副腎摘出後，起立性低血圧が増悪したpure progressive autonomic failureの1例

A Patient with Pure Progressive Autonomic Failure whose Orthostatic Hypotension was Aggravated after Adrenalectomy

　41歳，女性．1990年10月起立時のめまいのため当科を受診し，起立性低血圧と診断され経過観察されていた．1992年9月，右副腎腫瘍（non-functioning adenoma）摘出術を施行後，徐々に血圧は低下し，1994年8月に数秒間の意識消失発作が認められたため精査加療目的で当科に入院した．自律神経機能検査では広汎な節後性障害を主体とする交感および副交感神経障害を認めた．中枢神経症状を全く伴わず，純粋に自律神経症状のみを呈することからpure progressive autonomic failure（pure PAF）と診断した．L-threo-3,4-dihydrox-yphenylserineの投与により，起立性低血圧，自覚症状は著しく改善した、pure PAFの女性例は比較的稀であり，本症例では起立性低血圧の増悪に副腎摘出が関与した可能性が考えられた．

The case of a woman with pure progressive auto-nomic failure (PAF) whose orthostatic hypotension was aggravated after adrenalectomy was reported. The 37-year-old woman visited our hospital in October 1990, because of dizziness on standing. Since her blood pressure was 96/40mmHg in standing position and 110/ 70mmHg in recumbent position, she was diagnosed as having mild orthostatic hypotension at the initial visit and was followed up without medication. In September 1992, she underwent right adrenalectomy because of non-functioning adenoma. Thereafter, her symptoms and orthostatic hypotension became progressively worse. In August 1994, she had a syncopal attack and was admitted to our hospital. She showed marked orthostatic hypotension (recumbent; 112/65 mmHg, sitting; 66/42mmHg) without increases in plasma catecholamine levels. Autonomic function tests revealed both sympathetic and parasympathetic dysfun-ctions of mainly postganglionic origins. Other physical examinations on admission including neurological and mental status examinations showed no abnormalities. A MRI scan of the central nervous system also showed no abnormalities. From the onset, she had signs of neither Parkinsonism nor cerebellar or peripheral neur-opathy. From these findings, we diagnosed her as pure PAF. Treatment with L-threo-3, 4-dihydroxyphenyl-serine increased her blood pressure level and markedly improved her symptoms which had been caused by orthostatic hypotension. Since her blood pressure de-creased and orthostatic hypotension became worse after right adrenalectomy, it is suggested that aldoster-one and cortisol released from the adrenal gland may play an important role in maintaining blood pressure in patients with pure PAF.