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Epilepsy-associated long-term mortality after aneurysmal subarachnoid hemorrhage
Conclusions: Comorbid epilepsy in 12-month survivors of SIA-SAH is associated with increased risk of death in long-term follow-up. Survivors of SIA-SAH require long-term dedicated follow-up, including identification and effective treatment of comorbid epilepsy to prevent avoidable deaths. (Source: Neurology)
Source: Neurology - July 17, 2017 Category: Neurology Authors: Huttunen, J., Lindgren, A., Kurki, M. I., Huttunen, T., Frösen, J., Koivisto, T., von und zu Fraunberg, M., Immonen, A., Jääskeläinen, J. E., Kälviäinen, R. Tags: Outcome research, Cohort studies, All Epilepsy/Seizures, Subarachnoid hemorrhage ARTICLE Source Type: research

Diagnosis of DWI-negative acute ischemic stroke: A meta-analysis
Objective: To determine the prevalence of diffusion-weighted imaging (DWI)–negative acute ischemic stroke (AIS) and to identify clinical characteristics of patients with DWI-negative AIS. Methods: We systematically searched PubMed and Ovid/MEDLINE for relevant studies between 1992, the year that the DWI sequence entered clinical practice, and 2016. Studies were included based upon enrollment of consecutive patients presenting with a clinical diagnosis of AIS prior to imaging. Meta-analysis was performed to synthesize study-level data, estimate DWI-negative stroke prevalence, and estimate the odds ratios (ORs) for cl...
Source: Neurology - July 17, 2017 Category: Neurology Authors: Edlow, B. L., Hurwitz, S., Edlow, J. A. Tags: MRI, DWI, All Cerebrovascular disease/Stroke, Infarction ARTICLE Source Type: research

Insulin resistance among obese middle-aged is associated with decreased cerebrovascular reactivity
Conclusions: IR is associated with impaired CVR; the relationship appears to be driven by the degree of IR and not by obesity. These rarely reported results suggest that early forms of cerebrovascular dysfunction exist among obese middle-aged individuals with significant IR but without type 2 diabetes mellitus. These functional vascular abnormalities may help explain the associations among IR, diabetes, and dementia, and suggest that interventions aiming to improve IR or CVR may help prevent cognitive decline later in life. (Source: Neurology)
Source: Neurology - July 17, 2017 Category: Neurology Authors: Frosch, O. H., Yau, P. L., Osorio, R. S., Rusinek, H., Storey, P., Convit, A. Tags: MRI, All Cerebrovascular disease/Stroke ARTICLE Source Type: research

Characterization of patients with longstanding idiopathic REM sleep behavior disorder
Objective: To evaluate the presence of prodromal markers of Parkinson disease (PD) in patients with longstanding idiopathic REM sleep behavior disorder (IRBD), a small subgroup of individuals with IRBD with long-term follow-up thought not to be at risk of developing PD. Methods: Demographic, clinical, and neuroimaging markers of PD were evaluated in 20 patients with polysomnographic-confirmed longstanding IRBD and in 32 matched controls. Results: Patients were 16 men and 4 women with mean age of 72.9 ± 8.6 years and mean follow-up from IRBD diagnosis of 12.1 ± 2.6 years. Patients more often had objective sm...
Source: Neurology - July 17, 2017 Category: Neurology Authors: Iranzo, A., Stefani, A., Serradell, M., Marti, M. J., Lomena, F., Mahlknecht, P., Stockner, H., Gaig, C., Fernandez-Arcos, A., Poewe, W., Tolosa, E., Högl, B., Santamaria, J., For the SINBAR (Sleep Innsbruck Barcelona) group Tags: Parkinson's disease/Parkinsonism, Parasomnias ARTICLE Source Type: research

Disease progression in C9orf72 mutation carriers
Conclusions: The pattern of scores of the ALSFRS-R, letter fluency, and FBI distinguished between ALS, ALS-FTD, and FTD presentations of C9orf72 mutation carriers and asymptomatic carriers. Longitudinal changes in these measures occurred with disease progression in a manner consistent with presenting phenotype. (Source: Neurology)
Source: Neurology - July 17, 2017 Category: Neurology Authors: Floeter, M. K., Traynor, B. J., Farren, J., Braun, L. E., Tierney, M., Wiggs, E. A., Wu, T. Tags: Amyotrophic lateral sclerosis, Executive function, Frontotemporal dementia, All Genetics ARTICLE Source Type: research

