Lennox-Gastaut 증후군을 동반한 대상 이소성 뇌회백질 1례

A Case of Band Heterotopia with Lennox-Gastaut Syndrome

저자들은 다양한 형태의 전신 발작과 경한 지능 발달의 지연을 주소로 내원한 8세 여아에서 뇌 자기공명영상 및 뇌파검사를 시행하여 Lennox-Gastaut 증후군을 동반한 대상 이소성 뇌회백질로 진한하였기에, 임상경과 및 SPECT 소견 들을 정리하여 문헌고찰과 함께 보고하였다.

Band heterotopia is a rare neuronal migration disorder, resulting in epilepsy and mental retardation. Epilepsy in band heteropopia, of which Lennox-Gastaut syndrome constituted about 20%, varied in nature and degree of everity. Band heterotopia can be diagnosed by brain magnetic resonance imaging(MRI), showing another diffuse layer of gray matter underlying the normal-looking cortex with intervening thin rim of white matter. While positron emission tomography(PET) with (18)-fluorodeoxyglucose revealed glucose uptake similar to the overlying cortex, single photon emission computerized tomography(SPECT) findings of band heterotopia have not been reported. We report a 8-year-old girl who presented with variable types of generalized seizures and mild mental retardation. She was diagnosed as having band heterotopia with Lennox-Gastaut syndrome by MRI and interictal electroencephalogram(EEG) showing immature background and generalized 2 ㎐ slow spike and wave complexes. Interictal SPECT, using Tc 99m hexamethyl propylenamine oxide(Tc 99m-HMPAO), revealed the same degree of perfusion in both the areas of band heterotopia and the overlying cortex. By using valproate and lamotrigine, she is now in stable condition with a significant decrease in seizure frequency.