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Editorialist response: A model for predicting the growth of unruptured intracranial aneurysms: Beyond fortune telling
I thank Dr. Munakomi for the comments on the prediction of unruptured intracranial aneurysms. I agree that computational fluid dynamics (CFD) may provide valuable insight into the rupture risk of unruptured aneurysms, in addition to currently known patient- and aneurysm-specific predictive factors. However, CFD models require rigorous and prospective validation before they can be applied to clinical decision-making for patients with unruptured aneurysm. Until such time, we must rely on the wisdom of experienced cerebrovascular physicians and published data from high-volume aneurysm treatment centers to extrapolate the risk...
Source: Neurology - September 11, 2017 Category: Neurology Authors: Ding, D. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: A model for predicting the growth of unruptured intracranial aneurysms: Beyond fortune telling
This article could change the treatment of unruptured aneurysms. In addition to the variables studied in this article, there is recent work evaluating the role of computational flow dynamics when assessing aneurysm-specific rupture risk.2 Future studies that include this entity might improve the ability to assess rupture risk. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Munakomi, S. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: Monitoring long-term efficacy of fampridine in gait-impaired patients with multiple sclerosis
Encouraged by Dr. Davis' insightful comment on our article,1 we retrospectively quantified the long-term responsiveness to fampridine of the patients with multiple sclerosis in our sample cotreated with natalizumab over a period of 2 years. Of 17 patients (32%) treated with natalizumab at the screening visit, only 6 were still receiving treatment with natalizumab 2 years later; natalizumab was stopped in 5 patients and a further 6 discontinued the study. Monitoring the efficacy of fampridine over 2 years in the 6 patients concomitantly treated with natalizumab did not reveal an increased benefit with respect to walking fun...
Source: Neurology - September 11, 2017 Category: Neurology Authors: Filli, L., Zörner, B., Killeen, T., Linnebank, M. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Monitoring long-term efficacy of fampridine in gait-impaired patients with multiple sclerosis
I read the article by Filli et al.1 with great interest. The authors reported that the responsiveness (efficacy) of prolonged-release fampridine for improving walking in multiple sclerosis (MS) increases over time.1 While the underlying mechanism is not known, its understanding might provide important insight for enhancing the efficacy of fampridine. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Davis, F. A. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In "Monitoring long-term efficacy of fampridine in gait-impaired patients with multiple sclerosis," authors Filli et al. showed that prolonged-release fampridine improved walking speed, endurance, and self-perceived ambulatory function in patients with multiple sclerosis (MS). Furthermore, patients showed additional drug responsiveness with long-term treatment. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Karam, C., Griggs, R. C. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Proptosis: A forgotten observation by Miller Fisher on his syndrome
A 61-year-old man presented with rapidly progressive vision change and facial droop. There was proptosis (figure) and profound facial diplegia in addition to the more classic triad of ophthalmoplegia, ataxia, and areflexia (video at Neurology.org). The clinical diagnosis of Miller Fisher syndrome was confirmed by electrophysiologic studies. He made a complete recovery after IV immunoglobulin infusion. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Braksick, S. A., Rabinstein, A. A., Wijdicks, E. F. M. Tags: All Clinical Neurology, Clinical neurology examination, All Neuromuscular Disease, Guillain-Barre syndrome VIDEO NEUROIMAGES Source Type: research

Ancillary staff A difficult kind of listening
Teflon coated nickel wire fed through a tungsten guide spot welded from one gold plate pin to the next by the numbers. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Krieger, D. Tags: Surgical therapy-tumor, All clinical neurophysiology, All Ethics in Neurology/Legal issues REFLECTIONS: NEUROLOGY AND THE HUMANITIES Source Type: research

Rare case of ribose 5 phosphate isomerase deficiency with slowly progressive leukoencephalopathy
We report the second case of RPID with a novel homozygous missense mutation. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Naik, N., Shah, A., Wamelink, M. M. C., van der Knaap, M. S., Hingwala, D. Tags: MRS CLINICAL/SCIENTIFIC NOTES Source Type: research