The evolving genetic risk for sporadic ALS
Conclusions: Previous analyses have identified SALS-predisposing variants only in terms of their rarity in normal control populations. By incorporating variant pathogenicity as well as variant frequency, we demonstrated that the genetic risk contributed by these genes for SALS is substantially lower than previous estimates. (Source: Neurology)
Source: Neurology - July 17, 2017 Category: Neurology Authors: Gibson, S. B., Downie, J. M., Tsetsou, S., Feusier, J. E., Figueroa, K. P., Bromberg, M. B., Jorde, L. B., Pulst, S. M. Tags: All Neuromuscular Disease, Amyotrophic lateral sclerosis, All Genetics, Association studies in genetics ARTICLE Source Type: research

Chronic headache: What's in a name?
"What's in a name? That which we call a rose—By any other name would smell as sweet," Shakespeare wrote in his famous play Romeo and Juliet (II, ii, 1–2). This may be true from a poetic point of view for roses, but we also know how it ended for Romeo and Juliet. When it comes to classification of headache disorders nomenclature, it is crucial for research and clinical practice. In this issue of Neurology®, Probyn et al.1 describe prognostic factors for chronic headache, by means of an extensive systematic review. The enormous amount of work that went into producing this article, as well as it...
Source: Neurology - July 17, 2017 Category: Neurology Authors: Louter, M. A., Ward, T. N., Terwindt, G. M. Tags: Migraine, Tension headache EDITORIALS Source Type: research

Epilepsy and mortality after aneurysmal subarachnoid hemorrhage
Aneurysm rupture causes 85% of spontaneous subarachnoid hemorrhage (SAH), and saccular intracranial aneurysms (SIA) account for the majority of ruptured aneurysms. SAH represents approximately 5% of all strokes. Despite this relatively low proportion, the disease has a disproportionately high burden, accounting for a quarter of life years lost due to cerebrovascular disease in one study,1 largely explained by the relatively young age of affected patients and high fatality. (Source: Neurology)
Source: Neurology - July 17, 2017 Category: Neurology Authors: Harroud, A., Crepeau, A. Z. Tags: Outcome research, Cohort studies, All Epilepsy/Seizures, Subarachnoid hemorrhage EDITORIALS Source Type: research

Is all ALS genetic?
In this issue of Neurology®, Gibson et al.1 address an important issue in amyotrophic lateral sclerosis (ALS) genetics; namely, what percentage of isolated or sporadic forms of ALS that have an identifiable genetic factor are likely responsible for disease pathogenesis. To address this, the authors used a 3-step approach: first, they selected 87 patients they diagnosed with sporadic ALS (SALS) and who were of European ancestry (to get as a homogenous a study cohort as possible). They then screened the exons of all 23,000 genes, focusing on variants in the 31 genes earlier linked to ALS, as well as 2 repeat intron ...
Source: Neurology - July 17, 2017 Category: Neurology Authors: Andersen, P. M. Tags: All Neuromuscular Disease, Amyotrophic lateral sclerosis, All Genetics, Association studies in genetics EDITORIALS Source Type: research

Chance juxtapositions and (un)biased methods in science: More efficient at inefficiency
A recent article in Neurology® hammered home several points made all the more cogent by proposals relative to the Federal budget and allegations made during the 2016 Presidential campaign. Shepheard et al.1 demonstrated that the extracellular domain (ECD) of the low-affinity neurotrophin receptor, p75NTR, is found in higher-than-normal concentration in the urine of patients with amyotrophic lateral sclerosis (ALS). This urinary excess of p75ECD varies in magnitude in proportion to other more cumbersome and more expensive measures of nerve loss and muscle dysfunction. This may make measurement of urinary p75ECD a p...
Source: Neurology - July 17, 2017 Category: Neurology Authors: Schor, N. F. Tags: SPECIAL EDITORIALS Source Type: research

