Neurology

OBJECTIVE: To validate the current criteria of visual snow and to describe its common phenotype using a substantial clinical database. METHODS: We performed a web-based survey of patients with self-assessed visual snow (n = 1,104), with either the complete visual snow syndrome (n = 1,061) or visual snow without the syndrome (n = 43). We also describe a population of patients (n = 70) with possible hallucinogen persisting perception disorder who presented clinically with visual snow syndrome...

OBJECTIVE: To estimate changes in costs and utilization trends for disease-modifying therapies (DMTs) from 2011 to 2017 in the US Medicaid program. METHODS: Using quarterly Medicaid State Drug Utilization Data from 2011 to 2017, we summarize trends in spending, utilization, and costs per prescription for 15 multiple sclerosis (MS) DMTs including brand and generic versions of glatiramer acetate. We use interrupted time series regression to estimate the effect of market entry of generic glatiramer acetate on cost per prescription of other self-administered DMTs...

OBJECTIVE: To investigate the efficacy of N -acetylcysteine (NAC) for decreasing elevated oxidative stress and increasing physical endurance in individuals with ryanodine receptor 1-related myopathies ( RYR1 -RM). METHODS: In this 6-month natural history assessment (n = 37) followed by a randomized, double-blinded, placebo-controlled trial, 33 eligible participants were block-randomized (1:1) to receive NAC (n = 16) or placebo (n = 17), orally for 6 months (adult dose 2,700 mg/d; pediatric dose 30 mg/kg/d)...

No abstract text is available yet for this article.

OBJECTIVE: We studied the potential of quantitative MRI (qMRI) as a surrogate endpoint in Duchenne muscular dystrophy by assessing the additive predictive value of vastus lateralis (VL) fat fraction (FF) to age on loss of ambulation (LoA). METHODS: VL FFs were determined on longitudinal Dixon MRI scans from 2 natural history studies in Leiden University Medical Center (LUMC) and Cincinnati Children's Hospital Medical Center (CCHMC). CCHMC included ambulant patients, while LUMC included a mixed ambulant and nonambulant population...

OBJECTIVE: Studies of outcome after traumatic brain injury (TBI) are hampered by the lack of robust injury severity measures that can accommodate spatial-anatomical and mechanistic heterogeneity. In this study we introduce a Mahalanobis distance measure ( M ) as an intrinsic injury severity measure that combines in a single score the many ways a given injured brain's connectivity can vary from that of healthy controls. Our objective is to test the hypotheses that M is superior to univariate measures in (1) discriminating patients and controls and (2) correlating with cognitive assessment...

OBJECTIVE: To determine whether different phenotypes of cervical dystonia (CD) express different types and levels of somatosensory impairment. METHODS: We assessed somatosensory function in patients with CD with and without tremor (n = 12 each) and in healthy age-matched controls (n = 22) by measuring tactile temporal discrimination thresholds of the nondystonic forearm and proprioceptive acuity in both the dystonic (head/neck) and nondystonic body segments (forearm/hand) using a joint position-matching task...

OBJECTIVE: To gain a better understanding of the complex patterns of causes that contribute to death due to multiple sclerosis (MS) by assessing the relationship between MS and other causes of death listed on death certificates. METHODS: Multiple cause of death data for all adult deaths (aged ≥18 years) in British Columbia, Canada, between 1986 and 2013 were accessed. All causes, as listed on the death certificate, whether underlying or contributing, were considered "any mention" causes...

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OBJECTIVE: To provide the first clinical report that 2 calcitonin gene-related peptide (CGRP) therapies, a small molecule CGRP receptor antagonist and an anti-CGRP receptor antibody, can be used concomitantly to treat refractory migraine. METHODS: Case reports are presented of 2 patients participating in a long-term safety study of rimegepant 75 mg oral tablets for acute treatment (NCT03266588). After Food and Drug Administration approval of erenumab, both patients started subcutaneous erenumab monthly as allowed per protocol...

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OBJECTIVE: We evaluated the interaction of serum folate and vitamin B12 with methylenetetrahydrofolate reductase ( MTHFR ) C677T genotypes on the risk of first ischemic stroke and on the efficacy of folic acid treatment in prevention of first ischemic stroke. METHODS: A total of 20,702 hypertensive adults were randomized to a double-blind treatment of daily enalapril 10 mg and folic acid 0.8 mg or enalapril 10 mg alone. Participants were followed up every 3 months...

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OBJECTIVE: To evaluate whether structural and functional changes occur in the afferent visual system of patients with stiff-person syndrome (SPS) and whether these changes correlate with disease burden, given the high concentration of γ-aminobutyric acid receptors, which are generally thought to be involved in SPS pathogenesis, in the retina. METHODS: In this single-center, cross-sectional study, patients with SPS and healthy controls (HCs) underwent optical coherence tomography (OCT), with a subset undergoing high- and low-contrast visual acuity (VA) assessments...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

OBJECTIVE: To describe and compare phenotypic features of posttraumatic headaches (PTH) and headaches unrelated to concussion. METHODS: Participants are a random sample of recently deployed soldiers from the Warrior Strong cohort, consisting of soldiers with (n = 557) and without (n = 1,030) a history of a recent mild traumatic brain injury (mTBI; concussion). mTBI+ soldiers were subdivided as PTH+ (n = 230) and PTH- (n = 327). Headache classification was based on a detailed phenotypic questionnaire...

No abstract text is available yet for this article.

OBJECTIVE: To understand whether the clinical phenotype of nonfluent/agrammatic primary progressive aphasia (nfvPPA) could present differences depending on the patient's native language. METHODS: In this cross-sectional study, we analyzed connected speech samples in monolingual English (nfvPPA-E) and Italian speakers (nfvPPA-I) who were diagnosed with nfvPPA and matched for age, sex, and Mini-Mental State Examination scores. Patients also received a comprehensive neuropsychological battery...

OBJECTIVE: To review systematically community-based primary care interventions for epilepsy in low- and middle-income countries to rationalize approaches and outcome parameters in relation to epilepsy care in these countries. METHODS: A systematic search of PubMed, EMBASE, Global Index Medicus, CINAHL, and Web of Science was undertaken to identify trials and implementation of provision of antiseizure medications, adherence reinforcement, and/or health care provider or community education in community-based samples of epilepsy...