Rinshō shinkeigaku = Clinical neurology
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Cluster headaches are characterized by strictly unilateral paroxysmal attacks of severe pain with associated autonomic sign and symptom. Prevalence is 5 times higher in men than in women in our cases. About 10-15% of patients have chronic symptoms without remissions, but we estimated less frequent in Japanese (6.6% in our series). ⋯ Treatment of cluster headache includes both acute therapy aimed at aborting individual attacks and prophylactic therapy aimed at preventing recurrent attacks during the cluster period. There are many choices using for both therapies. Based on our clinical experience, we recommended the combination of nasal sumatriptan for acute attacks and verapamil 240 mg/day for prophylaxis.
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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.