約20年にわたり排尿障害が先行し発症したShy-Drager症候群の1剖検例

AN AUTOPSY CASE OF SHY-DRAGER SYNDROME PRECEDED BY URINARY DISTURBANCE FOR OVER 20 YEARS

　抄録　約20年にわたり排尿障害が先行し発症したShy-Drager症候群の1剖検例を報告した。症例は死亡時43歳女性，19歳頃より頻尿，排尿困難，残尿感出現し33歳時無抑制収縮型の神経因性膀胱との診断を受ける。その後尿失禁出現し，排尿困難も著明となり36歳時には混合収縮型神経因性膀胱へと変化した。39歳時より起立性低血圧出現し，42歳時には神経因性膀胱，起立性低血圧の他にHorner徴候，四肢および躯幹の小脳失調，四肢腱反射亢進，四肢の固縮および振戦，構音障害，眼球運動障害を認め，無汗傾向となり，立ちくらみ，めまいのためベッド上臥床状態となった。経時的CT scanによる観察にて，脳幹，小脳の進行性萎縮を認めた。また自律神経機能検査の結果より広範な機能低下の存在が考えられた。43歳時呼吸不全にて死亡，剖検では肉眼的に脳幹，小脳の萎縮を認め，組織学的にも中脳黒質，橋核，延髄下オリーブ核，小脳プルキニエ細胞，脊髄中間外側部，仙髄オヌフ核に強い細胞脱落，グリア増生を認めた。Shy-Drager症候群の報告は現在までに多数見られるが，本例のごとく約20年にわたり排尿障害のみが先行した例の報告はなく，排尿障害のみを呈する症例に対しても長期におよぶ慎重な経過観察が必要と思われる。

An autopsy case of Shy-Drager syndrome pre-ceded by urinary disturbance for over 20 years was reported. A 43-year-old woman was admitted to our hospital because of urinary disturbance and orthostatic hypotension. At the age of 19 she deve-loped urinary disturbance with polyuria and reten-tion. These symptoms were getting worse with years, and at the age of 33 she was diagnosed to have neurogenic bladder of uninhibited type. Dur-ing her hospital course her symptom became worse, and by the age of 42 she showed marked dysarthria, disturbance of smooth pursuit eye mo-vement, Horner's syndrome, marked rigidity and tremor of four extremities, generalized hyperrefle-xia, marked limb and truncal ataxia, neurogenic bladder and orthostatic hypotension. Serial brain CT scan revealed progressive brain stem and cere-bellar atrophy with clinical course. Severe auto-nomic nervous system dysfunctions were also do-cumented. She died of respiratory failure at the age of 43. On autopsy, brain stem and cerebellum showed marked atrophy macroscopically. Micro-scopically marked depletion of neuron was seen in the substantia nigra, pontine nuclei, inferior olive, Purkinje cells, the intermediolateral column of spinal cord and Onuf's nucleus of S 2. Although numerous cases of Shy-Drager syndrome have been reported in the past, there is no case which deve-loped this syndrome after urinary disturbance of over 20 year's duration. We should be alert to observe the cases with longstanding urinary dis-turbances in order to not overlook degenerative disorders as exmplified in this case.