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Case No. 179
0000-00-00~0000-00-00
출제자 : 관리자
Hit : 307
| Period | 0000-00-00~0000-00-00 |
|---|---|
| Diagnosis | |
| Clinical information | 9세 남아, Seizure로 내원. |
| Discussion | Tuber cinereum hamartoma Hypothalamic hamartomas (tuber cinereum hamartomas) are developmental malformations which cause the classic triad of gelastic epilepsy, central precocious puberty and developmental delay. The seizures are of varying severity but have a progressive course in the majority leading to a catastrophic epilepsy associated with cognitive and behavioural decline. The hypothalamic hamartoma has been shown to be intrinsically epileptogenic and most likely generates generalized seizures through the mamillothalamic pathways. Increased T2-weighted signal intensity relative to gray matter, lack of contrast enhancement, and stable lesion size are the most frequently reported MR imaging features of hypothalamic hamartomas. The transcallosal anterior interforniceal route currently offers the best chance for seizure freedom with the least surgical morbidity, although the newer alternative modalities of endoscopic disconnection and radiosurgery are currently being evaluated. References 1. Maixner W. Hypothalamic hamartomas--clinical, neuropathological and surgical aspects. Childs Nerv Syst 2006;22:867-73. 2. Freeman JL, Coleman LT, Wellard RM, Kean MJ, Rosenfeld JV, Jackson GD, Berkovic SF, Harvey AS. MR imaging and spectroscopic study of epileptogenic hypothalamic hamartomas: analysis of 72 cases. AJNR Am J Neuroradiol 2004;25:450-62. |
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