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Sex differences in Alzheimer risk: Brain imaging of endocrine vs chronologic aging
Objective: This observational multimodality brain imaging study investigates emergence of endophenotypes of late-onset Alzheimer disease (AD) risk during endocrine transition states in a cohort of clinically and cognitively normal women and age-matched men. Methods: Forty-two 40- to 60-year-old cognitively normal women (15 asymptomatic perimenopausal by age [CNT], 13 perimenopausal [PERI], and 14 postmenopausal [MENO]) and 18 age- and education-matched men were examined. All patients had volumetric MRI, 18F-fluoro-2-deoxyglucose (FDG)–PET (glucose metabolism), and Pittsburgh compound B–PET scans (β-amyloi...
Source: Neurology - September 25, 2017 Category: Neurology Authors: Mosconi, L., Berti, V., Quinn, C., McHugh, P., Petrongolo, G., Varsavsky, I., Osorio, R. S., Pupi, A., Vallabhajosula, S., Isaacson, R. S., de Leon, M. J., Brinton, R. D. Tags: PET, Alzheimer's disease, Cognitive aging ARTICLE Source Type: research

Brain network efficiency is influenced by the pathologic source of corticobasal syndrome
Conclusions: Our results indicate that the underlying pathologic sources of corticobasal syndrome can be classified more accurately using graph theoretical statistics derived from patterns of white matter network organization in association cortex than by regional gray matter density alone. These results highlight the importance of a multimodal neuroimaging approach to diagnostic analyses of corticobasal syndrome. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Medaglia, J. D., Huang, W., Segarra, S., Olm, C., Gee, J., Grossman, M., Ribeiro, A., McMillan, C. T., Bassett, D. S. Tags: Alzheimer's disease, Frontotemporal dementia, Corticobasal degeneration, MCI (mild cognitive impairment) ARTICLE Source Type: research

Developmental outcomes of cord blood transplantation for Krabbe disease: A 15-year study
Conclusions: The surviving patients function at a much higher level than untreated children or symptomatic children who underwent HSCT. These results show that early HSCT changes the natural history of this disease by improving both lifespan and functional abilities. Classification of evidence: This study provides Class IV evidence that for children with early-infantile Krabbe disease, early HSCT improves lifespan and functional abilities. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Wright, M. D., Poe, M. D., DeRenzo, A., Haldal, S., Escolar, M. L. Tags: Leukodystrophies, Developmental disorders, All Demyelinating disease (CNS) ARTICLE Source Type: research

A natural history study of X-linked myotubular myopathy
Conclusions: MTM is one of the most severe neuromuscular disorders, with affected individuals requiring extensive mechanical interventions for survival. However, among study participants, the disease course was more stable than predicted, with more individuals surviving infancy and early childhood. These data reflect the disease burden of MTM but offer hope in terms of future therapeutic intervention. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Amburgey, K., Tsuchiya, E., de Chastonay, S., Glueck, M., Alverez, R., Nguyen, C.-T., Rutkowski, A., Hornyak, J., Beggs, A. H., Dowling, J. J. Tags: Muscle disease, Natural history studies (prognosis) ARTICLE Source Type: research

High frequency of gastrointestinal manifestations in myotonic dystrophy type 1 and type 2
Conclusions: GI manifestations were common in both DM1 and DM2, with a relatively high frequency of gallbladder removal in DM1 and DM2 occurring at a younger age compared to normative data in the literature. Studies are needed to determine the pathomechanism of how sex, weight gain, and duration of disease contribute to GI manifestations and how these manifestations affect quality of life and clinical care for patients with DM1 and DM2. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Hilbert, J. E., Barohn, R. J., Clemens, P. R., Luebbe, E. A., Martens, W. B., McDermott, M. P., Parkhill, A. L., Tawil, R., Thornton, C. A., Moxley, R. T., On behalf of the National Registry Scientific Advisory Committee/Investigators Tags: All Clinical Neurology, Gastrointestinal, Muscle disease ARTICLE Source Type: research