Spotlight on the July 18 issue
(Source: Neurology)
Source: Neurology - July 17, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Teaching Video NeuroImages: Bent spine syndrome as an early presentation of late-onset Pompe disease
We report a 56-year-old man with genetically proven late-onset Pompe disease (LOPD) showing BSS (figure 1 and video at Neurology.org). He complained of axial weakness that progressively forced him to walk stretching his arms backward since his 20s. Whole-body muscle MRI revealed severe fatty replacement of lumbar paraspinal (figure 2) and iliopsoas muscles, and spirometry showed restrictive ventilatory defect. Periodic acid-Schiff–positive vacuoles and reduced acid α-glucosidase activity were demonstrated in muscle biopsy. While lumbar hyperlordosis is a typical feature of LOPD, other trunk abnormalities may ap...
Source: Neurology - July 17, 2017 Category: Neurology Authors: Magrinelli, F., Tosi, M., Tonin, P. Tags: MRI, Clinical neurology examination, Muscle disease, All Genetics RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Amlodipine-responsive trigeminal neuralgia: An alibi for vascular compression theory
A 55-year-old woman developed recurrent short-lasting shock-like pain involving her left lower face. Initiation of amlodipine for concurrently detected hypertension relieved her of pain besides normalizing her blood pressure. Interestingly, occasions of noncompliance with amlodipine brought her pain back. Her cranial MRI revealed tortuous blood vessels compressing the left rostro-antero-lateral medulla and left trigeminal nerve root (figure 1). Vascular compression of medulla is known to produce neurogenic hypertension1 (figure e-1 at Neurology.org). Possibly, a decrease in vessel tortuosity2 mediated by voltage-gated L-ty...
Source: Neurology - July 17, 2017 Category: Neurology Authors: Uniyal, R., Paliwal, V. K., Kumar, N., Malhotra, H. S., Garg, R. K., Neyaz, Z. Tags: MRI, Trigeminal neuralgia RESIDENT AND FELLOW SECTION Source Type: research

International Issues: Teleneurology in humanitarian crises: Lessons from the Medecins Sans Frontieres experience
We describe our experiences as voluntary neurology teleconsultants with Médecins Sans Frontières in order to highlight both the promises and challenges of teleneurology in humanitarian contexts. We identified the major advantages of this service as (1) minimal resources and incurred costs while (2) changing a patient's clinical course favorably, and (3) creating a community for the field referrer and neurology specialist. Current challenges include (1) limited diagnostic resources and difficult diagnostic and therapeutic decision-making, (2) need for greater continuity and familiarity between the field site a...
Source: Neurology - July 17, 2017 Category: Neurology Authors: Saadi, A., Mateen, F. J. Tags: RESIDENT AND FELLOW SECTION Source Type: research

Autologous hematopoietic stem cell transplantation in multiple sclerosis: A meta-analysis
(Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research

Revisiting neurofibromatosis type 2 diagnostic criteria to exclude LZTR1-related schwannomatosis
(Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research