Staying in service with posttraumatic headache: A retrospective cohort study of patient outcome
Objective: To predict the probability of a military outcome (medical discharge/retirement) in patients with mild traumatic brain injury from a clinical analysis of predetermined patient and headache characteristics. Methods: This retrospective cohort study sampled all new patients referred for headache evaluation at the Brain Injury Clinic of the Womack Army Medical Center, Ft. Bragg, NC (August 2008–January 2010). Headache characteristics were extracted and analyzed. Multivariable binary logistic regressions were conducted to predict probability of medical discharge/retirement. Results: Ninety-five soldiers (age 3...
Source: Neurology - September 11, 2017 Category: Neurology Authors: Finkel, A. G., Klaric, J. S., Yerry, J. A., Choi, Y. S. Tags: ARTICLE Source Type: research

Downregulation of early visual cortex excitability mediates oscillopsia suppression
Conclusions: Our findings provide neurophysiologic evidence that cortically mediated adaptive mechanisms in V1/V2 play a critical role in suppressing oscillopsia in patients with BVF. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Ahmad, H., Roberts, R. E., Patel, M., Lobo, R., Seemungal, B., Arshad, Q., Bronstein, A. Tags: Nystagmus, Oscillopsia, All Neurotology, Vertigo, TMS ARTICLE Source Type: research

18F-AV-1451 binds to motor-related subcortical gray and white matter in corticobasal syndrome
Conclusions: 18F-AV-1451 asymmetrically binds to motor-related subcortical gray and white matter structures in patients with CBS. This pattern corresponds to tau pathology distribution in postmortem studies, and motor deficit in patients with CBS may be associated with tau accumulation predominantly in the subcortical white matter underlying the motor cortex, leading to disruptions in motor-related networks. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Cho, H., Baek, M. S., Choi, J. Y., Lee, S. H., Kim, J. S., Ryu, Y. H., Lee, M. S., Lyoo, C. H. Tags: PET ARTICLE Source Type: research

Utilization of rehabilitation therapy services in Parkinson disease in the United States
Conclusions: This claims-based analysis suggests that rehabilitation therapy utilization among older patients with PD in the United States is lower than reported for countries with comparable health care infrastructure. Neurologist care is associated with rehabilitation therapy use; provider supply is not. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Fullard, M. E., Thibault, D. P., Hill, A., Fox, J., Bhatti, D. E., Burack, M. A., Dahodwala, N., Haberfeld, E., Kern, D. S., Klepitskava, O. S., Urrea-Mendoza, E., Myers, P., Nutt, J., Rafferty, M. R., Schwalb, J. M., Shulman, L. M., Willis, A. W., On beh Tags: All Health Services Research, Parkinson's disease/Parkinsonism, All epidemiology ARTICLE Source Type: research

National randomized controlled trial of virtual house calls for Parkinson disease
Objective: To determine whether providing remote neurologic care into the homes of people with Parkinson disease (PD) is feasible, beneficial, and valuable. Methods: In a 1-year randomized controlled trial, we compared usual care to usual care supplemented by 4 virtual visits via video conferencing from a remote specialist into patients' homes. Primary outcome measures were feasibility, as measured by the proportion who completed at least one virtual visit and the proportion of virtual visits completed on time; and efficacy, as measured by the change in the Parkinson's Disease Questionnaire–39, a quality of life sca...
Source: Neurology - September 11, 2017 Category: Neurology Authors: Beck, C. A., Beran, D. B., Biglan, K. M., Boyd, C. M., Dorsey, E. R., Schmidt, P. N., Simone, R., Willis, A. W., Galifianakis, N. B., Katz, M., Tanner, C. M., Dodenhoff, K., Aldred, J., Carter, J., Fraser, A., Jimenez-Shahed, J., Hunter, C., Spindler, M., Tags: Parkinson's disease/Parkinsonism, Clinical trials Randomized controlled (CONSORT agreement) ARTICLE Source Type: research

Direct oral anticoagulant- vs vitamin K antagonist-related nontraumatic intracerebral hemorrhage
Conclusions: DOAC-related ICH is associated with smaller baseline hematoma volume and lesser neurologic deficit at hospital admission compared to VKA-related ICH. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Tsivgoulis, G., Lioutas, V.-A., Varelas, P., Katsanos, A. H., Goyal, N., Mikulik, R., Barlinn, K., Krogias, C., Sharma, V. K., Vadikolias, K., Dardiotis, E., Karapanayiotides, T., Pappa, A., Zompola, C., Triantafyllou, S., Kargiotis, O., Ioakeimidis, M., Tags: Intracerebral hemorrhage ARTICLE Source Type: research