Neuroinflammation drives anxiety and depression in relapsing-remitting multiple sclerosis
Conclusions: Mood alterations are induced by intrathecal inflammation, even though not clinically apparent, and are able to predict inflammatory reactivations in RRMS. Inflammation is therefore a biological event, not less important than the traditional psychosocial factors, involved in mood disorders. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Rossi, S., Studer, V., Motta, C., Polidoro, S., Perugini, J., Macchiarulo, G., Giovannetti, A. M., Pareja-Gutierrez, L., Calo, A., Colonna, I., Furlan, R., Martino, G., Centonze, D. Tags: All Psychiatric disorders, Depression, Multiple sclerosis ARTICLE Source Type: research

Epstein-Barr virus, cytomegalovirus, and multiple sclerosis susceptibility: A multiethnic study
Objective: To determine whether Epstein-Barr virus (EBV) or cytomegalovirus (CMV) seropositivity is associated with multiple sclerosis (MS) in blacks and Hispanics and to what extent measures of the hygiene hypothesis or breastfeeding could explain these findings. EBV and CMV have been associated with MS risk in whites, and the timing and frequency of both viruses vary by factors implicated in the hygiene hypothesis. Methods: Incident cases of MS or its precursor, clinically isolated syndrome (CIS), and matched controls (blacks, 111 cases/128 controls; Hispanics, 173/187; whites, 235/256) were recruited from the membershi...
Source: Neurology - September 25, 2017 Category: Neurology Authors: Langer-Gould, A., Wu, J., Lucas, R., Smith, J., Gonzales, E., Amezcua, L., Haraszti, S., Chen, L. H., Quach, H., James, J. A., Barcellos, L. F., Xiang, A. H. Tags: Multiple sclerosis, Case control studies, Risk factors in epidemiology ARTICLE Source Type: research

No association between dietary sodium intake and the risk of multiple sclerosis
Conclusions: Our findings suggest that higher dietary sodium intake does not increase the risk of developing MS. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Cortese, M., Yuan, C., Chitnis, T., Ascherio, A., Munger, K. L. Tags: Multiple sclerosis, All epidemiology, Cohort studies, Risk factors in epidemiology ARTICLE Source Type: research

Aneurysm rupture: Another reason to abstain from smoking
Smoking independently increases risk for aneurysm rupture and aneurysm formation.1,2 It may weaken the wall of intracranial aneurysms due to inflammation, making them more vulnerable to trigger factors and therefore to rupture.3 Previous studies have assessed the effect of current smoking. In this issue of Neurology®, Can et al.4 assess the effect of both smoking intensity and duration on intracranial aneurysm rupture. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Buis, D. R., Batjer, H. H. Tags: EDITORIALS Source Type: research

Stem cell transplantation in Krabbe disease: New truths discovered and opinions change
...Institutions must go hand in hand with the progress of the human mind. As that becomes more developed, more enlightened, as new discoveries are made, new truths discovered and manners and opinions change, with the change of circumstances, institutions must advance also to keep pace with the times. —Thomas Jefferson, July 12, 1816 (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Graf, W. D. Tags: Metabolic disease (inherited), Leukodystrophies, All Demyelinating disease (CNS), All Genetics EDITORIALS Source Type: research

X-linked myotubular myopathy: Living longer and awaiting treatment
The congenital myopathies (CMs) are a cluster of genetic disorders affecting myofiber structure and function. The salient clinical features are hypotonia, weakness, and motor impairment. The presentation varies from the most severe impairment in the newborn to a range of phenotypes in infancy ("floppy baby"), childhood, or occasionally adulthood. The prevalence of CMs is 1:26,000 children (birth to 17 years).1 The CMs were named on the basis of distinctive morphologic abnormalities seen on muscle biopsy: nemaline myopathy, with rods due to protein accumulation; core myopathy, with cores or multiminicores devoid o...
Source: Neurology - September 25, 2017 Category: Neurology Authors: Finkel, R. S., Darras, B. T. Tags: EDITORIALS Source Type: research

Dietary sodium intake: An etiologic dead end in multiple sclerosis
Multiple sclerosis (MS) is a disease of complex etiology with many unknown, hidden elements. Apart from strong genetic influence working at population and individual levels, environmental factors are important as they are essential to understanding and explaining the true increase in incidence observed in many places since the middle of the 20th century.1,2 In recent decades, environmental factors, some of them associated with lifestyle, have been elaborated, e.g., cigarette smoking, obesity, and vitamin D shortage3 and other dietary factors.4 Among possible factors attracting recent attention is dietary sodium intake. Sod...
Source: Neurology - September 25, 2017 Category: Neurology Authors: Koch-Henriksen, N., Lauer, K. Tags: EDITORIALS Source Type: research

