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I.はじめに
Amyloid polyneuropathyは稀な遺伝性ニューロパチーで,外国では少数の報告がみられるが1)3)5)6)11)12)13),わが国では中尾ら10)と著者ら2)の報告があるに過ぎない。
従来,ニューロパチーの研究には,末梢神経伝導速度が広く用いられており,特にCharcot-Marie-Tooth病やHereditary sensory radicular neuropathyなどの遺伝性ニューロパチーでは,罹患例のみならず発病前期の保因者にも運動神経伝導速度(以下MCVと略す)の低下がみられ,MCVはこれらの遺伝性ニューロパチーの家系において,保因者を発見する有力な手段となることが明らかにされている4)7)〜9)。
The measurements of motor nerve conduction velocities of the ulner and tibial nerves were carried out on the six affected and four non-affected membersin a family which included 25 patients of amyloid polyneuropathy.
Motor nerve conduction velocities were decreased only in the affected members, and the degree of the decrease was correlated with duration after onset of the neurological symptomes.
In two affected members, sural nerve biopsies and motor nerve conduction velocities were examined, and motor nerve conduction velocities were not parallel with quantity of the deposits of amyloid materials in the endoneurium.
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