Chapman Kevin E, Kim Do-Young, Rho Jong M, Ng Yu-Tze, Kerrigan John F
Barrow Neurological Institute and St. Joseph's Hospital & Medical Center, Phoenix, AZ 85013, USA.
Epilepsy Res. 2011 May;94(3):218-21. doi: 10.1016/j.eplepsyres.2011.02.004. Epub 2011 Mar 4.
Seizures associated with hypothalamic hamartoma (HH) are notoriously intractable to medical therapy, and while surgical resection affords most affected patients with complete or near seizure-freedom, there remains a need to identify alternative treatments. In this retrospective study, we identified six patients from a large cohort of 220 patients with HH who were treated with the ketogenic diet (KD). Four patients had a 50-90% reduction in multiple seizure types (including gelastic, partial-onset and atonic seizures), and two individuals failed to respond. In order to study possible mechanisms, we then performed microelectrode recordings of small neurons in surgically resected HH tissue slices. Exposure to ketone bodies decreased spontaneous firing in 5 of 7 small HH neurons. These preliminary results suggest that seizures associated with HH may respond favorably to the KD, and that ketone bodies might directly modulate the intrinsic epileptogenicity of HH tissue.
与下丘脑错构瘤（HH）相关的癫痫发作 notoriously 难以通过药物治疗控制，虽然手术切除能使大多数受影响患者完全或几乎不再发作，但仍需要确定其他治疗方法。在这项回顾性研究中，我们从一大组220例HH患者中识别出6例接受生酮饮食（KD）治疗的患者。4例患者多种癫痫发作类型（包括痴笑性、部分性发作和失张力发作）减少了50 - 90%，2例患者无反应。为了研究可能的机制，我们随后对手术切除的HH组织切片中的小神经元进行了微电极记录。7个HH小神经元中有5个暴露于酮体后自发放电减少。这些初步结果表明，与HH相关的癫痫发作可能对KD反应良好，并且酮体可能直接调节HH组织的内在致痫性。