Subcutaneous immunoglobulin in myasthenia gravis exacerbation: A prospective, open-label trial
Objective: To investigate the efficacy, tolerability, and safety of subcutaneous immunoglobulin (SCIg) in patients with mild to moderate myasthenia gravis (MG) exacerbation. Methods: We performed a prospective, open-label, phase 3 trial in patients with MG aged 18 years or older and mild to moderate worsening (transition from Myasthenia Gravis Foundation of America class I to II/III or class II to III), treated with SCIg (2 g/kg), self-administered over 4 weeks. The primary endpoint was change in quantitative MG (QMG) score from baseline to study end at 6 weeks. Secondary endpoints included change in manual muscle testing...
Source: Neurology - September 11, 2017 Category: Neurology Authors: Beecher, G., Anderson, D., Siddiqi, Z. A. Tags: Outcome research, Myasthenia, All Clinical trials, Patient safety, Class IV ARTICLE Source Type: research

Nivolumab-related myasthenia gravis with myositis and myocarditis in Japan
Conclusions: The prompt and correct recognition of MG following treatment with immune checkpoint inhibitors in patients with cancer is important. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Suzuki, S., Ishikawa, N., Konoeda, F., Seki, N., Fukushima, S., Takahashi, K., Uhara, H., Hasegawa, Y., Inomata, S., Otani, Y., Yokota, K., Hirose, T., Tanaka, R., Suzuki, N., Matsui, M. Tags: Autoimmune diseases, Myasthenia, Muscle disease, Incidence studies ARTICLE Source Type: research

Alemtuzumab CARE-MS II 5-year follow-up: Efficacy and safety findings
Conclusions: Alemtuzumab provides durable efficacy through 5 years in patients with an inadequate response to prior therapy in the absence of continuous treatment. Classification of evidence: This study provides Class III evidence that alemtuzumab provides efficacy and slowing of brain atrophy through 5 years. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Coles, A. J., Cohen, J. A., Fox, E. J., Giovannoni, G., Hartung, H.-P., Havrdova, E., Schippling, S., Selmaj, K. W., Traboulsee, A., Compston, D. A. S., Margolin, D. H., Thangavelu, K., Chirieac, M. C., Jody, D., Xenopoulos, P., Hogan, R. J., Panzara, M. Tags: Autoimmune diseases, Clinical trials Randomized controlled (CONSORT agreement), Class III, Multiple sclerosis ARTICLE Source Type: research

Alemtuzumab CARE-MS I 5-year follow-up: Durable efficacy in the absence of continuous MS therapy
Conclusions: Based on these data, alemtuzumab provides durable efficacy through 5 years in the absence of continuous treatment, with most patients not receiving additional courses. ClinicalTrials.gov identifier: NCT00530348; NCT00930553. Classification of evidence: This study provides Class III evidence that alemtuzumab durably improves efficacy outcomes and slows BVL in patients with RRMS. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Havrdova, E., Arnold, D. L., Cohen, J. A., Hartung, H.-P., Fox, E. J., Giovannoni, G., Schippling, S., Selmaj, K. W., Traboulsee, A., Compston, D. A. S., Margolin, D. H., Thangavelu, K., Rodriguez, C. E., Jody, D., Hogan, R. J., Xenopoulos, P., Panzara, M Tags: Autoimmune diseases, Clinical trials Randomized controlled (CONSORT agreement), Class III, Multiple sclerosis ARTICLE Source Type: research

As the world turns, why do some people adapt to vestibular failure and others do not?
Chronic vertigo and oscillopsia are 2 of the most vexing problems that neurologists encounter. Even when the etiology is known, there is often a limit to the therapy that can be offered to the patient beyond vestibular rehabilitation. Oscillopsia diminishes over time in many individuals, but the mechanism for this adaptive change is unclear. In this issue of Neurology®, Ahmad et al.1 examine the central mechanisms that may be responsible for adaptation after bilateral vestibular failure. Most of the cases in this cohort had vestibular failure from either idiopathic or autoimmune causes. Ahmad et al. found that the pati...
Source: Neurology - September 11, 2017 Category: Neurology Authors: Galetta, S. L. Tags: EDITORIALS Source Type: research

The virtual house call: A 21st-century innovation in the care of patients with Parkinson disease
As the debate on health care reform rages in the United States, one thing is exceedingly clear: chronic disease accounts for a growing majority of American health care costs.1 Many chronic diseases, particularly Parkinson disease (PD), can benefit from specialty care. Patients with PD show more improvements in motor symptoms and less frequently receive inappropriate medications when treated by a specialist.2,3 However, reduced mobility due to PD, combined with the physical distance to the specialist's office, poses substantial travel burdens and may limit access to care. Telemedicine, utilizing video and audio conferencing...
Source: Neurology - September 11, 2017 Category: Neurology Authors: Shprecher, D. R., Majersik, J. J. Tags: EDITORIALS Source Type: research

