-related disorders: expansion of the phenotypic spectrum and long-term efficacy of deep brain stimulation.

Heterozygous mutations in are associated with an early-onset, progressive and often complex dystonia (DYT28). Key characteristics of typical disease include focal motor features at disease presentation, evolving through a caudocranial pattern into generalized dystonia, with prominent oromandibular, laryngeal and cervical involvement. Although -related disease is emerging as one of the most common causes of early-onset genetic dystonia, much remains to be understood about the full spectrum of the disease.

Patterns of brain activity in choice or instructed go and no-go tasks.

The goal of this study was to investigate the decision making process for choosing what movements to make. We used electroencephalography (EEG) to investigate patterns of the contingent negative variation (CNV) associated with free-choice decisions to move or abstain, comparing them to conditions where actions were commanded. Our primary hypothesis was that choice tasks would differ significantly from each other and exhibit EEG patterns akin to their command-driven counterparts after the decisions were made, at least, in the 50 ms block of time prior to movement.

Autologous hematopoietic stem cell transplantation for a patient with multiple autoimmune diseases.

Level of autoantibodies after autologous hematopoietic stem cell transplantation. AGA, antigliadin antibody; AHSCT, autologous hematopoietic stem cell transplantation; Anti-GAD65, Ab anti-glutamic acid decarboxylase epitope 65 antibody; Anti-TPO, anti-thyroid peroxidase antibody; CU, chemiluminescent unit.

Gender disparity and abuse in functional movement disorders: a multi-center case-control study.

Background: To determine gender differences in rates of sexual and physical abuse in functional movement disorders compared to controls and evaluate if the gender disparity of functional movement disorders is associated with abuse history.

Methods: We performed a retrospective case-control study of self-reported trauma data from 696 patients (512 women) with functional movement disorders from six clinical sites compared to 141 controls (98 women) and population data. Chi-square was used to assess gender and disorder associations; logistic regression was used to model additive effects of abuse and calculate the attributable fraction of abuse to disorder prevalence.

Cost-effectiveness of Erenumab Versus Surgical Trigger Site Deactivation for the Treatment of Migraine Headaches: A Systematic Review.

Background: Migraine headache is a common, debilitating condition responsible for astronomical societal burden. The chronicity of migraine headaches necessitates the use of many healthcare services. Preventative treatment remains the desirable option for this patient population.

In vivo assessment of neurodegeneration in Spinocerebellar Ataxia type 7.

Spinocerebellar Ataxia type 7 (SCA7) is a neurodegenerative disease characterized by progressive cerebellar ataxia and retinal degeneration. Increasing loss of visual function complicates the use of clinical scales to track the progression of motor symptoms, hampering our ability to develop accurate biomarkers of disease progression, and thus test the efficacy of potential treatments. We aimed to identify imaging measures of neurodegeneration, which may more accurately reflect SCA7 severity and progression.

Gender as a Risk Factor for Functional Movement Disorders: The Role of Sexual Abuse.

Background: The prevalence of functional movement disorders is 2 to 3 times higher in women than in men. Trauma and adverse life events are important risk factors for developing functional movement disorders. On a population level, rates of sexual abuse against women are higher when compared with the rates against men.

Applicability of long-term electroencephalography in pre-mortem diagnosis of Creutzfeldt-Jakob disease: A case report.

Creutzfeldt-Jakob disease accounts for more than 90% of all sporadic prion disease cases. The molecular MM2 genotype has been divided into cortical and thalamic subtypes based on structures involved and is characterized clinically by progressive dementia without ataxia or typical electroencephalography changes. Proposed diagnostic criteria for MM2 cortical type sporadic Creutzfeldt-Jakob disease include progressive dementia, cortical hyper-intensity on diffusion-weighted magnetic resonance imaging, increased cerebrospinal fluid 14-3-3 protein level, and the exclusion of other types of dementia.