Author update: Sarcoplasmic MxA expression: A valuable marker of dermatomyositis
Soon after our article published,1 we learned the sale of the myxovirus resistance A (MxA) polyclonal antibodies used in the study (Mx1/2/3 [H-285], sc-50509, Santa Cruz Biotechnology, Dallas, TX) had been discontinued. Furthermore, we received inquiries from several physicians concerning alternate MxA antibodies. We tested the company's monoclonal antibody alternate (Mx1/2/3 [C-1], sc-166412) on frozen muscle sections at various dilutions in 2% bovine serum albumin in PBS using the Ventana immunohistochemistry detection system (Ventana Medical Systems, Tucson, AZ) with or without the enhancement mode. Muscle samples teste...
Source: Neurology - July 10, 2017 Category: Neurology Authors: Uruha, A., Suzuki, S., Nishino, I. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Hand postures in primary and secondary generalized tonic-clonic seizures
We thank Dr. Mintzer for the interest in our article1 and for the comments on the statistical significance of our findings in response to our prior comment to Dr. Lanska.2,3 (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Siegel, J., Tatum, W. O. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Hand postures in primary and secondary generalized tonic-clonic seizures
I congratulate Drs. Siegel and Tatum1 for the novel examination of hand postures in different seizure types. However, I have concerns that the statistical analysis was not done properly. (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Mintzer, S. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In response to the article "Hand postures in primary and secondary generalized tonic-clonic seizures," Dr. Mintzer explains why the statistical methodology employed by the authors, Fisher exact test with Bonferroni correction for multiple comparisons, was not ideal and that the version for multiple groups would have been preferable. (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Alcauskas, M., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Dramatic improvement of tardive dyskinesia movements by inline skating
A 25-year-old woman with severe tardive dyskinesia (TD) due to neuroleptics had substantial improvement of movements while inline skating (video at Neurology.org). She received pallidal deep brain stimulation (DBS), and gait and inline skating were assessed before and after DBS; her twin sister served as a control (figures 1 and 2). Possible explanations for her improvement include (1) balance stability required by inline skating provides external cues that are less prominent during gait1; and (2) dystonia consistently responds to geste antagoniste.2 Since TD has variable response to treatments, we propose research into al...
Source: Neurology - July 10, 2017 Category: Neurology Authors: Casagrande, S. C. B., Cury, R. G., de Lima-Pardini, A. C., Coelho, D. B., Souza, C. d. O., dos Santos Ghilardi, M. G., Silveira-Moriyama, L., Teixeira, L. A., Barbosa, E. R., Fonoff, E. T. Tags: Gait disorders/ataxia, Surgery/Stimulation, Motor Control VIDEO NEUROIMAGES Source Type: research

The entire world
(Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Provencher, L. Tags: Medical care, Methods of education, Professional conduct and ethics REFLECTIONS: NEUROLOGY AND THE HUMANITIES Source Type: research

Failure of alemtuzumab therapy to control MOG encephalomyelitis
Myelin oligodendrocyte glycoprotein (MOG) immunoglobulin G (IgG)–associated encephalomyelitis (EM) is a rare autoimmune disorder that displays substantial clinicoradiologic overlap with aquaporin-4 (AQP4)-IgG-seropositive neuromyelitis optica spectrum disorders (NMOSD) and classic multiple sclerosis (MS).1–3,e1 The long-term outcome is often poor.2,3,e1 Recent evidence suggests that many disease-modifying agents approved for the treatment of MS may be ineffective or even harmful in AQP4-IgG-positive and MOG-IgG-positive patients.2–4 (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Wildemann, B., Jarius, S., Schwarz, A., Diem, R., Viehöver, A., Hähnel, S., Reindl, M., Korporal-Kuhnke, M. Tags: MRI, Autoimmune diseases, Multiple sclerosis, Devic's syndrome CLINICAL/SCIENTIFIC NOTES Source Type: research

Patients with ALS show highly correlated progression rates in left and right limb muscles
Conclusions: First, this study demonstrates a high degree of power when using unilateral treatment approaches to detect a slowing in disease progression in smaller groups of patients, thus allowing for paired statistical tests. These findings will be useful in transplantation trials that use muscle decline to track disease progression in ALS. Second, these findings discuss methods, such as tactical selection of muscle groups, which can improve the power efficiency of all ALS clinical trials. (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Rushton, D. J., Andres, P. L., Allred, P., Baloh, R. H., Svendsen, C. N. Tags: All Clinical Neurology, Amyotrophic lateral sclerosis ARTICLE Source Type: research

A propensity score analysis for comparison of T-3b and VATET in myasthenia gravis
Objective: We performed propensity score (PS) models to compare the outcome of patients with myasthenia gravis (MG) submitted to 2 different surgical approaches: extended transsternal (T-3b) or thoracoscopic extended thymectomy (VATET). Methods: Patients' clinical data were retrieved from the MG database of the C. Besta Neurologic Institute Foundation. In the PS analysis, a matching ratio of 1:1 of the main clinical variables was obtained for the 2 groups of patients and treatment effect was estimated by comparing their outcome. Results: A total of 210 patients met the inclusion criteria, by having a complete set of clin...
Source: Neurology - July 10, 2017 Category: Neurology Authors: Brenna, G., Antozzi, C., Montomoli, C., Baggi, F., Mantegazza, R., For the INCB-MG Group Tags: Autoimmune diseases, Myasthenia, Clinical trials Observational study (Cohort, Case control) ARTICLE Source Type: research

