Rinshō shinkeigaku = Clinical neurology
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In this communication, I first show some points we should mind in the conventional peripheral nerve conduction studies and later present clinical usefulness of motor root stimulation for peripheral neuropathy. CONVENTIONAL NERVE CONDUCTION STUDIES (NCS): The most important point revealed by the conventional NCSs is whether neuropathy is due to axonal degeneration or demyelinating process. Precise clinical examination with this neurophysiological information leads us to a diagnosis and treatment. ⋯ Motor root stimulation clearly revealed demyelination in a patient with CIDP in whom sural nerve biopsy findings suggested axonal degeneration, that must be secondary to demyelination. In a patient with tomacular neuropathy, magnetic stimulation revealed conduction delay in the spinal nerve within the spinal canal (Clin Neurol (Jap), 28: 447-452, 1988). Based on the above results, combination of NCSs and magnetic motor root stimulation must brush up the neurophysiological approach to peripheral neuropathy.
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It has been almost 15 years since the first edition of the International Headache Classification appeared in 1988. It was widely accepted and well tolerated. However, rapid progress of the headache research is pushing for a drastic revision of the classification. ⋯ Basically, the most important diagnostic criteria, those of migraine and tension-type headache, remain unchanged. Several new entities such as chronic migraine, hypnic headache, hemicrania continua, benign thunderclap headache and medication overuse headache have been added. This will encourage intensified headache researches in the future.
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Duchenne muscular dystrophy (DMD) is an X-linked, lethal muscle disorder caused by mutations in the dystrophin gene. An adeno-associated virus (AAV) vector-mediated gene transfer is one of attractive approaches to the treatment of DMD, though it has a limitation in insertion size up to 4.9 kb. Therefore, a full-length dystrophin cDNA (14 kb) cannot be incorporated into an AAV vector. ⋯ Especially in the latter occasion, less than 20% of muscle fibers were microdystrophin positive at 24 weeks after the injection, but specific tetanic force of the injected muscle was not statistically different from that of control normal muscle. In conclusion, deltaCS1 micro-dystrophin introduced by an AAV vector could be a powerful tool for the gene therapy of DMD. A bigger animal model, canine X-linked muscular dystrophy will contribute to pre-clinical study of gene therapy.
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Case Reports
[Valproate-induced hyperammonemic encephalopathy in a patient with Sjögren's syndrome].
We describe a 56-year-old woman with hyperammonemic encephalopathy caused by side effect of valproic acid (VPA). Ten months before the admission to our hospital, she had the first attack of convulsive seizure. Diagnosis of epilepsy was made, and the oral administration of VPA (800mg/day) was started at another hospital. ⋯ Other laboratory findings, including positive antinuclear antibody, antibodies to Sjögren's syndrome A, reduced lacrimal secretion in Schirmer's test, and cell infiltration in the salivary gland on lip biopsy specimen, suggested the presence of Sjögren's syndrome. The hyperammonemia occurs by the side effect of VPA, often has basal disease or drug interactions. It was, however, especially in patients with basal disease or other drugs, obscure whether (and how) Sjögren's syndrome contributed to the development of hyperammonemic encephalopathy in this case, since she took only VPA.
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Comparative Study
[Behavioral situation at onset of paradoxical brain embolism due to patent foramen ovale].
The goal of this study was to determine whether there was difference in behavioral situation at onset of stroke between the patients with right-to-left shunt whose stroke was diagnosed as paradoxical brain embolism according to the new criteria proposed by Strategies against Stroke Study for Young Adults in Japan (SASSY-Japan) and whose stroke was not. Among 365 consecutive patients with possible acute ischemic stroke who underwent transesophageal echocardiography, we compared clinical profiles of the following three patient groups: patients with patent foramen ovale whose stroke was diagnosed as paradoxical brain embolism (group A), patients with patent foramen ovale whose stroke was not diagnosed as paradoxical brain embolism (group B), and patients without patent foramen ovale whose stroke was diagnosed as cardiogenic brain embolism (group C). Patent foramen ovale was present on echocardiogram in 76 of 365 patients (21%). ⋯ In group A, the frequency of Valsalva maneuver or long-time sitting position at stroke onset was higher than the frequency of detection of venous thrombus (21%). The result indicates that Valsalva maneuver and long-time sitting position contribute to occurrence of paradoxical brain embolism. We think that these behavioral situations are appropriate diagnostic criteria for paradoxical brain embolism.