NEURO

Intravenous immunoglobulins (IVIg) represent an established cornerstone for the immunotherapy of chronic inflammatory demyelinating polyneuropathy (CIDP). Efficacy of IVIg for CIDP was proven in a large phase III trial. Yet, data on longer-term efficacy and effects in distinct subgroups are scarce. Our trial investigates the long-term efficacy of IVIg treatment in CIDP patients.In this observational real-world study, we retrospectively analyzed 49 CIDP patients receiving continuous IVIg treatment with a mean initial dosage of 87 g (1 g/kg body weight) every 4 weeks over a mean time of 45 months between 2010 and 2018. from Journal of the Neurological Sciences https://ift.tt/3jw4kET

Cerebral microinfarcts (CMI) are common lesions, carrying an important contribution to small-vessel–related cognitive impairment. CMIs were previously found to cause local microstructural damage and disruption of white matter integrity. This study examines CMIs influence on cortical thickness in remote brain areas. from Journal of the Neurological Sciences https://ift.tt/3jtatBv

Healthy brain tissue pulsates with the cardiac cycle, but whether brain tissue pulsations (BTPs) are impaired by tissue ischemia due to ischemic stroke is currently unclear. This study is the first to explore the clinical potential of measuring BTPs using ultrasound in acute ischemic stroke patients.BTPs were measured in 24 healthy volunteers (aged 52–82 years) and 14 acute ischemic stroke patients (aged 51–86 years) using a novel Transcranial Tissue Doppler (TCTD) method. Measurements were quick to perform and were well tolerated by all subjects. from Journal of the Neurological Sciences https://ift.tt/3l2pwTg

Neurometabolic causes of dystonia are heterogenous and can be challenging to diagnose, yet many of these disorders are potentially treatable. The first step in the workup is to clinically phenotype the underlying condition, followed by ordering selected diagnostic tests based on the clinician's judgement and clinical suspicion. In this review, we highlight the diagnostic clues to various disorders, including lysosomal storage diseases, mitochondrial cytopathies, metal storage disorders, organic acidurias, disorders in carbohydrate metabolism, neurotransmitter diseases and vitamin and cofactor deficiencies. from Journal of the Neurological Sciences https://ift.tt/36t2Vev

Introduction: Our previous community-based study demonstrated that some individuals with AVIM [asymptomatic ventriculomegaly with features of idiopathic normal pressure hydrocephalus (iNPH) on magnetic resonance imaging (MRI)] progressed to iNPH in several years. In this hospital-based study, we investigated the progression rate from AVIM to iNPH and its possible predictors.Methods: We conducted a prospective study of participants with AVIM from several medical institutions/hospitals in Japan. AVIM is defined as “asymptomatic ventriculomegaly with features of iNPH on MRI”; in the present study, asymptomatic was defined as “0 (no symptoms) or 1 (presence of only subjective, but not objective, symptoms) on the iNPH Grading Scale (iNPH-GS).” We also measured possible predicting factors for AVIM-to-iNPH progression, including age, sex, body weight, blood pressure, diabetes mellitus, dyslipidemia, history of mental disease/head injury/sinusitis/smoking/alcohol-intake, Evans index, and the p

Abnormalities of eye movement and visual processing are common in traumatic brain injury. The King-Devick test (KDT) has been widely used in the detection and recovery of concussion. Current recommendations propose performing the initial test at baseline and then repeating annually to account for potential learning effects. In practice, this may still account for large deviations. The aim of this study was to determine the number of trials needed for a player to achieve a ceiling effect and to determine the validity of the existing protocol requiring two tests at baseline. from Journal of the Neurological Sciences https://ift.tt/2EYKbbw

There has been an increasing prevalence of Moyamoya disorder (MMD) reported from recent US literature. There is a paucity of data available regarding trends of prevalence and epidemiological factors in the United States. To test the hypothesis that racial-, sex-specific MMD hospitalizations and epidemiological factors have been increasing in the United States over the last decade. from Journal of the Neurological Sciences https://ift.tt/3nb27kd

Anosmia is common in Coronavirus disease 2019, but its impact on prognosis is unknown. We analysed whether anosmia predicts in-hospital mortality; and if patients with anosmia have a different clinical presentation, inflammatory response, or disease severity. from Journal of the Neurological Sciences https://ift.tt/34ex6TN

The intracranial arterial vasculature has numerous anatomical variants, which vary from largely benign to having remarkable clinical implications. The significance of a lesser known variant described as a junctional dilatation of the basilar artery tip has not yet been described in the literature. from Journal of the Neurological Sciences https://ift.tt/344GyJD