CSF sAPP{beta}, YKL-40, and neurofilament light in frontotemporal lobar degeneration
Conclusions: The combination of sAPPβ with YKL-40 and with NfL in CSF could be useful to increase the certainty of the diagnosis of FTLD-related syndromes in clinical practice. Classification of evidence: This study provides Class III evidence that CSF levels of sAPPβ, YKL-40, and NfL are useful to identify patients with FTLD-related syndromes. (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Alcolea, D., Vilaplana, E., Suarez-Calvet, M., Illan-Gala, I., Blesa, R., Clarimon, J., Llado, A., Sanchez-Valle, R., Molinuevo, J. L., Garcia-Ribas, G., Compta, Y., Marti, M. J., Pinol-Ripoll, G., Amer-Ferrer, G., Noguera, A., Garcia-Martin, A., Fortea, Tags: MRI, Frontotemporal dementia, Cerebrospinal Fluid, Cognitive aging, Assessment of cognitive disorders/dementia ARTICLE Source Type: research

The incidence of SUDEP: A nationwide population-based cohort study
Objective: To identify all cases of sudden unexpected death in epilepsy (SUDEP) among people in Sweden during 1 year and to determine the SUDEP incidence in relation to age, sex, and psychiatric comorbidity. Methods: We included all individuals with a hospital-based ambulatory care or hospital discharge diagnosis of epilepsy in the Swedish National Patient Registry during 1998–2005 who were alive on January 1, 2008. Deaths during 2008 were identified by linkage to the National Cause of Death Registry. Death certificates, medical charts, and police and autopsy reports were extensively reviewed to identify SUDEP cases...
Source: Neurology - July 10, 2017 Category: Neurology Authors: Sveinsson, O., Andersson, T., Carlsson, S., Tomson, T. Tags: Incidence studies, All Epilepsy/Seizures ARTICLE Source Type: research

Modulation of intrinsic resting-state fMRI networks in women with chronic migraine
Conclusions: Our results demonstrated decreased overall resting-state functional connectivity of the 3 major intrinsic brain networks in women with CM, and these patterns were associated with frequency of moderate to severe headache and cutaneous allodynia. (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Androulakis, X. M., Krebs, K., Peterlin, B. L., Zhang, T., Maleki, N., Sen, S., Rorden, C., Herath, P. Tags: fMRI ARTICLE Source Type: research

Comment: The virtual neurologist
In 1999, Levine and Gorman1 introduced telestroke as the first major application of telemedicine to neurology. Today telestroke is standard care globally. Now, Mùˆller et al.2 have conducted one of the largest teleneurology clinical trials for nonacute headaches. (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Dorsey, E. R. Tags: All Headache, Outcome research, Clinical trials Randomized controlled (CONSORT agreement), Patient safety ARTICLE Source Type: research

A randomized trial of telemedicine efficacy and safety for nonacute headaches
Conclusion: Telemedicine consultation for nonacute headache is as efficient and safe as a traditional consultation. ClinicalTrials.gov identifier: NCT02270177. Classification of evidence: This study provides Class III evidence that a one-time telemedicine consultation for nonacute headache is noninferior to a one-time traditional consultation regarding long-term treatment outcome and safety. (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Müller, K. I., Alstadhaug, K. B., Bekkelund, S. I. Tags: All Headache, Outcome research, Clinical trials Randomized controlled (CONSORT agreement), Patient safety ARTICLE Source Type: research

