Abstract
A case of so-called “moyamoya” disease with intracerebral hematoma is reported. This 39-year-old man was transfered from another hospital to Kitasato University Hospital on May 24, 1977, because of disturbance of consciousness and left hemiplegia. He was well until 7 days prior to admission, when he suddenly noticed severe headache and weakness of the left arm and leg. 3 days prior to admission, he became stuporous.
On admission, he reacted only to painful stimuli. The left pupil was 4 mm in diameter while the right was 3 mm. The both pupils reacted to light briskly. Deep tendon reflexes of the left upper and lower extremities were markedly increased. The Babinski's sign was positive on both sides.
Right carotid angiogram showed occlusion of the supraclinoid portion of the internal carotid artery. There was an abnormal vascular network, which had characteristics of that of “moyamoya” disease, just above the site of occlusion. Through the abnormal vascular network, ascending branches of the middle cerebral artery were faintly opacified. The anterior cerebral artery was opacified via the posterior pericallosal artery and deviated to the left. The posterior communicating and posterior cerebral arteries were dilated and displaced downwards.
The computed tomography showed a large hematoma located in the right putaminal region. On May 25, right frontoparietal craniotomy was performed and the intracerebral hematoma was evacuated. An anastomosis between the superficial temporal artery and the cortical branch of the middle cerebral artery (STA-MCA anastomosis) was performed at the same time. The patient made a remarkable recovery following the surgery. His consciousness became normal within 3 days. The left-sided motor palsy also showed marked improvement. When seen 6 months after the operation, he had only mild residual weakness of the left hand and resumed a full-time work as a business man.
Repeated angiography (Nov. 14, 1977) showed excellent patency of the anastomosis, with extensive irrigation of the right middle cerebral territories through the anastomosis. The “moyamoya” vessels were almost completely disappeared at this time.
There are two mechanisms to develop clinical manifestations in the so-called “moyamoya” disease. One is cerebral ischemia due to occlusion of the major vessels at the base of the brain. The other one is hemorrhage which is considered to be from rupture of the fragile thin-walled “moyamoya” vessels or from aneurysms arised from these vessels. The former mechanism is mainly observed in younger age group and the latter is seen in adult cases.
There is no doubt that the creation of STA-MCA anastomosis increases blood flow and thus prevents cerebral ischemia to a certain extent in this condition. At the same time, the creation of new route of blood supply results in decreasing necessity of the “moyamoya” vessels which have been considered to be markedly dilated small collaterals. As a fact, in the present case, the “moyamoya” vessels have almost completely disappeared following the placement of the STA-MCA anastomosis as proved by follow-up angiographic studies. By decreasing blood flow through the “moyamoya” vessels by this surgery, the authors believe that the chances of hemorrhaging from these abnormal vessels will be markedly decreased.