Spotlight on the September 26 issue
(Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Multiple sclerosis, inflammation in the brain, and mood
In their study, "Neuroinflammation drives anxiety and depression in relapsing-remitting multiple sclerosis," Dr. Rossi et al.1 investigated the relationship between mood and inflammation. They did this by looking at a group of people who have multiple sclerosis (MS). It has long been observed that inflammation occurs in the brains and spinal cords of people with a specific kind of MS called relapsing-remitting MS. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Karceski, S. Tags: PATIENT PAGES Source Type: research

Teaching Video NeuroImages: Olivary enlargement and pharyngeal nystagmus
A 77-year-old woman diagnosed with a pontine cavernoma developed progressive difficulty swallowing. A videofluoroscopic swallowing study showed low-frequency rhythmic contractions of the soft palate and upper larynx (video at Neurology.org). Brain MRI revealed hypertrophy in the right inferior olivary nucleus (figure). (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Finlay, J. B., Yacovino, D. A. Tags: MRI, Clinical neurology examination, Nystagmus RESIDENT AND FELLOW SECTION Source Type: research

Teaching NeuroImages: Wallerian degeneration in evolving pediatric stroke
An 8-year-old girl presented with acute hemiparesis and facial palsy. MRI demonstrated right middle cerebral artery territory infarction (figure, A and B), secondary to traumatic dissection. Following discharge, multiple visits for nonspecific neurologic symptoms prompted repeat short-term imaging, initially concerning for right midbrain infarction (figure, C–H). (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Jimenez-Gomez, A., Stowe, R. C. Tags: Childhood stroke, MRI, DWI, Other Education RESIDENT AND FELLOW SECTION Source Type: research

Mystery Case: Diagnostic challenges in a young patient with hypereosinophilia
A 48-year-old woman with recent diagnosis of nonischemic cardiomyopathy and longstanding history of asthma and allergic rhinitis without additional vascular risk factors had intermittent chest pain and dyspnea for 6 weeks, treated with antibiotics and oral steroids without benefit. Subsequently, she developed bilateral leg edema, orthopnea, and chest pain, and was hospitalized twice at another institution. Transthoracic echocardiogram (TTE) demonstrated an ejection fraction (EF) of 30%. Cardiac catheterization was normal. CT of the chest showed a large pericardial effusion (~300 mL) and bilateral pleural effusions. She had...
Source: Neurology - September 25, 2017 Category: Neurology Authors: Ortiz, J. G., Douglas, P. W., Gill, C. E., Mehrotra, S., Biller, J. Tags: Stroke in young adults, Infarction RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A case of altered mental status, not otherwise specified
A 33-year-old right-handed woman presented with a 1-week history of rapid cognitive decline. The behavioral changes emerged gradually, with the first sign marked by erratic driving resulting in arrest. The patient was nonchalant about the detainment, which was out of character. In the preceding week, she had a precipitous decline in cognitive abilities, including a loss of interest in grooming, emotional outbursts, fecal and urinary incontinence, difficulty performing household chores, and jerky movements of her arms and legs impairing her ability to walk. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Swor, D. E., Sharp, F. R., Jickling, G. C. Tags: MRI, All Clinical Neurology, Clinical neurology examination, All Neuropsychology/Behavior, All Toxicology RESIDENT AND FELLOW SECTION Source Type: research

Doctors without weapons: "You people are pathetic"
"How can you come to a war and not bring a weapon? Every soldier must have a weapon. That's the rule!" My boss was emphatic. "You people are pathetic." (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Riggs, J. E. Tags: All Clinical Neurology, All Trauma, All Ethics in Neurology/Legal issues REFLECTIONS: NEUROLOGY AND THE HUMANITIES Source Type: research