Immune checkpoint inhibitor therapy: A double-edged sword?
Cancer and autoimmunity can be viewed as differing consequences of deranged immune responses. The overexpression of certain molecules could result in development of cancer (failure of the immune system to control tumor cell growth), whereas oversuppression of the same molecules could lead to autoimmunity (failure of the immune system to regulate autoreactive responses). Hence, finely balanced immune surveillance against cancer and autoimmunity is critical. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Kim, H. J., Evoli, A. Tags: EDITORIALS Source Type: research

Can immune reprogramming with alemtuzumab induce permanent remission in multiple sclerosis?
Disease-modifying therapies (DMTs) for multiple sclerosis (MS) have developed tremendously over the last 2 decades. Currently, more than 16 different products targeting various immunologic components have been approved for MS treatment in several countries. (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Wiendl, H., Bourdette, D., Ciccarelli, O. Tags: All Clinical trials, Multiple sclerosis EDITORIALS Source Type: research

Spotlight on the September 12 issue
(Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research

Teaching NeuroImages: Early-onset dementia and demyelinating neuropathy disclosing cerebrotendinous xanthomatosis
A 40-year-old woman presented with a 5-year history of numbness in hands and feet, apathy, compulsive behavior, and aggression towards others. Medical history disclosed juvenile-onset cataracts. Examination disclosed tendinous xanthoma. Electroneuromyography disclosed sensorimotor demyelinating polyneuropathy. Neuroimaging studies showed global cortical atrophy. Tc-99m brain SPECT scan showed marked hypoperfusion involving the frontal and temporal lobes (figure). Plasma cholestanol levels and bile alcohol glycoconjugates were elevated, enabling a diagnosis of cerebrotendinous xanthomatosis (CTX). (Source: Neurology)
Source: Neurology - September 11, 2017 Category: Neurology Authors: Souza, P. V. S., Bortholin, T., Naylor, F. G. M., Pinto, W. B. V. R., Oliveira, A. S. B. Tags: MRI, SPECT, Metabolic disease (inherited), Peripheral neuropathy, Frontotemporal dementia RESIDENT AND FELLOW SECTION Source Type: research

Emerging Subspecialties in Neurology: Autoimmune neurology
Autoimmune neurology is one of the most rapidly evolving fields in modern neurology. Autoantibodies that recognize nervous system self-antigens, including ion channels, receptors for neurotransmitters, and neuronal intrinsic and extrinsic proteins involved in synaptic transmission, are all recognized as targets of pathogenic autoantibodies. The accelerating rate of new antigen discovery in recent decades is impressive (figure). The diversity of neurologic presentations, the unique pathophysiology, and the complexity of treating these disorders justifies dedicated fellowship training to acquire the expertise needed to diagn...
Source: Neurology - September 11, 2017 Category: Neurology Authors: Lopez-Chiriboga, A. S., Clardy, S. L. Tags: All Immunology RESIDENT AND FELLOW SECTION Source Type: research

