 Category: Neurology Authors: Lee, J., Inoue, M., Mlynash, M., Mann, S. K., Cereda, C. W., Ke, M., Albers, G. W., Olivot, J. M. Tags: Stroke prevention, MRI ARTICLE Source Type: research

Evaluation of hyperacute infarct volume using ASPECTS and brain CT perfusion core volume
Objective: To compare the accuracy of Alberta Stroke Program Early Computed Tomography Score (ASPECTS) and CT perfusion to detect established infarction in acute anterior circulation stroke. Methods: We performed an observational study in 59 acute anterior circulation ischemic stroke patients who underwent brain noncontrast CT, CT perfusion, and MRI within 100 minutes from CT imaging. ASPECTS scores were calculated by 4 blinded vascular neurologists. The accuracy of ASPECTS and CT perfusion core volume to detect an acute MRI diffusion lesion of ≥70 mL was evaluated using receiver operating characteristics analysis and ...
Source: Neurology - June 12, 2017 Category: Neurology Authors: Demeestere, J., Garcia-Esperon, C., Garcia-Bermejo, P., Ombelet, F., McElduff, P., Bivard, A., Parsons, M., Levi, C. Tags: CT, MRI, All Cerebrovascular disease/Stroke ARTICLE Source Type: research

Thalamus as a "hub" to predict outcome after epilepsy surgery
The role of the thalamus in mediating seizure activity has been investigated for decades.1 In 1952, Penfield2 proposed "centrencephalic" interactions as an integral part not only of generalized but also of focal automatisms. The dynamic integration between the centrencephalic system and cerebral cortex was hypothesized as pivotal for normal brain function, as well as a mechanism for seizure propagation. The concept was later hotly debated, and generalized seizures with 3-Hz spike wave discharges were considered mainly centrencephalic.1 (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: Jobst, B. C., Cascino, G. D. Tags: All Epilepsy/Seizures, Epilepsy surgery, Functional neuroimaging, Partial seizures EDITORIALS Source Type: research

Beyond clinical syndromes in primary progressive aphasia: Seeking etiologic diagnoses
Primary progressive aphasia (PPA) is an acquired disorder in which speech and language problems are the dominant (salient) manifestations during the initial symptomatic illness.1 Because of substantial syndromic–etiologic heterogeneity in some subtypes of PPA, it is a case study in the challenges of linking clinical syndromes to etiology. In a disorder like PPA, there is a pressing need to develop methodologies to diagnose underlying etiology. While definitive treatment for the neurodegenerative cognitive disorders is still in the future, basic and translational neuroscience is at the threshold of testing promising n...
Source: Neurology - June 12, 2017 Category: Neurology Authors: Knopman, D. S., Nestor, P. J. Tags: EDITORIALS Source Type: research

Imaging-based selection of patients for acute stroke treatment: Is it ready for prime time?
Historically, brain imaging in acute stroke has sought to exclude brain hemorrhage in order to allow therapy aiming at recanalization of the occluded intracranial artery. The National Institute of Neurological Disorders and Stroke study, published in 1995, found a clinical benefit of IV thrombolysis in acute stroke after exclusion of brain hemorrhage based on noncontrast CT (NCCT).1 More than 20 years later, the decision at most centers whether to give thrombolysis within the first 4.5 hours remains based on NCCT; often, the bolus of recombinant tissue plasminogen activator (rt-PA) is administered in the CT scanner suite e...
Source: Neurology - June 12, 2017 Category: Neurology Authors: Carrera, E., Wintermark, M. Tags: CT, MRI, Infarction EDITORIALS Source Type: research

Spotlight on the June 13 issue
(Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Pearls & Oy-sters: Retrievable and awake: A case report of solitaire stent employment for venous pulsatile tinnitus
A 50-year-old woman presented with a 2-year history of right-sided pulsatile tinnitus. Her medical history was unremarkable. Over the preceding 2 years, with the diagnosis of idiopathic pulsatile tinnitus, a bruit gradually developed and severely disturbed her sleep (Tinnitus Handicap Inventory score of 58). (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: Du, Z., Liu, X., Cao, X., Chen, X., Mang, J., Wang, J., Li, B. Tags: Tinnitus RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: Acute onset facial droop in a 36-year-old pregnant woman
A 36-year-old woman, G1P0, 22 weeks pregnant, presented to the emergency department for evaluation of acute onset facial droop. Her medical history included ulcerative colitis, primary sclerosing cholangitis, and heterozygosity for the prothrombin G20210A mutation. She was on 10,000 units of subcutaneous heparin twice daily for a previous deep vein thrombosis secondary to her prothrombin mutation; she was noncompliant with prescribed aspirin. (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: George, I. C., Youn, T. S., Marcolini, E. G., Greer, D. M. Tags: Other cerebrovascular disease/ Stroke, Autoimmune diseases, Critical care, All Demyelinating disease (CNS) RESIDENT AND FELLOW SECTION Source Type: research