Does compensatory hyperparathyroidism predispose to ischemic stroke? Decreased bone mass and increased bone turnover with valproate therapy in adults with epilepsy; An alternative to vitamin D supplementation to prevent fractures in patients with MS; High prevalence of vitamin D deficiency and reduced bone mass in Parkinson's disease
Because of known issues leading to several retractions of papers by Y. Sato et al., the editors of Neurology® have, as a matter of due diligence, researched other papers that this group published in Neurology. To ensure that the scientific literature is correct, the Editor chooses to publish an Expression of Concern regarding 3 observational studies and a Letter to the Editor published in Neurology prior to the retracted clinical trials.1–4 (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Tags: EXPRESSION OF CONCERN Source Type: research

Mystery Case: CSF-1R mutation is a cause of intracranial cerebral calcifications, cysts, and leukoencephalopathy
The authors retract the article "Mystery Case: CSF-1R mutation is a cause of intracranial cerebral calcifications, cysts, and leukoencephalopathy" by X. Ayrignac et al., which appeared in the Resident & Fellow Section in volume 86, page e262, because 4 of the figures were previously published in another journal (European Neurology 2012;67:151–153).2 The earlier article was not cited and there were 3 common authors (Magnin, Berger, and Labauge) on the papers. In addition to the duplicate publication, 1 image attributed to the index case in this article was attributed to his mother in the earlier article ...
Source: Neurology - September 18, 2017 Category: Neurology Tags: RETRACTION Source Type: research

Author response: The autism "epidemic": Ethical, legal, and social issues in a developmental spectrum disorder
We thank Machado et al. for the comments on our review,1 and for the observations of abnormal anatomic and functional connectivity using diffusion-weighted MRI (DW-MRI). Innumerable studies have compared patients with autism spectrum disorders (ASD) and nonaffected controls at rest or while performing particular tasks, most often in small selected subject samples. For example, studies linked autism with the retino-collicular magnocellular visual pathways that project via the pulvinar to the amygdala, occipital V1, and multiple other areas, including the dorsal occipito-parietal/midtemporal streams, which participate with t...
Source: Neurology - September 18, 2017 Category: Neurology Authors: Graf, W. D., Miller, G., Epstein, L. G., Rapin, I. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: The autism "epidemic": Ethical, legal, and social issues in a developmental spectrum disorder
Graf et al.1 wrote an interesting review on autism spectrum disorder (ASD), emphasizing the term "autism epidemic," supported by an editorial.2 Among other factors, increasing ASD diagnoses can be attributed to broader diagnostic criteria and the recognition of ASD existence differentiated from other neurodevelopmental diseases.2–4 One important issue, defended by the authors, is early screening and diagnosis with emphasis on the evolving practice of genetic testing for ASD.1 (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Machado, C., Estevez, M., Rodriguez, R., Leisman, G. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Long-term cerebral white and gray matter changes after preeclampsia
We thank Dr. Culebras for the comment on our article.1 Sleep apnea might be among the pathophysiologic pathways linking preeclampsia with increased risk of cerebrovascular disease in previously preeclamptic women. In fact, research recently focused on the role of sleep apnea in the etiopathogenesis of cerebrovascular and cardiovascular disease and demonstrated an increased stroke risk in patients with obstructive sleep apnea (OSA).2,3 Intermittent hypoxia with consecutive vascular dysregulation has been identified as a possible mechanism whereby OSA increases risk of stroke. This might be relevant to previously preeclampti...
Source: Neurology - September 18, 2017 Category: Neurology Authors: Siepmann, T., Boardman, H., Bilderbeck, A., Griffanti, L., Kenworthy, Y., Zwager, C., McKean, D., Francis, J., Neubauer, S., Yu, G. Z., Lewandowski, A. J., Sverrisdottir, Y. B., Leeson, P. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Long-term cerebral white and gray matter changes after preeclampsia
The article by Siepmann et al.1 identified long-term cerebral white matter changes and reduced cortical volume, presumably of vascular origin, in young women after pregnancy complicated by preeclampsia. The authors invoked persistent inflammation as the putative agent. Sleep apnea may be a link among pregnancy, preeclampsia, and cerebrovascular disease. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Culebras, A. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In "Long-term cerebral white and gray matter changes after preeclampsia," the authors found that preeclampsia was associated with chronic temporal lobe white matter changes and reduced cortical volume in young women; the changes were consistent with persistent inflammation. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Alcauskas, M., Galetta, S. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Optical coherence tomography angiography in pituitary tumor
A 32-year-old man with a pituitary tumor had bitemporal hemianopia (figure 1). Peripapillary retinal nerve fiber layer (RNFL) in optical coherence tomography (OCT) was reduced, which corresponded to visual field defects. OCT angiography showed a dropout of capillaries and correlated well with RNFL loss in the OCT (figure 2). Compressive optic neuropathy may be associated with loss of the retinal ganglion cell layer and impaired peripapillary retinal perfusion. OCT angiography may be helpful to detect various optic neuropathies and in analyzing the vascular status of the optic nerve head and RNFL.1 (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Kim, K. H., Kim, U. S. Tags: All Imaging, Optic nerve, Visual loss NEUROIMAGES Source Type: research

