[A case of "pure" progressive autonomic failure with impairment of cerebral blood flow autoregulation].
A 43-year-old male with "pure" progressive autonomic failure ("pure" PAF) was admitted to our hospital in June 1988, with a chief complaint of recent syncopal attacks while standing. Since the age of 35. He has noticed dyshidrosis on his left side. The family history was negative. Neurological examination showed dyshidrosis on his left side and profound orthostatic hypertension. No other neurological abnormalities were noted. Autonomic function tests revealed sympathetic and parasympathetic dysfunction as follows: Adrenaline administered subcutaneously gave a hyperactive pressor response from 136/82 mmHg to 190/116 mmHg with the pulse increasing from 54 to 150 beats per min. Hidrosis was observed on the right side of the body in response to heat. However, it was observed over the whole body in response to pilocarpine. In the studies of pupillary response to instillation of 1.25% epinephrine, both pupils were unchanged in diameter; after 5.0% tyramine, both pupils were dilated from 3.5 mm to 5.0; and after 2.5% methacholine, the right and left pupils, each originally 4.5mm were constricted to 3.5 mm and 4.0 mm, respectively. From the above data, this case was diagnosed as "pure" PAF. 133Xe inhalation method. The mean regional CBF (F1) values were reduced from 91 to 53 ml/100 g brain/min by postural change from supine to sitting position, associated with a decrease in mean arterial blood pressure (MABP) of 28 mmHg (from 103 to 75 mmHg). This result shows the impairment of CBF autoregulation in this patient. This case adds further evidence to the hypothesis that the autonomic nervous system plays a role in CBF autoregulation.
Nishimura
H
H
Tachibana
H
H
Takeda
K
K
Tsuchiyama
M
M
Sugita
M
M
D000328:Adult; D001342:Autonomic Nervous System Diseases / Q000503:physiopathology*; D002560:Cerebrovascular Circulation; D006706:Homeostasis / Q000502:physiology*; D006801:Humans; D007024:Hypotension, Orthostatic; D008297:Male; D011674:Pulse