Standard operating procedures improve acute neurologic care in a sub-Saharan African setting
Objective: Quality of neurologic emergency management in an under-resourced country may be improved by standard operating procedures (SOPs). Methods: Neurologic SOPs were implemented in a large urban (Banjul) and a small rural (Brikama) hospital in the Gambia. As quality indicators of neurologic emergency management, performance of key procedures was assessed at baseline and in the first and second implementation years. Results: At Banjul, 100 patients of the first-year intervention group exhibited higher rates of general procedures of emergency management than 105 control patients, such as neurologic examination (99.0% vs 91.4%; p
Source: Neurology - July 10, 2017 Category: Neurology Authors: Jaiteh, L. E. S., Helwig, S. A., Jagne, A., Ragoschke-Schumm, A., Sarr, C., Walter, S., Lesmeister, M., Manitz, M., Blass, S., Weis, S., Schlund, V., Bah, N., Kauffmann, J., Fousse, M., Kangankan, S., Ramos Cabrera, A., Kronfeld, K., Ruckes, C., Liu, Y., Tags: ARTICLE Source Type: research

Baseline motor findings and Parkinson disease prognostic subtypes
Conclusions: Our data show that motor profile at baseline can predict prognosis in most PD cases. These findings can be incorporated into clinical practice. (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Rajput, A. H., Rajput, M. L., Ferguson, L. W., Rajput, A. Tags: Parkinson's disease/Parkinsonism ARTICLE Source Type: research

Outcomes of Colorado children with acute flaccid myelitis at 1 year
Conclusions: At 1 year, children with AFM demonstrated functional gains but weakness persisted. EMG changes correlated with persistent deficits better than imaging. Despite improvements, AFM had substantial long-term functional effects on affected children. (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Martin, J. A., Messacar, K., Yang, M. L., Maloney, J. A., Lindwall, J., Carry, T., Kenyon, P., Sillau, S. H., Oleszek, J., Tyler, K. L., Dominguez, S. R., Schreiner, T. L. Tags: Viral infections, Prognosis, Anterior nerve cell disease, All Pediatric, EMG ARTICLE Source Type: research

Coated-platelets predict stroke at 30 days following TIA
Objective: To examine the potential for coated-platelets, a subset of highly procoagulant platelets observed on dual agonist stimulation with collagen and thrombin, for predicting stroke at 30 days in patients with TIA. Methods: Consecutive patients with TIA were enrolled and followed up prospectively. ABCD2 scores were obtained for each patient. Coated-platelet levels, reported as percent of cells converted to coated-platelets, were determined at baseline. The primary endpoint was the occurrence of stroke at 30 days. Receiver operator characteristic (ROC) analysis was used to calculate area under the curve (AUC) values f...
Source: Neurology - July 10, 2017 Category: Neurology Authors: Kirkpatrick, A. C., Vincent, A. S., Dale, G. L., Prodan, C. I. Tags: All Cerebrovascular disease/Stroke, Infarction ARTICLE Source Type: research

Structural MRI markers of brain aging early after ischemic stroke
Conclusions: Brain structure is likely to be compromised before ischemic stroke by vascular risk factors. Smaller hippocampal and total brain volumes and increased WMH load represent proxies for underlying vascular brain injury. (Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Werden, E., Cumming, T., Li, Q., Bird, L., Veldsman, M., Pardoe, H. R., Jackson, G., Donnan, G. A., Brodtmann, A. Tags: MRI, All Cerebrovascular disease/Stroke, Clinical trials Observational study (Cohort, Case control), Alzheimer's disease, Vascular dementia ARTICLE Source Type: research

SUDEP: An important cause of premature mortality in epilepsy across the life spectrum
Sudden unexpected death in epilepsy (SUDEP) was originally defined as a sudden, unexpected death in an individual with epilepsy, witnessed or not, and not associated with drowning or status epilepticus, for which a cause cannot be identified upon autopsy examination.1 Cases can be divided into definite, probable, and possible based upon the level of diagnostic certainty, especially after postmortem examination.2 As research criteria have evolved, a more comprehensive set of diagnostic criteria has been derived, including the term plus to indicate that another cause could have resulted in death, e.g., SUDEP probable/plus.3 ...
Source: Neurology - July 10, 2017 Category: Neurology Authors: Buchhalter, J., Cascino, G. D. Tags: EDITORIALS Source Type: research