Author response: Hans Jacob and brain research on Hamburg "euthanasia" victims: "Awaiting further brains!"
I appreciate Dr. Burlon's response to my article,1 highlighting not only the tragic actions of 2 doctors, but also a strategy to employ when deciding how to best care for patients. By questioning whether they would have implemented "euthanasia" on neuropsychiatric patients if they were close relatives and not institutionalized "useless eaters," perhaps Drs. Knigge and Bayer would not have murdered. In addition, part of Knigge's motivation, to scientifically evaluate the murdered patients' brains, was fulfilled by Hans Jacob, who performed the neuropathologic examinations. Jacob's work lent legitimacy to...
Source: Neurology - September 4, 2017 Category: Neurology Authors: Zeidman, L. A. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: Hans Jacob and brain research on Hamburg "euthanasia" victims: "Awaiting further brains!"
Can we learn from history? To answer this, it is helpful to take a deeper look at the actions and motivations of Dr. Bayer and Dr. Knigge, 2 doctors from Hamburg who killed children for brains. First, they needed a bureaucratic organization (Reichsausschussverfahren) on which to place the deadly and inhumane decision. Second, they dehumanized the children, labeling them as worthless and a load to parents and society, and identified their cruel decision as a religious type of salvation or grace of charity. Third, and possibly the strongest motivation, their careers greatly benefited. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Burlon, M. M. H. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Author response: A dedicated scholarly research program in an adult and pediatric neurology residency program
We thank Dr. Meador for the comment on our study examining the effect of the scholarly research program implemented in our neurology residency program.1 We agree that the shifting demands on neurologists relate to the crucial issue of burnout, though academic practice neurologists had lower burnout rates and higher rates of career satisfaction than neurologists in clinical practice.2 However, Dr. Meador previously noted that, in the current climate, younger academic faculty members are uniquely vulnerable to fail in their pursuit of successful research careers based on a variety of factors, including lack of protected time...
Source: Neurology - September 4, 2017 Category: Neurology Authors: Robbins, M. S., Mehler, M. F. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Letter re: A dedicated scholarly research program in an adult and pediatric neurology residency program
I applaud Robbins et al.1 for successfully improving scholarly research in their residency program. In general, education on clinical research is inadequate in medical schools and postgraduate clinical programs.2 Flexner3 stressed that original research, and the critical method of research, should play an important role in medical schools. However, clinical research at academic medical centers is under stress.2 The amount of time spent by academic physicians on clinical research has declined over the last several decades due to increasing demands to provide clinical care.2 Another recent publication provided data to this r...
Source: Neurology - September 4, 2017 Category: Neurology Authors: Meador, K. J. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Editors' Note
: In the April 4, 2017, issue of Neurology®, Drs. Robbins et al. described the implementation and assessment of a formal scholarly activity program within their residency curriculum. They documented a significant increase in scholarly output associated with the program, including an increase in publications, clinical and laboratory research, and presentations at scientific meetings, as well as an increase in the number of faculty willing to be mentors. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Alcauskas, M., Galetta, S. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research

Intra-arterial milrinone may differentiate fulminant RCVS from vasculitis
A 39-year-old woman taking a monoamine oxidase inhibitor presented with a 1-week history of severe non-thunderclap headache and visual field deficits. Brain CT showed infarcts in both parietal lobes, with narrowing of intracranial vessels on CT angiogram (figure 1), suggesting either vasculitis or reversible cerebral vasoconstriction syndrome (RCVS). Despite treatment with both methylprednisolone and nimodipine, she experienced progressive aphasia and right leg weakness. She underwent an urgent cerebral angiogram (figure 2), during which intra-arterial milrinone reversed both the vasoconstriction and its symptoms, thereby ...
Source: Neurology - September 4, 2017 Category: Neurology Authors: Laneuville, M., Ding, J., Shamy, M., Lum, C., Dowlatshahi, D. Tags: All Headache, Other cerebrovascular disease/ Stroke NEUROIMAGES Source Type: research

{beta}-N-methylamino-L-alanine analysis in the brains of patients with Kii ALS/PDC
The Kii Peninsula of Japan and the island of Guam are known as high-incidence areas of amyotrophic lateral sclerosis and parkinsonism–dementia complex (ALS/PDC). The disorder is clinically characterized by a variable presentation of parkinsonism, dementia, and motor neuron symptoms and pathologically by tau protein deposits in the CNS.1 Both genetic and environmental factors are implicated in ALS/PDC pathogenesis. Exposure to β-N-methylamino-l-alanine (BMAA), a neurotoxin produced by cyanobacteria, has been proposed as a risk factor for ALS/PDC in Guam.2 It is not known whether Kii ALS/PDC shares the same etiolo...
Source: Neurology - September 4, 2017 Category: Neurology Authors: Kokubo, Y., Banack, S. A., Morimoto, S., Murayama, S., Togashi, T., Metcalf, J. S., Cox, P. A., Kuzuhara, S. Tags: Parkinson's disease/Parkinsonism, Amyotrophic lateral sclerosis, All Cognitive Disorders/Dementia, Other toxicology CLINICAL/SCIENTIFIC NOTES Source Type: research

A critique of phrenology in Moby-Dick
Phrenology has a fascinating, although controversial, place in the history of localizationism of brain and mental functions. The 2 main proponents of phrenology were 2 German-speaking doctors, Joseph Gall (1758–1828) and Johann Spurzheim (1776–1832). According to their theory, a careful examination of skull morphology could disclose personality characters. Phrenology was initially restricted to medical circles and then diffused outside scientific societies, reaching nonscientific audiences in Europe and North America. Phrenology deeply penetrated popular culture in the 19th century and its tenets can be observe...
Source: Neurology - September 4, 2017 Category: Neurology Authors: de Souza, L. C., Teixeira, A. L., de Oliveira, G. N. M., Caramelli, P., Cardoso, F. Tags: History of Neurology HISTORICAL NEUROLOGY Source Type: research