Tetrabenazine and subthalamic stimulation in graft-induced dyskinesias
Putaminal fetal ventral mesencephalic stem cell transplantation has been advocated as a sustainable treatment for Parkinson disease (PD). Double-blind randomized clinical trials have inconclusively proven their benefit in the motor symptoms of PD despite evidence of graft viability, as shown by [18F]-fluorodopa PET.1 At variable intervals after grafting, 15%–57% of patients develop graft-induced dyskinesias (GID), which are characterized by violent, choreo-ballistic dyskinesias occurring regardless of the patient's medication state.2 GID are particularly difficult to manage: they do not respond to withdrawal of antip...
Source: Neurology - September 18, 2017 Category: Neurology Authors: Levy, A., Fasano, A. Tags: CLINICAL/SCIENTIFIC NOTES Source Type: research

Medication overuse headache: An entrenched idea in need of scrutiny
It is a widely accepted idea that medications taken to relieve acute headache pain can paradoxically worsen headache if used too often. This type of secondary headache is referred to as medication overuse headache (MOH); previously used terms include rebound headache and drug-induced headache. In the absence of consensus about the duration of use, amount, and type of medication needed to cause MOH, the default position is conservative. A common recommendation is to limit treatment to no more than 10 or 15 days per month (depending on medication type) to prevent headache frequency progression. Medication withdrawal is often...
Source: Neurology - September 18, 2017 Category: Neurology Authors: Scher, A. I., Rizzoli, P. B., Loder, E. W. Tags: Secondary headache disorders, All epidemiology, Natural history studies (prognosis) VIEWS & amp;amp; REVIEWS Source Type: research

Quality improvement in neurology: Essential Tremor Quality Measurement Set
Essential tremor (ET) is one of the most common movement disorders in the world, with prevalence rates in the general population ranging from 0.4% to 4.6%.1,2 The incidence of ET increases with age,1 with the average age at onset in mid-to-late 40s.3 ET is estimated to affect as many as 7 to 10 million Americans.3,4 Clinically, ET is characterized by bilateral, symmetric, postural tremor in hands and forearms, with or without kinetic tremor, in the absence of abnormal posturing or task specificity.5 ET can also affect lower extremities, head, and voice.6,7 Symptoms may be barely noticeable, or severe and disabling. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Zesiewicz, T. A., Sullivan, K. L., Ponce de Leon, M., Bennett, A., Hohler, A. D. Tags: All Health Services Research, All Movement Disorders, All Practice Management SPECIAL ARTICLE Source Type: research

A case-control study of hormonal exposures as etiologic factors for ALS in women: Euro-MOTOR
Conclusions: This large case-control study across 3 different countries has demonstrated an association between exogenous estrogens and progestogens and reduced odds of ALS in women. These results are at variance with previous findings, which may be partly explained by differential regulatory, social, and cultural attitudes toward pregnancy, birth control, and HRT across the countries included. Our results indicate that hormonal factors may be important etiologic factors in ALS; however, a full understanding requires further investigation. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Rooney, J. P. K., Visser, A. E., D'Ovidio, F., Vermeulen, R., Beghi, E., Chio, A., Veldink, J. H., Logroscino, G., van den Berg, L. H., Hardiman, O., For the Euro-MOTOR Consortium Tags: Amyotrophic lateral sclerosis, Case control studies, Risk factors in epidemiology ARTICLE Source Type: research

