We surveyed patients with multifocal motor neuropathy (MMN) in comparison with those with amyotrophic lateral sclerosis (ALS) in Japan. This retrospective study consisted of 47 patients with MMN and 1,051 patients with ALS from major neuromuscular centers in Japan from 2005 to 2009. The ratio of MMN to ALS patients (0-0.10) varied among the centers, but mostly converged to 0.05. The prevalence was estimated to be 0.3 cases for MMN and 6.63 cases for ALS per 100,000 persons. Twenty-seven of 47 patients (56.5%) showed conduction block (CB). Of the 45 patients who received intravenous immunoglobulin (MVg), 34(75.6 %) demonstrated clear clinical improvement.
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Intravenous immunoglobulin (IVIG) is an immunomodulatory therapy derived from pooled donor immunoglobulins and used for treatment of various autoimmune conditions. Here we report the diagnosis and management of IVIG-induced chronic severe neutropenia with absolute neutrophil count <0.5×10/µL in a patient with multifocal motor neuropathy.
View Article and Find Full Text PDFBiomedicines
January 2025
Second Department of Internal Medicine, Division of Nephrology, Kansai Medical University, Hirakata 573-1010, Japan.
: Charcot-Marie-Tooth (CMT) disease is an inherited peripheral neuropathy primarily involving motor and sensory neurons. Mutations in INF2, an actin assembly factor, cause two diseases: peripheral neuropathy CMT-DIE (MIM614455) and/or focal segmental glomerulosclerosis (FSGS). These two phenotypes arise from the progressive degeneration affecting podocytes and Schwann cells.
View Article and Find Full Text PDFClin Neurophysiol
January 2025
Department of Clinical Neurophysiology, Rigshospitalet, Copenhagen, Denmark; Department of Clinical Medicine and Neuroscience, University of Copenhagen, Denmark; Department of Neurology, Rigshospitalet, Copenhagen, Denmark.
Objective: To investigate motor axonal excitability in multifocal motor neuropathy (MMN) associated with involuntary muscle activity.
Methods: Two MMN patients with continuous involuntary finger movements (MMNifm) were compared to 11 patients without movements (MMNnfm). Clinical examination, EMG of the abductor pollicis brevis muscle, nerve conduction studies, motor unit number estimation, excitability studies, and mathematical modeling were conducted in the patients with MMN and compared to controls.
NeuroSci
December 2024
Department of Pediatrics, Dokkyo Medical University, Tochigi 321-0293, Japan.
Background: Acute disseminated encephalomyelitis (ADEM) is a rare, immune-mediated inflammatory disorder of the central nervous system (CNS), typically characterized by the acute onset of multifocal demyelination. The pathogenesis of ADEM remains unclear, but it is believed to be triggered by an autoimmune response, often following viral infections or vaccinations.
Case Report: This case report describes a 3-year-old child who developed ADEM after receiving two concurrent influenza vaccines: one for seasonal influenza and one for the 2009 H1N1 pandemic.
Neuroscience
January 2025
School of Health and Human Sciences, Indiana University Indianapolis Indianapolis IN USA.
Most activities of daily life involve some degree of coordinated, bimanual activity from the upper limbs. However, compared to single-handed movements, bimanual movements are processed, learned, and controlled from both hemispheres of the brain. Transcranial direct current stimulation (tDCS) is a non-invasive brain stimulation technique that enhances motor learning by modulating the activity of movement-associated brain regions.
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