Efficacy of single or combined midodrine and pyridostigmine in orthostatic hypotension
Objective: To evaluate the long-term (for up to 3 months) efficacy and safety of single or combined therapy with midodrine and pyridostigmine for neurogenic orthostatic hypotension (OH). Methods: This was a randomized, open-label clinical trial. In total, 87 patients with symptomatic neurogenic OH were enrolled and randomized to receive 1 of 3 treatments: midodrine only, pyridostigmine only, or midodrine + pyridostigmine. The patients were followed up at 1 and 3 months after treatment. The primary outcome measures were improvement in orthostatic blood pressure (BP) drop at 3 months. Secondary endpoints were improvement of...
Source: Neurology - September 4, 2017 Category: Neurology Authors: Byun, J.-I., Moon, J., Kim, D.-Y., Shin, H., Sunwoo, J.-S., Lim, J.-A., Kim, T.-J., Lee, W.-J., Lee, H. S., Jun, J.-S., Park, K.-I., Lee, S.-T., Jung, K.-H., Jung, K.-Y., Lee, S. K., Chu, K. Tags: Autonomic diseases, All Clinical trials, Class IV ARTICLE Source Type: research

Rituximab as treatment for anti-MuSK myasthenia gravis: Multicenter blinded prospective review
This study provides Class IV evidence that for patients with anti-MuSK MG, rituximab increased the probability of a favorable outcome. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Hehir, M. K., Hobson-Webb, L. D., Benatar, M., Barnett, C., Silvestri, N. J., Howard, J. F., Howard, D., Visser, A., Crum, B. A., Nowak, R., Beekman, R., Kumar, A., Ruzhansky, K., Chen, I.-H. A., Pulley, M. T., LaBoy, S. M., Fellman, M. A., Greene, S. M., Tags: Myasthenia, Clinical trials Methodology/study design, Clinical trials Observational study (Cohort, Case control) ARTICLE Source Type: research

Clinicopathologic features of myositis patients with CD8-MHC-1 complex pathology
Conclusions: CD8-MHC-1 complex is present in patients with PM, IBM, or unclassifiable group. The data may serve as an argument for a trial of immunosuppressive treatment in p62-immunonegative patients with unclassifiable myositis. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Ikenaga, C., Kubota, A., Kadoya, M., Taira, K., Uchio, N., Hida, A., Maeda, M. H., Nagashima, Y., Ishiura, H., Kaida, K., Goto, J., Tsuji, S., Shimizu, J. Tags: Muscle disease ARTICLE Source Type: research

Anti-inflammatory disease-modifying treatment and short-term disability progression in SPMS
Conclusions: Our pooled analysis of the currently available disease-modifying agents used after conversion to SPMS suggests that, on average, these therapies have no substantial effect on relapse-unrelated disability outcomes measured by the EDSS up to 4 years. Classification of evidence: This study provides Class IV evidence that for patients with SPMS, disease-modifying treatment has no beneficial effect on short-term disability progression. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Lorscheider, J., Jokubaitis, V. G., Spelman, T., Izquierdo, G., Lugaresi, A., Havrdova, E., Horakova, D., Trojano, M., Duquette, P., Girard, M., Prat, A., Grand'Maison, F., Grammond, P., Pucci, E., Boz, C., Sola, P., Ferraro, D., Spitaleri, D., Lechner-Sc Tags: Clinical trials Observational study (Cohort, Case control), Class IV, Multiple sclerosis ARTICLE Source Type: research

Clinical and genetic characteristics of sporadic adult-onset degenerative ataxia
Conclusions: Our study provides quantitative data on the clinical phenotype and progression of sporadic ataxia with adult onset. Screening for causative mutations with a gene panel approach yielded a genetic diagnosis in 6% of the cohort. ClinicalTrials.gov registration: NCT02701036. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Giordano, I., Harmuth, F., Jacobi, H., Paap, B., Vielhaber, S., Machts, J., Schöls, L., Synofzik, M., Sturm, M., Tallaksen, C., Wedding, I. M., Boesch, S., Eigentler, A., van de Warrenburg, B., van Gaalen, J., Kamm, C., Dudesek, A., Kang, J.-S., T Tags: Gait disorders/ataxia, Multiple system atrophy, Cerebellum, Natural history studies (prognosis) ARTICLE Source Type: research

