Abstract
The authors has experienced 3 cases of moyamoya disease with sudden onset of intraventricular hemorrhage, which showed the focal neurological signs as hemiparesis or aphasia without the evidence of hemorrhage or infarction in the area of responsible cerebral lesion. In case 1, hypertensive hydrocephalus was recognized at the time of developing the focal neurological signs, and these signs were disappeared along with the improvement of hypertensive hydrocephalus by cerebrospinal fluid drainage and shunt surgery. The focal neurological signs may be produced by the decrease of perfusion pressure in the area, where has been threatened from chronic ischemic condition in moyamoya disease.
In case 2 and 3, although we could not deny the hypertensive hydrocephalic stage, we could followed up these cases without the treatment of hydrocephalus. However, at the time when the focal neurological signs developed, EEG and single photon emission computerized tomography (SPECT) demonstrated the electro-physiological dysfunction and the decrease of cerebral blood flow in the responsible cerebral lesion.
In these cases, the focal neurological signs may be produced by the compression of casting like intraventricular hematoma itself, to the area of circulatory vulnerable condition in moyamoya disease.
