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Journal of Korean Neurosurgical Society 1977;6(1): 221-226.
Temporal Lobe Agenesis Syndrome.
J O Doh, Y S Kim, K C Lee, Y S Hahn, H J Lee
Department of Neurosurgery, Yonsei University, College of Medicine, Seoul, Korea.
ABSTRACT
The temporal lobe agenesis syndrome is the expansion of the bony boundaries of the middle cranial fossa and a deficiency of the temporal lobe with arachnoid cyst. We have seen three unusual cases of the temporal lobe agenesis syndrome. All of the arachnoid cysts in our series behaved as intracranial masses, were associated with some minor neurological deficits and were diagnosed as brain tumor and chronic subdural hematoma preoperatively. In our series, two patients, showed paradoxical changes of the adjoing bony structure of the skull. There was a widening of the middle fossa, elevation of the lesser wing of the sphenoid and thinning of the themporal bone. The carotid angiograms showed the upward and medial displacement of the middle cerebral artery with avascular temporal region and the missing and abnormal course of the middle cerebral vein. We have discussed the pathogenesis of the arachnoid cyst. The osteoplastic craniotomy with removal of arachnoid cyst and opening the passage with the subarachnoid space in the region of the basal cisterns seems effective.
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