신하영 교수

BACKGROUND: Spinal cord involvement in Behçet's disease is not well studied. OBJECTIVE: To evaluate the clinical, laboratory and magnetic resonance imaging characteristics of spinal cord involvement in Behçet's disease. METHODS: We retrospectively reviewed 10 spinal cord involvements in seven patients with Behçet's disease. RESULTS: The median age of onset for spinal cord involvement was 32 (23-45 years). Two patients showed a secondary progressive course. Cerebrospinal fluid findings revealed m

Importance: A proportion of people with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) have a relapsing disease course and persistent anti-myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) seropositivity. Few studies have investigated whether treatment of the first MOGAD attack is associated with the long-term disease course and/or MOG-IgG seronegative conversion. Objective: To investigate the association of time to treat the first acute MOGAD attack with re

INTRODUCTION: Predictive factors for myasthenic crisis after transsternal thymectomy have been reported, but little is known about myasthenic crisis after videoscopic thymectomy (MCAVT). METHODS: We investigated 146 myasthenia gravis patients who underwent videoscopic thymectomy. RESULTS: Patients with MCAVT had a lower forced vital capacity (FVC) (2.1 vs. 3.0 L, P < 0.001) than those without. Low-frequency repetitive nerve stimulation showed decremental responses of the orbicularis oculi (47.1%

BACKGROUND AND PURPOSE: Patients with muscle-specific tyrosine kinase (MuSK) antibody (MuSK-Ab)-positive myasthenia gravis (MG) show distinct responses to acetylcholinesterase inhibitors (AChEIs). Although clinical responses to AChEIs in MuSK-Ab MG are reasonably well known, little is known about the electrophysiologic responses to AChEIs. We therefore investigated the clinical and electrophysiologic responses to AChEIs in MuSK-Ab-positive MG patients. METHODS: We retrospectively reviewed the me

The present results demonstrate that putaminal atrophy in MSA as measured on brain MRI represents an effective tool for differentiating MSA from PD.

Background and purpose Anti-titin antibodies are antistriational antibodies associated with thymoma-associated myasthenia gravis (MG). We evaluated whether the patients with anti-titin antibody are more frequently hospitalized to manage thymoma-associated MG than those patients without anti-titin antibody. Methods Patients with thymoma-associated MG who conducted the serological test for anti-titin antibody were retrospectively included. Disease severity, treatments, MG-related annual hospitaliz

To explore the clinical significance of anti-cytosolic 5'-nucleoditase 1A (NT5c1A) antibody seropositivity in inflammatory myopathies, we measured anti-NT5c1A antibodies and analyzed their clinical features. Anti-NT5c1A antibodies were measured in the sera of 103 patients with inflammatory myopathies using an enzyme-linked immunosorbent assay. Positivity for anti-NT5c1A antibody was found in 13 (12.6%) of 103 patients with inflammatory myopathy. Anti-NT5c1A antibody was most frequently identifie

The risk of osteoporosis in patients with chronic inflammatory neuropathy (CIN) has not been evaluated in detail. We conducted a population-based case-control study nested in a retrospective cohort to analyze osteoporosis risk among patients with CIN using a nationwide database. Patients with CIN based on the Korean Classification of Disease diagnostic code were included and were matched to controls. A Cox proportional hazards regression model was used to evaluate the effect of CIN on osteoporos

title: Thymectomy and disease duration in non-thymomatous acetylcholine receptor antibody-positive myasthenia gravis: a single-centre, cross-sectional study, doi: 10.1136/jnnp-2022-329834, category: Article

INTRODUCTION: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients possess multiple risk factors for osteoporosis, but few studies have evaluated bone mineral density (BMD) in CIDP patients. METHODS: We retrospectively compared the BMD of CIDP patients with that of normal controls, and evaluated the clinical factors associated with osteoporosis. RESULTS: Total BMD was lower in CIDP patients than in normal controls (P = 0.017). In a comparison of 16 osteoporotic CIDP patients

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), which is caused by mutations in SACS gene, is a very rare neurodegenerative disorder characterized by the clinical triad of early onset cerebellar ataxia, pyramidal tract features, and sensorimotor polyneuropathy. Herein, we report a 35-year-old Korean male who presented with gait disturbance and lower extremity weakness. Neuroimaging and ophthalmologic evaluation revealed features consistent with ARSACS. Mutation in SACS gene w