Human dorsal root ganglion in vivo morphometry and perfusion in Fabry painful neuropathy
Objective: To evaluate functional and morphometric magnetic resonance neurography of the dorsal root ganglion and peripheral nerve segments in patients with Fabry painful neuropathy. Methods: In this prospective study, the lumbosacral dorsal root ganglia and proximal peripheral nerve segments of the lower extremity were examined in 11 male patients with Fabry disease by a standardized 3T magnetic resonance neurography protocol. Volumes of L3 to S2 dorsal root ganglia, perfusion parameters of L5-S1 dorsal root ganglia and the spinal nerve L5, and the cross-sectional area of the proximal sciatic nerve were compared to healt...
Source: Neurology - September 18, 2017 Category: Neurology Authors: Godel, T., Bäumer, P., Pham, M., Köhn, A., Muschol, N., Kronlage, M., Kollmer, J., Heiland, S., Bendszus, M., Mautner, V.-F. Tags: MRI, Metabolic disease (inherited), Peripheral neuropathy, Neuropathic pain ARTICLE Source Type: research

MRI substrates of sustained attention system and cognitive impairment in pediatric MS patients
Conclusions: Our results suggest that the age-expected level of sustained attention system functional competence is achieved in patients with pediatric MS. Inefficient regulation of the functional interaction between different areas of this system, due to abnormal white matter integrity, may result in global cognitive impairment in these patients. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: De Meo, E., Moiola, L., Ghezzi, A., Veggiotti, P., Capra, R., Amato, M. P., Pagani, E., Fiorino, A., Pippolo, L., Pera, M. C., Comi, G., Falini, A., Filippi, M., Rocca, M. A. Tags: MRI, fMRI, Multiple sclerosis ARTICLE Source Type: research

Cognition and gray and white matter characteristics of presymptomatic C9orf72 repeat expansion
Conclusions: This study demonstrates that a decline in cognitive functioning, white matter integrity, and gray matter volumes are present in presymptomatic C9orf72RE carriers. These findings suggest that neuropsychological assessment, T1-weighted MRI, and diffusion tensor imaging might be useful to identify early biomarkers in the presymptomatic stage of FTD or amyotrophic lateral sclerosis. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Papma, J. M., Jiskoot, L. C., Panman, J. L., Dopper, E. G., den Heijer, T., Donker Kaat, L., Pijnenburg, Y. A. L., Meeter, L. H., van Minkelen, R., Rombouts, S. A. R. B., van Swieten, J. C. Tags: DWI, Volumetric MRI, Frontotemporal dementia ARTICLE Source Type: research

Effect of simvastatin on CSF Alzheimer disease biomarkers in cognitively normal adults
Conclusions: Simvastatin-related reductions in CSF p-tau181 concentrations may be modulated by LDL cholesterol. The potential disease-modifying effects of simvastatin on CSF phospho-tau should be further investigated in persons with hypercholesterolemia. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Li, G., Mayer, C. L., Morelli, D., Millard, S. P., Raskind, W. H., Petrie, E. C., Cherrier, M., Fagan, A. M., Raskind, M. A., Peskind, E. R. Tags: Clinical trials Randomized controlled (CONSORT agreement), Alzheimer's disease ARTICLE Source Type: research

Sleep architecture and the risk of incident dementia in the community
Conclusions: Despite contemporary interest in slow-wave sleep and dementia pathology, our findings implicate REM sleep mechanisms as predictors of clinical dementia. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Pase, M. P., Himali, J. J., Grima, N. A., Beiser, A. S., Satizabal, C. L., Aparicio, H. J., Thomas, R. J., Gottlieb, D. J., Auerbach, S. H., Seshadri, S. Tags: All Sleep Disorders, All Cognitive Disorders/Dementia, Alzheimer's disease, Cognitive aging, Cohort studies ARTICLE Source Type: research

Erenumab (AMG 334) in episodic migraine: Interim analysis of an ongoing open-label study
Conclusions: One-year efficacy, supported by functional improvements and favorable safety and tolerability profiles, supports further investigation of erenumab as a preventive treatment in patients with EM. Clinicaltrials.gov identifier: NCT01952574. Classification of evidence: This study provides Class IV evidence that for patients with episodic migraine, erenumab reduces long-term MMD and improves headache-related disability and migraine-specific quality of life. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Ashina, M., Dodick, D., Goadsby, P. J., Reuter, U., Silberstein, S., Zhang, F., Gage, J. R., Cheng, S., Mikol, D. D., Lenz, R. A. Tags: Migraine, All Clinical trials, Class IV ARTICLE Source Type: research

