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急性発症の痙性麻痺と膀胱直腸障害を主症状とし,MRIにて上部延髄腹側にTl低,T 2高信号域を認めた38歳男性症例を報告する。本例は26歳時に丘疹性ムチン沈着症を発症,同じ頃より光線過敏症,関節痛が出没していた。皮膚,関節症状と抗核抗体高値などから全身性エリテマトーデス(SLE)と診断,神経症状もこれに伴うものと考えられた。SLE患者が延髄や脊髄の障害を合併する頻度は低い。一方,男性がSLEを発症する頻度も低く,その症状は非定型といわれる。本報告ではこの稀な症例につき臨床症状を述べ,急性経過をとる痙性麻痺患者の鑑別にSLEを考慮する必要性があることを考察した。
We report a 38-year-old man systemic lupus erythematosus who presented with an acute onset of paraplegia and urinary retention. The man had a 12 -year history of nodular cutaneous mucinosis andarthralgia. In 1994, he was admitted to our hospital with a sudden onset of weakness and numbness of the right leg followed by an emergence of similar symptoms in the left leg. His elder sister had died at 16 years of age after sufferring from systemic lupus erythematosus for 6 years. On examination, the patient had skin rash on his chest, back, head, fore-head, and extremities.
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