Two Cases of Moyamoya Disease Occurred in a Family

Moyamoya disease is a rare obstructive cerebrovascular disease characterized by a cerebral angiographic picture of bilateral stenosis or occlusion of main cerebral arteries with an abnormal vascular network at the base of the brain. Although its pathogenesis is not clear, there is extensive evidence that this disease has tendency to show inheritance and familial occurrence. We have experinced such two cases of moyamoya disease. One was 4-year-old boy who had a history of TIA and bilateral hemiparesis(CaseI) and another was 8-year-old boy. The older brother of the Case I had unilateral symptom. They were treated with EDAS and split duroence-phalosynangiosis and had good results in their neurologic and postoperative angiographic state.

Three Cases of Cerebellar Hemangioblastoma in a von Hippel-Lindau Family: Case Report

Hemangioblastoma is one of the benign tumors in the central nervous system. It is often associated with von Hippel-Lindau disease(VHL disease), a well known autosomal dominant hereditary tumor syndrome. We have experienced three cases of hemangioblastoma associated with von Hippel-Lindau disease in a same family, mother(case 1, 58 years old), son(case 2,19 years old, dead) and daughter(case 3, 17 years old). All of them had cerebellar hemangioblastomas. Renal cyst was associated in the case of mother, but not in the son or daughter. Hemangioblastoma associated with von Hippel-Lindau disease frequently occurres in multiple regions such as cerebellum, spinal cord, medulla oblongata. Also it is frequently presented with renal cell carcinoma, pheochromocytoma, cyst in pancreas, kidney and liver, and epididymal cystadenoma. The age of onset of von Hippel-Lindau disease in the second generation was younger than that of the first generation in this family.

Four Cases of Surgically Managed Distal Anterior Cerebral Artery Aneurysm

A series of 4 patients with aneurysm of distal anterior cerebral artery(DACA), operated with microtechniques, is reviewed. Because the incidence of aneurysms in this location was only 2 to 4% of that of total aneurysm, little expericences on surgical management of this aneurysm have been reported. An aneurysm in this location is difficult to approach surgically because of their multiplicity, characteristically broad and sclerotic base, with small subarachnoid space in the interhemispheric fissure. We surgically treated DACA aneurysms of 4 cases through the interhemispheric approach via the unilateral frontal paramedian craniotomy on the nondominant side.

Peripheral T-Cell Lymphoma of the Spinal Epidural Spaces: Case Report

A case of peripheral T-cell lymphoma, large cell type(NIH) is reported. The literature review yielded a rare case report of a malignant lymphoma especially in the even more rare epidural site. The patient's symptoms were relieved by decompressive laminectomy and subtotal removal of mass and combined scheduled chemotherapy with radiotherapy. The pathological examination revealed malignant lymphoma, diffuse, large cell type(NIH) by H & E and immunologic marker analysis.

Clinical Analysis of External Ventricular Drainage in the Treatment of Intraventricular Hemorrhage

A series of 54 adult patients with intraventricular hemorrhage(IVH) diagnosed by computed tomography(CT) scan and treated with external ventricular drainage were reviewed. In cases who occurred poor drainage of bloody cerebrospinal fluid obstruction, irrigation with urokinase solution(6,000 U/ml) was tried. Hypertension and ruptured aneurysms accounted for 65% of the cases. Although most cases showed poor prognosis, we experienced dramatic improvements in four comatose patients who had fixed dilated pupils after spontaneous intraventricular hemorrhage with secondary acute hydrocephalus. We believe ventriculostomy to be effective in managing IVH especially in the early stage before cerebral herniation occurs. This procedure, accomplished by a convenient and relatively simple technique, led to evacuration of intraventricular blood, or the temporary or permanent cessation of progression of the hydrocephalic process, decrease in ventricular size, and normalization of ICP. However, a relatively high incidence of infection was problematic.