Stenting for symptomatic vertebral artery stenosis: The Vertebral Artery Ischaemia Stenting Trial
Conclusions: Stenting in extracranial stenosis appears safe with low complication rates. Large phase 3 trials are required to determine whether stenting reduces stroke risk. ISRCTN.com identifier: ISRCTN95212240. Classification of evidence: This study provides Class I evidence that for patients with symptomatic vertebral stenosis, angioplasty with stenting does not reduce the risk of stroke. However, the study lacked the precision to exclude a benefit from stenting. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Markus, H. S., Larsson, S. C., Kuker, W., Schulz, U. G., Ford, I., Rothwell, P. M., Clifton, A., For the VIST Investigators Tags: Stroke prevention, All Cerebrovascular disease/Stroke, Clinical trials Randomized controlled (CONSORT agreement) ARTICLE Source Type: research

Incidence and management of seizures after ischemic stroke: Systematic review and meta-analysis
Conclusions: The burden of stroke-related seizures and epilepsy due to ischemic stroke is substantial. Further studies are required to determine risk factors for epilepsy following ischemic stroke and optimal secondary prevention. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Wang, J. Z., Vyas, M. V., Saposnik, G., Burneo, J. G. Tags: All Cerebrovascular disease/Stroke, Incidence studies, All Epilepsy/Seizures ARTICLE Source Type: research

Genetic epilepsy with febrile seizures plus: Refining the spectrum
Conclusion: As 37/409 (9%) affected individuals have focal epilepsies, we suggest that GEFS+ be renamed genetic epilepsy with febrile seizures plus rather than generalized epilepsy with febrile seizures plus. The phenotypic overlap between GEFS+ and the classic generalized epilepsies is considerably greater than first thought. The clinical and molecular data suggest that the 2 major groups of generalized epilepsies share genetic determinants. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Zhang, Y.-H., Burgess, R., Malone, J. P., Glubb, G. C., Helbig, K. L., Vadlamudi, L., Kivity, S., Afawi, Z., Bleasel, A., Grattan-Smith, P., Grinton, B. E., Bellows, S. T., Vears, D. F., Damiano, J. A., Goldberg-Stern, H., Korczyn, A. D., Dibbens, L. M., Tags: All Genetics ARTICLE Source Type: research

Jean Holowach Thurston, MD (1917-2017)
Jean Holowach Thurston, MD, Emeritus Professor of Neurology and Pediatrics at Washington University in St. Louis, died on April 29, 2017, two months short of her 100th birthday. Her career encompassed a spectrum of disciplines that is unfathomable in our current era of subspecialization. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Connolly, A., Gurnett, C. A., Rothman, S., Zempel, J. Tags: IN MEMORIAM Source Type: research

Medication overuse headache: An ongoing debate
Medication overuse headache (MOH) has incited passionate debate among headache clinicians and researchers in recent years. The existence of MOH as a diagnostic category1 and as a potentially modifiable risk factor for headache chronification is well-recognized by most headache specialists.2 However, the greatest controversies seem to exist over whether medication overuse should be regarded as cause or consequence; and over the treatment strategies, including advice about withdrawal of frequently used symptomatic (acute pain relieving) medication and whether prophylactic treatment should be initiated during withdrawal or af...
Source: Neurology - September 18, 2017 Category: Neurology Authors: Louter, M. A., Robbins, M. S., Terwindt, G. M. Tags: EDITORIALS Source Type: research

Vertebral artery stenosis: The hurdles of stenting are too high
Up to a quarter of patients with vertebrobasilar ischemic stroke or TIA have a symptomatic stenosis of the vertebrobasilar arteries.1,2 Patients with vertebral artery (VA) stenosis>50% have a high risk of recurrent stroke, comparable to patients with symptomatic carotid artery stenosis, with the highest risk during the first weeks after the initial TIA or ischemic stroke.3 Endovascular treatment of symptomatic VA stenosis has appeal as a treatment option that is widely performed, despite the scarcity of evidence for its safety or benefit. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Compter, A., Chaturvedi, S. Tags: Stroke prevention, All Cerebrovascular disease/Stroke EDITORIALS Source Type: research

