Andrew M, Parr J R, Stacey R, Rosenfeld J V, Hart Y, Pretorius P, Nijhawan S, Zaiwalla Z, McShane M A
Department of Paediatric Neurology, Oxford Radcliffe Hospitals NHS Trust, Oxford, UK.
Childs Nerv Syst. 2008 Feb;24(2):275-9. doi: 10.1007/s00381-007-0448-9. Epub 2007 Sep 9.
Hypothalamic hamartomas (HHs) are commonly associated with severe epilepsy resistant to anticonvulsant therapy. Historically, surgical resection of HHs resulted in considerable morbidity.
Two series of patients who successfully underwent resection using a transcallosal approach have now been published; we report the first UK experience of this technique in a series of five patients with HHs and gelastic epilepsy resistant to anticonvulsant therapy. Patients were assessed pre- and postoperatively for seizure activity, endocrine function, ophthalmology, and neurocognitive function. Two patients had precocious puberty and all had evidence of developmental delay and behavioral problems. Postoperatively, all children experienced at least a 50% reduction in seizure frequency with abolition of major seizure types; one child remains seizure-free. One child developed a mild postoperative right hemiparesis and one developed transient diabetes insipidus.
There were no adverse developmental effects of surgery. Transcallosal resection of HHs ameliorates resistant epilepsy syndromes associated with HH.
下丘脑错构瘤(HHs)通常与对抗惊厥治疗耐药的严重癫痫相关。从历史上看,HHs的手术切除会导致相当高的发病率。
现已发表了两组成功采用经胼胝体入路进行切除的患者系列报道;我们报告了英国首例采用该技术治疗的5例HHs合并对抗惊厥治疗耐药的痴笑性癫痫患者的经验。对患者在术前和术后进行了癫痫发作活动、内分泌功能、眼科及神经认知功能评估。2例患者有性早熟,所有患者均有发育迟缓及行为问题的证据。术后,所有儿童的癫痫发作频率至少降低了50%,主要癫痫发作类型消失;1例儿童无癫痫发作。1例儿童术后出现轻度右侧偏瘫,1例出现短暂性尿崩症。
手术未产生不良发育影响。经胼胝体切除HHs可改善与HH相关的耐药癫痫综合征。