[An association of Shy-Drager syndrome with moyamoya disease--a case report].
A 58-year-old female was admitted to our hospital because of orthostatic syncope, and difficulty in urination and walking for 2 years. At age 35, she suffered from cerebral hemorrhage and was diagnosed as Moyamoya disease (MD). Neurological examination on admission revealed severe orthostatic syncope so that she could not stand. At that time, her blood pressure and heart rate were 104/74 mmHg and 78/min in supine position and 52/48 mmHg and 52/min in 60 degrees head-up position. She also had severe pollakisuria, cerebellar ataxia and dysarthria. Cranial nerves, motor strength and deep reflexes were normal. ECG, chest X-ray, and EEG were normal. Aschner, Czermak, and cold pressor tests revealed no response. Urodynamic study revealed autonomic bladder. MRI showed only enlarged fourth ventricle because of atrophy of the pons. Bilateral CAG revealed "Moyamoya" vessels in the cerebral basal regions. Cerebral blood flow (CBF) was measured by 133Xe inhalation method. Mean arterial blood pressure changed from 134 mmHg to 126 mmHg in 45 degrees head-up tilting and CBF decreased from 47.5 mg/100 g/min to 37 ml/100 g/min position. Though there was no relationship between SDS and MD, each one shows dysautoregulation of CBF, it is supposed that a severe orthostatic syncope attack was resulted from synergism of both effects.
Third Division of Internal Medicine, Shimane Medical University.
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K
Imaoka
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Kobayashi
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J
Fukuda
K
K
Okada
T
T
Tsunematsu
D001157:Arterial Occlusive Diseases / Q000150:complications*; D001342:Autonomic Nervous System Diseases / Q000150:complications*; D002339:Carotid Arteries / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009072:Moyamoya Disease / Q000150:complications* / Q000175:diagnosis; D012791:Shy-Drager Syndrome / Q000150:complications* / Q000175:diagnosis; D013575:Syncope / Q000209:etiology; D014057:Tomography, X-Ray Computed