Not all SCN1A epileptic encephalopathies are Dravet syndrome: Early profound Thr226Met phenotype
Conclusions: Here, we present a phenotype-genotype correlation for SCN1A. We describe a distinct SCN1A phenotype, early infantile SCN1A encephalopathy, which is readily distinguishable from the well-recognized entities of Dravet syndrome and genetic epilepsy with febrile seizures plus. This disorder has an earlier age at onset, profound developmental impairment, and a distinctive hyperkinetic movement disorder, setting it apart from Dravet syndrome. Remarkably, 8 of 9 children had the recurrent missense mutation p.Thr226Met. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Sadleir, L. G., Mountier, E. I., Gill, D., Davis, S., Joshi, C., DeVile, C., Kurian, M. A., For the DDD Study, Mandelstam, S., Wirrell, E., Nickels, K. C., Murali, H. R., Carvill, G., Myers, C. T., Mefford, H. C., Scheffer, I. E. Tags: All Clinical Neurology, All Movement Disorders, All Pediatric, Epilepsy semiology ARTICLE Source Type: research

APOE genotype and early {beta}-amyloid accumulation in older adults without dementia
Conclusions: APOE 4 carriage and older age were predictors of longitudinal Aβ accumulation within the Aβ– group but not the Aβ+ group. APOE 2 carriage was protective against longitudinal Aβ accumulation within the Aβ– group. APOE genotype in conjunction with chronologic age may aid in participant selection for primary prevention trials aimed at halting Aβ accumulation before abnormal levels are reached. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Lim, Y. Y., Mormino, E. C., For the Alzheimer's Disease Neuroimaging Initiative Tags: PET, Alzheimer's disease, MCI (mild cognitive impairment), All Genetics ARTICLE Source Type: research

Longitudinal diffusion changes following postoperative delirium in older people without dementia
Conclusions: Our study raises the possibility that delirium has an effect on the development of brain microstructural abnormalities, which may reflect brain changes underlying cognitive trajectories. Future studies are warranted to clarify whether delirium is the driving factor of the observed changes or rather a correlate of a vulnerable brain that is at high risk for neurodegenerative processes. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Cavallari, M., Dai, W., Guttmann, C. R. G., Meier, D. S., Ngo, L. H., Hshieh, T. T., Fong, T. G., Schmitt, E., Press, D. Z., Travison, T. G., Marcantonio, E. R., Jones, R. N., Inouye, S. K., Alsop, D. C., On behalf of the SAGES Study Group Tags: MRI, DWI, Delirium, Cognitive aging ARTICLE Source Type: research

Assessment of the interaction of age and sex on 90-day outcome after intracerebral hemorrhage
Conclusion: Unlike in ischemic stroke, there was no evidence that patient sex modified the effect of age on 90-day outcomes after ICH in a large multiracial/ethnic population. Future studies should evaluate biological reasons for these differences between stroke subtypes. Clinicaltrials.gov identifier: NCT01202864. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: James, M. L., Langefeld, C. D., Sekar, P., Moomaw, C. J., Elkind, M. S. V., Worrall, B. B., Sheth, K. N., Martini, S. R., Osborne, J., Woo, D., On behalf of the ERICH Investigators Tags: Outcome research, Clinical trials Observational study (Cohort, Case control), Intracerebral hemorrhage ARTICLE Source Type: research

White matter hyperintensity reduction and outcomes after minor stroke
Conclusions: Some WMH may regress after minor stroke, with potentially better clinical and brain tissue outcomes. The role of risk factor control requires verification. Interstitial fluid alterations may account for some WMH reversibility, offering potential intervention targets. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Wardlaw, J. M., Chappell, F. M., Valdes Hernandez, M. d. C., Makin, S. D. J., Staals, J., Shuler, K., Thrippleton, M. J., Armitage, P. A., Munoz-Maniega, S., Heye, A. K., Sakka, E., Dennis, M. S. Tags: MRI, Vascular dementia, Infarction ARTICLE Source Type: research

