[Cystic brain tumor associated with tuberous sclerosis -familial occurrence of tuberous sclerosis with normal intelligence- (author's transl)].
Brain tumors associated with tuberous sclerosis are often found in the wall of the ventricles near the foramen of Monro and present with symptoms of increased intracranial pressure due to occlusion of the CSF pathway. The authors reported a case of such a brain tumor which presented predominantly focal neurological signs because of a large cyst function. This 8-year-and-4-month old girl experienced her first seizure attack at the age of 1-year-and-3-month. Six moths before admission, she complained of headache which lasted for several days. CT scans at that time revealed mass lesions in both lateral ventricles which were mildly dilated. A round low density area, considered to be a cyst, was found lateral to the calcified tumor of the left lateral ventricle extending into the adjacent parenchyma. Two months later, she noticed weakness in her right extremities, difficulty in buttoning and tendency to stumbling, and these were slowly progressive. Her past history was non-contributory. CT examinations of her mother and elder brother with adenoma sebaceum indicated that both had multiple calcifications in the ventricular walls and they were clinically diagnosed as having an incomplete form of tuberous sclerosis. On admission to the Department of Neurosurgery, Shinshu University Hospital on March 23, 1979, the patient was noted to have right hemiparesis and hyper-reflexia. She could barely walk but was unable to write with her right hand. Fundoscopic examination was normal. On general physical examinations, characteristic skin lesions including adenoma sebaceum, shagreen patch and depigmented patches were found. The second CT scans taken at the time of admission revealed that the low density area markedly enlarged, while the size of the tumors and ventricles remained essentially unchanged. A left parietal craniotomy was performed with a preoperative diagnosis of cystic tumor of the lateral ventricle associated with tuberous sclerosis. The tumor in the left lateral ventricle was found extending from the trigone to the anterior horn beyond the foramen of Monro. Histopathological diagnosis of typicaL benign giant-celled astrocytoma was made. The immediate postoperative course was uneventful except for a temporary worsening of the right motor weakness. V-P shunt was necessary because she developed signs and symptoms of increased intracranial pressure due to hydrocephalus two weeks after operation. At the time of dismissal from the hospital one month postoperatively, she was neurologically free except for the mild right hemiparesis. To the best of our knowledge, the present case is the first reported in the literature, in which a brain tumor associated with cyst formation verified in a patient of tuberous sclerosis.