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Over the past 20 years, the most notable advance in understanding Guillain-Barré syndrome (GBS) has been the identification of an axonal variant. This advance arose chiefly through studies undertaken in East Asian countries and comprised two major aspects: first, the immunopathogenesis of axonal GBS related to anti-ganglioside antibodies and molecular(More)
BACKGROUND AND PURPOSE Several studies have reported that diffusion-weighted imaging (DWI) is able to help discriminate a Parkinson variant of multiple system atrophy (MSA-p) from Parkinson's disease (PD) on the basis of the increased regional apparent diffusion coefficient (rADC). We analyzed the usefulness of DWI by using the rADC for differential(More)
Although there have been reports regarding the frequent involvement of the pupils in Miller Fisher syndrome (MFS) and related syndromes, internal ophthalmoplegia has not been described as the initial sole manifestation of typical MFS. Recently, we encountered a woman with MFS whose initial manifestation was blurred vision because of bilateral tonic pupils.(More)
The development of hyperexcitability in amyotrophic lateral sclerosis (ALS) is a well-known phenomenon. Despite controversy as to the underlying mechanisms, cortical hyperexcitability appears to be closely related to the interplay between excitatory corticomotoneurons and inhibitory interneurons. Hyperexcitability is not a static phenomenon but rather shows(More)
Although abductor pollicis brevis (APB) and flexor pollicis longus (FPL) share a common peripheral nerve supply, these muscles subserve different functions and may be differently affected in neurodegenerative disease such as amyotrophic lateral sclerosis (ALS). As a consequence, differences in cortical excitability may potentially develop in relation to(More)
The flexor pollicis longus (FPL), a key muscle involved in fractionated thumb movements, may be relatively spared in amyotrophic lateral sclerosis (ALS) compared to the thenar group of muscles, termed the split-hand plus sign. Consequently, the diagnostic utility of the split-hand plus sign was prospectively assessed in ALS. In total, 103 patients (37 ALS(More)
The first dorsal interosseous (FDI) and abductor digiti minimi (ADM) muscles are innervated by the same ulnar nerve, but studies have shown that the former is much more severely affected in amyotrophic lateral sclerosis. In this study, threshold tracking was used to investigate whether membrane properties differ between FDI and ADM motor axons. In 12 normal(More)
Although oral corticosteroids are effective for the treatment of myasthenia gravis (MG), the possibility of steroid-induced exacerbation of symptoms, especially during the initial course of steroid therapy, has limited their use patients with severe MG. However, the factors influencing or predicting in exacerbation are not well understood. The purpose of(More)
Myotonia congenita (MC) is a form of nondystrophic myotonia caused by a mutation of CLCN1, which encodes human skeletal muscle chloride channel (CLC-1). We performed sequence analysis of all coding regions of CLCN1 in patients clinically diagnosed with MC, and identified 10 unrelated Korean patients harboring mutations. Detailed clinical analysis was(More)
Preferential involvement of thenar muscles compared to flexor pollicis longus (FPL), termed 'the split-hand plus sign', appears to be a clinical feature of amyotrophic lateral sclerosis (ALS). In an attempt to understand the pathophysiological mechanisms underlying this clinical phenomenon, threshold tracking transcranial magnetic stimulation techniques(More)