[Hyperthermia in a Shy-Drager syndrome patient--pathophysiological effects of body temperature and L-DOPS on orthostatic hypotension].

A 65-year-old man was admitted to our hospital because of syncope, hyperthermia and urinary disturbance. Neurological examination revealed cerebellar ataxia, muscular rigidity, hyperreflexia with Babinski sign in both sides, and various autonomic dysfunctions including anisocoria, orthostatic hypotension and neurogenic bladder. He was diagnosed as having Shy-Drager syndrome (SDS). Oral administration of L-threo-3,4-dihydroxyphenyl-serine (L-DOPS) (300 mg/day) was started for orthostatic hypotension. After discharge he suffered from pneumonia at his house, and he kept himself warm because of a chill. The patient then fell into hyperthermia (44.0 degrees C), resulting in unconsciousness and a state of shock. He was transferred to our hospital again and was treated by body cooling and drip infusion of dopamine after which he recovered completely within one day. Control of body temperature and blood pressure was examined by heat loading and head-up tilt after heat loading, with or without administration of L-DOPS. These examinations showed that his rectal body temperature rose easily during heat loading and that this phenomenon was enhanced by the administration of L-DOPS. Moreover as his body temperature became higher, he more easily developed syncope due to orthostatic hypotension. It is suggested that in SDS patients, L-DOPS facilitates orthostatic hypotension and syncope in high temperature conditions.