The "plus" side of epilepsy phenotyping
The first clinical description of generalized epilepsy with febrile seizures plus (GEFS+) by Scheffer and Berkovic1 in their landmark Brain article of 1997 represented a major step forward in the understanding of the genetic basis of the epilepsies. Coming 2 years after their identification, with collaborators, of the genetic basis for a relatively homogenous syndrome, autosomal dominant nocturnal frontal lobe epilepsy, the detailed phenotypic description of a single large Australian family with heterogeneous febrile seizure plus epilepsy phenotypes and a clear dominant pattern of inheritance put a novel perspective on gen...
Source: Neurology - September 18, 2017 Category: Neurology Authors: Zuberi, S. M., Striano, P. Tags: All Clinical Neurology, All Epilepsy/Seizures, Generalized seizures, Partial seizures EDITORIALS Source Type: research

Spotlight on the September 19 issue
(Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Teaching NeuroImages: RCVS causing simultaneous convexity subarachnoid hemorrhage and hemimedullary infarction
A 34-year-old previously healthy man presented with thunderclap headache, dysphagia, dysarthria, and oscillopsia. Neurologic examination revealed right-beating nystagmus, dysphagia, hypophonia, right hemianesthesia, and left hemiparesis. Brain imaging showed convexity subarachnoid hemorrhage (figure 1A) and left hemimedullary infarct (figure 1B). Catheter angiography showed tapering of left vertebral artery and diffuse segmental vasoconstriction (figure 2). Routine serum and CSF results were unremarkable. Thorough infectious, immunologic, and coagulopathy workup was negative. Reversible cerebral vasoconstriction syndrome (...
Source: Neurology - September 18, 2017 Category: Neurology Authors: Selvan, P., Levine, S. R. Tags: Stroke in young adults, Other cerebrovascular disease/ Stroke, All Cerebrovascular disease/Stroke, Infarction, Subarachnoid hemorrhage RESIDENT AND FELLOW SECTION Source Type: research

Opinion and Special Articles: Creation of a diversity and inclusion certificate program for neurology residents
Neurologists must provide excellent care to an increasingly diverse patient population. According to census projections, the United States will become a majority–minority nation by 2060, with no single ethnic or racial group making up a majority of the population.1 Diversity of gender, sex, sexual orientation, race, ethnicity, socioeconomic status, age, ability, and religion must be acknowledged and understood in order to provide equitable patient care. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Bank, A. M., Saadi, A., McKee, K. E., Mejia, N. I., Lyons, J. L. Tags: All Education, Methods of education RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A 50-year-old woman with SLE and a tumefactive lesion
A 50-year-old woman with a history of systemic lupus erythematosus (SLE) treated with methotrexate and hydroxychloroquine presented to the emergency department for evaluation of numbness and weakness of the left hand that began 12 hours earlier. (Source: Neurology)
Source: Neurology - September 18, 2017 Category: Neurology Authors: Choi, J.-H., Wallach, A. I., Rosales, D., Margiewicz, S. E., Belmont, H. M., Lucchinetti, C. F., Minen, M. T. Tags: MRI, Lupus, All Demyelinating disease (CNS), Multiple sclerosis, Devic's syndrome RESIDENT AND FELLOW SECTION Source Type: research

Clinical Reasoning: A 15-month-old boy with progressive lethargy and spasticity
A previously healthy and developmentally normal 15-month-old boy presented to the emergency department with 5 days of worsening altered mental status in the setting of an upper respiratory infection. He initially developed cough, rhinorrhea, and irritability without fever, vomiting, or diarrhea. His mental status at home had slowly declined, with increased sleepiness and progressively decreased activity. At the time of presentation, he had stopped playing, walking, sitting, or drinking. On his initial examination, he was breathing comfortably on room air and was afebrile with normal vital signs. He did not respond to stimu...
Source: Neurology - September 18, 2017 Category: Neurology Authors: Zhang, R., Ziobro, J., Harmon, J., Rodriguez Agramonte, F., Miller, M., Tochen, L., Jantausch, B., Hahn, A. Tags: All Infections, All Clinical Neurology, Parasitic infections, Retina, All Pediatric RESIDENT AND FELLOW SECTION Source Type: research

Motor neuron disease in patients with HIV infection
(Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research