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患者は15歳男性。処女歩行遅延以外に明らかな発達異常なし。亜急性に四肢筋力低下が出現。足の変形,足関節拘縮がみられ,神経伝導検査上著明な伝導障害の所見と髄液蛋白の軽度上昇を認めた。腓腹神経生検で著明なonion bulb様変化がみられ,一部血管周囲の細胞浸潤の所見も呈した。ステロイドパルス療法により筋力は著明に改善した。遺伝性疾患は否定的であり,慢性炎症性脱髄性多発根神経炎(chronic inHammaーtory demyelinating polyradiculoneuropathy:CIDP)と診断した。しかし遺伝性の末梢神経障害を疑わせる経過,所見,および脱髄性変化の高度な病理像はCIDPとしては非典型的であった。
A 15-year-old male developed symmetrical weak-ness of the limb muscles. He had not had any previ-ous developmental disorders except delayed initiation of walking. Flexion contraction of ankle joint and pes cavus deformity were seen. The cerebrospinal fluid protein concentration was elevated. Nerve conduction study showed severe conduction block and temporal dispersion. A rural nerve biopsy revealed remarkable onion bulb-like changes and perivascular infiltration of inflammatory cells. After high-dose corticosteroid treatment, he showed improvement in muscle strength.
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