Two Cases of Tuberous Sclerosis Associated with Subependymal Giant Cell Astrocytoma.

Ko, Yong; Kim, Kwang Myung; Chung, Hwan Yung; Kim, Nam Kyu; Oh, Suk Jun; Park, Chan Geum; Lee, Jung Dal; Lee, Seung Ro; Hahm, Chang Gok

Original Article

Journal of Korean Neurosurgical Society 1988;17(3): 535-544.

Yong Ko, Kwang Myung Kim, Hwan Yung Chung, Nam Kyu Kim, Suk Jun Oh, Chan Geum Park, Jung Dal Lee, Seung Ro Lee, Chang Gok Hahm

¹Department of Neurosurgery, School of Medicine Hanyang University, Korea.
²Department of Pathology, School of Medicine Hanyang Universty, Korea.
³Department of Radiology, School of Medicine Hanyang University, Korea.

ABSTRACT

Neurosurgical intervention in the syndrome is discussed in the light of two recently treated cases, one of which was admitted due to acute hydrocephalic attack, the other was confirmed by the typical computed tomographic findings as well as the classic triad of adenoma sebaceum, mental retardation and epilepsy. There cases had no family historty and were probably the result of new mutations and variable penetrance. Transcallosal microsurgical excision of tumor was accomplished in both cases and subependymal giant cell astrocytoma was confirmed. The hydrocephalus resolved but subduroperitoneal shunt was required in one case.

Key Words: Adenoma sebaceum; Mental retardation; Epilepsy; Phakoma; Subependymal giant cell astrocytoma