Education Research: Positive effect of scheduled faculty modeling on clerkship student bedside skills exposure and learning
Conclusions: Our study demonstrated that there was a significant increase in structured observation by students of neurologic bedside skills with the inclusion of a scheduled modeling experience, and we provide a qualitative description of the most common learning themes associated with this experience. (Source: Neurology)
Source: Neurology - June 12, 2017 Category: Neurology Authors: Thompson Stone, R., Tollefson, T., Epstein, R., Jozefowicz, R. F., Mink, J. W. Tags: Clinical neurology history, Clinical neurology examination, All Education, Methods of education RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Giant neurocysticercosis with unusual imaging manifestations
The Neurology® editors and the authors of the article "Teaching NeuroImages: Giant neurocysticercosis with unusual imaging manifestations,"1 published online in conjunction with the November 22, 2016, issue of Neurology, agree to the retraction of the article. Retraction follows publication of a WriteClick® Editor's Choice correspondence exchange in which a pervasive translation error was identified.2,3 The diagnosis should have been "cystic echinococcosis," not "cysticercus." The article has been corrected and republished.4 (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Tags: RETRACTION Source Type: research

Author response: Teaching NeuroImages: Idiopathic hypertrophic pachymeningitis
As discussed by Dr. Budhram, immunoglobulin G4 (IgG4)–related disease (RD) must be considered in patients with hypertrophic pachymeningitis (HP) as it accounts for a high proportion of cases originally thought to be idiopathic.1 IgG4-related HP is pathologically characterized by a lymphoplasmacytic infiltration of IgG4-positive plasma cells.2 The patient we presented had normal IgG4 levels in both serum and CSF.1 Dural biopsy was consistent with a chronic lymphohistiocytic pachymeningitis without substantial plasma cell infiltrate to suggest IgG4-RD. In addition, immunohistochemistry performed on the dural biopsy spe...
Source: Neurology - June 5, 2017 Category: Neurology Authors: Wasilewski, A., Samkoff, L. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Teaching NeuroImages: Idiopathic hypertrophic pachymeningitis
As alluded to by Drs. Wasilewski and Samkoff,1 immunoglobulin G4 (IgG4)–related disease (RD) is an increasingly recognized cause of hypertrophic pachymeningitis (HP) and likely accounts for a substantial subset of cases previously thought to be idiopathic.2 Although normal IgG4 levels in serum and CSF argue against IgG4-related HP in this case, meningeal biopsy remains the diagnostic gold standard.3,4 Since IgG4-RD is a major consideration, it is of critical importance to immunostain biopsied tissue for the number of IgG4+ cells/high-powered field (HPF) prior to labeling the disease entity idiopathic; more than 10 Ig...
Source: Neurology - June 5, 2017 Category: Neurology Authors: Budhram, A. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Teaching NeuroImages: Giant neurocysticercosis with unusual imaging manifestations
We thank Dr. Del Brutto for the interest in and comments on our NeuroImage.1 We made a translation error, as identified by Dr. Del Brutto. A typical example of parasitic disease is a single, large, low lesion without peripheral edema or enhancement. The postoperative pathologic examination showed parasite scolices in left temporal and occipital lobes; the final diagnosis was hydatid disease in Chinese. Accurate translation of hydatid disease is cystic echinococcosis, not cysticercus, in the English–Chinese dictionary; specifically, cystic echinococcosis caused by infection with Echinococcus spp. (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Guo, J. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Teaching NeuroImages: Giant neurocysticercosis with unusual imaging manifestations
The Teaching NeuroImage by Nie et al.1 is not of a cysticercus as mentioned by the authors; instead, it is a clear example of cystic hydatid disease, caused by infection with Echinococcus spp. A single, large, unilocular nonenhancing parenchymal brain cyst with well-defined borders and not surrounded by edema is fairly characteristic of this parasitic disease.2 In addition, scolices are clearly shown in surgical and histopathologic photographs. Proper interpretation of currently available sets of diagnostic criteria for neurocysticercosis is advised to avoid these diagnostic pitfalls.3,4 (Source: Neurology)
Source: Neurology - June 5, 2017 Category: Neurology Authors: Del Brutto, O. H. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research