Validating and comparing stroke prognosis scales
Conclusions: Our comparative analyses confirm differences in the prognostic accuracy of stroke scales. However, even the best performing scale had prognostic accuracy that may not be sufficient as a basis for clinical decision-making. (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Quinn, T. J., Singh, S., Lees, K. R., Bath, P. M., Myint, P. K., On behalf of the VISTA Collaborators Tags: Outcome research, Prognosis, All Cerebrovascular disease/Stroke ARTICLE Source Type: research

Sex-specific stroke incidence over time in the Greater Cincinnati/Northern Kentucky Stroke Study
Objective: Recent data suggest stroke incidence is decreasing over time, but it is unknown whether incidence is decreasing in women and men to the same extent. Methods: Within our population of 1.3 million, all incident strokes among residents ≥20 years old were ascertained at all hospitals during July 1993–June 1994 and calendar years 1999, 2005, and 2010. A sampling scheme was used to ascertain out-of-hospital cases. Sex-specific incidence rates per 100,000 among black and white participants, age- and race-adjusted, were standardized to the 2000 US Census population. Trends over time by sex were compared; a Bon...
Source: Neurology - September 4, 2017 Category: Neurology Authors: Madsen, T. E., Khoury, J., Alwell, K., Moomaw, C. J., Rademacher, E., Flaherty, M. L., Woo, D., Mackey, J., De Los Rios La Rosa, F., Martini, S., Ferioli, S., Adeoye, O., Khatri, P., Broderick, J. P., Kissela, B. M., Kleindorfer, D. Tags: Stroke prevention, All Cerebrovascular disease/Stroke, Incidence studies, Risk factors in epidemiology ARTICLE Source Type: research

Rare disease levels of evidence: Time for a new bar?
A report of the Canadian Task Force on the Periodic Health Examination was the first to propose clinical practice recommendations based on evidence in the medical literature, and proposed a rating scheme.1 From that original publication, the categorization of evidence has evolved, with this journal requiring clinical therapeutic studies to provide the level of evidence provided by a report.2 The most rigorous Class I evidence derives from a well-performed randomized controlled clinical trial (RCT). Such trials limit the probability of bias and systematic errors by randomization and masking of treatment assignment. In contr...
Source: Neurology - September 4, 2017 Category: Neurology Authors: Bril, V., Kaminski, H. J. Tags: EDITORIALS Source Type: research

Dynamic measurements of {beta}-amyloid accumulation: The early effect of APOE
Despite many disappointments, the strongest current in Alzheimer disease (AD) research remains the pursuit of anti-amyloid therapies. There is increasing recognition that if these compounds are to be effective, early initiation of treatment, before there is irreversible neuronal and synaptic loss, is more likely to be successful. Due to the complexity of AD, to assess individual risk we might need algorithms including multiple variables like age, APOE plus other genetic variants, clinical and neuropsychological information, vascular, metabolic, and lifestyle risk factors, comorbidities, and disease biomarkers. The earliest...
Source: Neurology - September 4, 2017 Category: Neurology Authors: Sanchez-Juan, P., Seshadri, S. Tags: EDITORIALS Source Type: research

Waxing and waning of white matter hyperintensities
Small vessel disease (SVD) manifests in myriad ways, most prominently as white matter hyperintensities (WMH).1,2 By the age of 60 years, virtually every healthy individual has evidence of WMH,3 and those with ischemic stroke have even more extensive changes.4 Cerebral ischemic injury from occlusion or stenosis of deep penetrating arteries due to (uncontrolled) hypertension ranks highest among the potential causes of WMH.5 These WMH are among the most important vascular contributors to cognitive decline, dementia, and parkinsonism.6 (Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: de Leeuw, F.-E., Nichols, F. Tags: MRI, All Cerebrovascular disease/Stroke, Vascular dementia EDITORIALS Source Type: research

Trends in stroke incidence in the United States: Will women overtake men?
Stroke poses a heavy burden on health systems, with serious implications for societies and economies. At the global level, stroke is the second leading cause of death and the third most common cause of disability.1 In the United States, about 795,000 strokes occur every year: on average, someone has a stroke every 40 seconds and someone dies as a result of a stroke every 4 minutes.2 Stroke incidence and mortality rates have decreased in the last decades3; however, the aging of populations and improvements in stroke management have resulted in higher numbers of people experiencing a stroke, and more stroke survivors requiri...
Source: Neurology - September 4, 2017 Category: Neurology Authors: Koton, S., Rexrode, K. M. Tags: EDITORIALS Source Type: research

Spotlight on the September 5 issue
(Source: Neurology)
Source: Neurology - September 4, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research