[A case report of cerebral venous dysgenesis]

A case classified to "cerebral venous dysgenesis" (Kagawa et al 1972) is reported. A 16-year-old girl was admitted complaining of psychomotor seizure which began seven years before. At the first admission (seven years ago) she was diagnosed as psychomotor seizure and cerebral venous dysgenesis by CT scan, EEG and cerebral angiography. Then she was treated by medication (anticonvulsants) for seven years. At the second admission (this time) left temporal lobectomy was performed by means of monitoring the cortical EEG. CT scan showed atrophy of the left temporal lobe. Spike and wave complex was seen in EEG. Carotid angiography disclosed the abnormal networks among the fine venules and their connection to tortuous and coiling veins. The abnormality was seen in the frontal, parietal, and temporal area. After operation psychomotor seizure has been controlled by medication. Pathologically tortuous vessels, gliosis and arachnoidal fibrosis were shown. This case should be classified to "cerebral venous dysgenesis" differentiated from the venous angioma. Classification of venous malformation, clinical signs, angiographical findings, and genesis were discussed.