Abstract
A 10-year-old girl with moyamoya disease was reported, who presented Gerstmann's syndrome. She had developed numbness of the right upper limb from 9 years of age and was admitted because of amnestic aphasia and Gerstmann's syndrome following numbness of the right upper and lower limbs at 10 years and 10 months of age. Bilateral carotid angiography revealed obstruction of bilateral carotid arteries at the point that both posterior communicans arteries branched, and cerebral telangiectasis along the middle cerebral arteries. Brain CT scanning on the 12 th day after the onset revealed a high density lesion in the posterior temporal to occipital area on the left hemisphere. One week after operation of encephalo-duro-STA-synangiosis, all the symptoms disappeared.
Systemic neuropsychological examination was done ; intelligence test, Illinois test of psycholinguistic abilities, developmental test of visual perception, McCarthy scales of children's abilities, Bender Gestalt test and diagnostic test for reading disabilities. As the result, selective disabilities of visual fixation as well as constructive apraxia and spatial agnosia were demonstrated. Therefore it was suggested that the systemic neuropsychological studies were necessary to reveal a basic mechanism of Gerstmann's syndrome and also it was useful to find symptoms complicated with this syndrome.