Acute flaccid myelitis and enterovirus D68: Deja vu all over again
In 2012, illnesses presenting with acute flaccid paralysis were reported in the United States—over 150 cases between 2012 and 2015—with a clinical picture of a prodromal illness followed by rapid onset of weakness and cranial nerve dysfunction in association with CSF pleocytosis and elevated protein level. MRI showed abnormal spinal cord gray matter signal. Treatment with IV immunoglobulin, plasmapheresis, steroids, and antiviral agents had no effect on the natural history of what was named acute flaccid myelitis (AFM). Epidemiologic analysis later showed an association with an enterovirus D68, which was groupe...
Source: Neurology - July 10, 2017 Category: Neurology Authors: Wiznitzer, M., Nath, A. Tags: Viral infections, Prognosis, Anterior nerve cell disease, All Pediatric, EMG EDITORIALS Source Type: research

Cerebrovascular disease affects brain structural integrity long before clinically overt strokes
In the current issue of Neurology®, Werden et al.1 examined a group of patients experiencing first-ever or recurrent stroke to understand the relationships between stroke and cortical structural integrity. They performed 3T magnetic resonance scanning within 6 weeks of the most recent stroke and found that stroke patients had smaller hippocampal volumes and greater white matter hyperintensity (WMH) volume compared to controls. Because of the short time lag from stroke to scan, the authors do not believe that the index stroke itself caused the shrinkage in the hippocampus; rather, they believe that the stroke and the st...
Source: Neurology - July 10, 2017 Category: Neurology Authors: Knopman, D. S., Hooshmand, B. Tags: MRI, All Cerebrovascular disease/Stroke, Clinical trials Observational study (Cohort, Case control), Alzheimer's disease, Vascular dementia EDITORIALS Source Type: research

Spotlight on the July 11 issue
(Source: Neurology)
Source: Neurology - July 10, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Teaching Video NeuroImages: Apraxia of eyelid closure following right hemispheric infarction
A 56-year-old man presented following a right middle cerebral artery territory infarction (figure). In addition to left hemiparesis and neglect, he was unable to close his left eye on command (video at Neurology.org), though he was able to blink spontaneously and keep his eyes closed during sleep. He could maintain closure of both eyes once manually closed. Apraxia of eyelid closure is distinguished clinically from eyelid closure weakness by ability to blink reflexively and maintain eyelid closure. Activation of the oculomotor cortex has been associated with voluntary eyelid closure and lesions in right frontal and parieta...
Source: Neurology - July 10, 2017 Category: Neurology Authors: Thon, J. M., Grossmann, L., Bhattacharyya, S. Tags: Clinical neurology examination, All Neuro-ophthalmology, Eyelids, Infarction RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Osteochondroma arising from the clavicle causing ipsilateral Horner syndrome
A 17-year-old female smoker noticed right eyelid droop for 6 months, with a constant right-sided pressure-like headache with photophobia, phonophobia, and nausea, right-sided lacrimation, and right-sided rhinorrhea. Examination revealed right Horner syndrome. A mass was palpable on the anterior aspect of her neck. Ultrasound of the neck, MRI head, and CT thorax showed a bony mass arising from the right medial clavicular head (figure 1). Histopathology from the excision confirmed an osteochondroma (figure 2). This was removed surgically with partial resolution of symptoms. This case demonstrates a rare cause of Horner syndr...
Source: Neurology - July 10, 2017 Category: Neurology Authors: Wei, D. Y., Bradley, M., Orosz, Z., Stevens, J. C. Tags: CT, MRI, All Clinical Neurology, Pupils RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A 54-year-old woman with dementia, myoclonus, and ataxia
A 54-year-old woman was referred for a second opinion regarding a 3-month history of subacute onset progressive cognitive decline. She complained of memory difficulties particularly with short term recall. Decision-making and organizational skills were increasingly difficult. She also developed sudden brief jerking movements of her body and progressive gait imbalance and incoordination leading to falls. Three months after onset, she could not work, drive, cook, or perform activities of daily living. She had not had episodes suggestive of seizures. Her medical history was remarkable for celiac disease diagnosed 9 years prev...
Source: Neurology - July 10, 2017 Category: Neurology Authors: Ali, F., Murray, J. A., Adams, A. C., Flanagan, E. P. Tags: Autoimmune diseases, All Clinical Neurology, All Toxicology, Assessment of cognitive disorders/dementia RESIDENT AND FELLOW SECTION Source Type: research