Bilateral optic disc edema (ODE) with preserved visual function is typically a result of papilledema, but the causes of this finding in the absence of raised intracranial pressure have not been systematically evaluated. The goal of this study was to determine the causes of bilateral ODE with preserved visual function not related to intracranial hypertension to help in developing a differential diagnosis for this finding. We retrospectively reviewed 221 consecutive patients presenting to a tertiary neuro-ophthalmology practice over a period of 2 years. from Journal of the Neurological Sciences https://ift.tt/3i3o3tW

Acute infections have been posited as potential precipitants or triggers of the occurrence of stroke among adults with traditional vascular risk factors. We evaluated associations between stroke occurrence and reported febrile illness within 4 weeks (potential antecedent infections) among West Africans. from Journal of the Neurological Sciences https://ift.tt/2Ge3LR3

Epidemiology provides an avenue for identifying disease pathogenesis, hence determining national incidence, along with socioeconomic and demographic variables involved in iNPH, can provide direction in elucidating the etiology and addressing healthcare inequalities. from Journal of the Neurological Sciences https://ift.tt/3kJpMpQ

Mutations in the transient receptor potential vanilloid 4 (TRPV4) gene, encoding a polymodal Ca2+ permeable channel, have been associated with a spectrum of dominantly inherited skeletal dysplasias and neuropathies. The clinical manifestations of TRPV4-associated disorders are highly heterogeneous. This study describes a large Chinese family with a novel mutation in the TRPV4 gene. Nineteen individuals from this family were investigated. Clinical, electrophysiological, and radiographic examinations were performed. from Journal of the Neurological Sciences https://ift.tt/361gQIu

Dear Editor, from Journal of the Neurological Sciences https://ift.tt/2EqosZN

Spinocerebellar ataxia type 36 (SCA36) is an autosomal dominant, slowly progressive cerebellar syndrome caused by unstable large expansion of GGCCTG hexanucleotide repeat (normal: 3–14 repeats, disease: 650–2500 repeats) in the first intron of the nucleolar protein 56 gene [1–3]. I read with interest the article by Ota et al. [4], which genetically characterized the first Japanese SCA36 family showing clinical anticipation. So far, there had been no or little evidence of association between number of GGCCTG repeat and age at onset of SCA36 patients. from Journal of the Neurological Sciences https://ift.tt/300889B

The motor weakness in sporadic hemiplegic migraine (SHM) is a poorly understood aura manifestation. Cortical spreading depression affecting motor excitability and alterations of neurovascular coupling may be integral to the development of migraine aura. from Journal of the Neurological Sciences https://ift.tt/2RMNrto

We appreciate the criticism raised by Dr. Matsuura questioning the genetic evaluation for NOP56 gene of Asidan (spinocerebellar ataxia 36) showing clinical anticipation. Asidan is an autosomal dominant neurodegenerative disorder caused by a hexanucleotide GGCCTG repeat expansion in intron 1 of the NOP56 gene, showing cerebellar ataxia, motor neuron disease phenotype, hearing loss and frontal cognitive impairment [1–3]. The NOP56 genetic mutation in Asidan is similar to the hexanucleotide GGGGCC repeat expansion in intron 1 of the C9orf72 gene observed in familial amyotrophic lateral sclerosis/frontotemporal dementia accompanied by Purkinje cell degeneration [4,5], suggesting the similar genetic and molecular mechanism between NOP56 and C9orf72 gene mutations [5]. from Journal of the Neurological Sciences https://ift.tt/3mAlcfH

Given the high prevalence of individuals diagnosed with substance use disorder, along with the elevated rate of relapse following treatment initiation, investigating novel approaches and new modalities for substance use disorder treatment is of vital importance. One such approach involves neuromodulation which has been used therapeutically for neurological and psychiatric disorders and has demonstrated positive preliminary findings for the treatment of substance use disorder. The following article provides a review of several forms of neuromodulation which warrant consideration as potential treatments for substance use disorder. from Journal of the Neurological Sciences https://ift.tt/3iQSK6P

Multiple sclerosis (MS) patients have been considered a higher-risk population for COVID-19 due to the high prevalence of disability and disease-modifying therapy use; however, there is little data identifying clinical characteristics of MS associated with worse COVID-19 outcomes. Therefore, we conducted a multicenter prospective cohort study looking at the outcomes of 40 MS patients with confirmed COVID-19. Severity of COVID-19 infection was based on hospital course, where a mild course was defined as the patient not requiring hospital admission, moderate severity was defined as the patient requiring hospital admission to the general floor, and most severe was defined as requiring intensive care unit admission and/or death. from Journal of the Neurological Sciences https://ift.tt/33MWlwk

Apraxia of eyelid opening (AEO) refers to impaired voluntary eyelid elevation of supranuclear origin. AEO is well-described in neurodegenerative disorders, but its frequency in stroke is unknown. from Journal of the Neurological Sciences https://ift.tt/2REoss1