Decrease in A{beta}42 predicts dopa-resistant gait progression in early Parkinson disease
(Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research

Prespecified dose-response analysis for A Very Early Rehabilitation Trial (AVERT)
(Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research

Author response: Mediodorsal nucleus and its multiple cognitive functions
We thank Dr. Onofrj for the comments on our review,1 and for highlighting past literature that suggested the mediodorsal nucleus of the thalamus may also be involved in the development of confabulations in addition to the clinical features described in our case of an isolated left-sided lesion. Indeed, Dr. Onofrj's group's recent clinical case of bilateral lesions of the mediodorsal thalamic nuclei and the associated imaging data lend further support to this concept. Their study and case series would interestingly suggest a possible laterality to the role of the thalamus in the phenomenon of confabulations and provide valu...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Golden, E. C., Graff-Radford, J., Jones, D. T., Benarroch, E. E. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Mediodorsal nucleus and its multiple cognitive functions
The review on mediodorsal (thalamic) nucleus by Golden et al.1 omitted referencing relevant information. Mediodorsal nucleus was the focus of a historic debate on the origin of confabulations in Korsakoff syndrome,2–4 which is characterized by confabulations (beyond amnesia) only if mediodorsal nuclei are involved.2–4 Clinical findings observed in isolated lacunes of mediodorsal nuclei could elucidate the point, yet isolated lacunes, like the one described in the representative case,1 are extraordinarily rare. (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Onofrj, M. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Long-term cortisol measures predict Alzheimer disease risk
I thank Drs. Lattanzi and Silvestrini for the thoughtful response to our article.1 I fully agree with their suggested mechanisms of action by which cortisol may increase risk for AD. In our sample, we had very few non-AD dementias, which precluded a more comprehensive assessment of how cortisol dysregulation may affect risk for other dementias, though it is a fascinating question. (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Moffat, S. D. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Long-term cortisol measures predict Alzheimer disease risk
We read with interest the article by Ennis et al.,1 which found cortisol dysregulation to be related to an increased risk for Alzheimer disease (AD), and built on the unresolved question of whether systemic homeostasis primarily contributes to AD expression or represents an epiphenomenon of the underlying brain pathology. Additional considerations might provide useful insights toward a better and more comprehensive understanding of this issue. Within their pleiotropic effects, corticosteroids can greatly influence metabolic functions as well as blood pressure levels and fluctuations, all of which play key roles in dementia...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Lattanzi, S., Silvestrini, M. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: Commenting on "Long-term cortisol measures predict Alzheimer disease risk," Drs. Lattanzi and Silvestrini point out the interrelationships among cortisol dysregulation, insulin resistance, and blood pressure variability in Alzheimer disease (AD) and suggest that the authors study the association between cortisol exposure and the risk of non-AD dementias. Dr. Onofrj critiques "Mediodorsal nucleus and its multiple cognitive functions" because it omitted discussion of confabulations. (Source: Neurology)
Source: Neurology - July 3, 2017 Category: Neurology Authors: Karam, C., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Proatlantal intersegmental artery with internal carotid artery stenosis
A 56-year-old man presented with vertigo of 4 days duration. Neurologic examination was unremarkable. CT angiograms revealed a right internal carotid artery (ICA) stenosis and an ipsilateral proatlantal intersegmental artery (PIA, type 1). The PIA arose from the ICA, ran upward, then took a dorsal course, and continued as the vertebral artery, serving as the major contributor of the posterior circulation (figure, A–C). Catheter-based angiogram showed ICA stenosis of 60% (figure, D). He chose drug therapy and remained asymptomatic on follow-up. Such combination can lead to TIA of the vertebrobasilar system; treatment ...
Source: Neurology - July 3, 2017 Category: Neurology Authors: Yu, W., Feng, Z., Zhao, C., Fu, C. Tags: CT, All Clinical Neurology, All Cerebrovascular disease/Stroke NEUROIMAGES Source Type: research