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一名幼儿第四脑室错构瘤内的病灶性癫痫样活动与癫痫性半面痉挛相关:病例说明

Intralesional epileptiform activity in a fourth ventricular hamartoma associated with epileptic hemifacial spasm in a toddler: illustrative case.

作者信息

Phillips H Westley, Papadakis Joanna E, Borden Brittany A, Pinto Anna, Tsuboyama Melissa, Chiu Michelle Y, Madsen Joseph R, Patil Rachit, Stone Scellig S D

机构信息

Department of Neurosurgery, Stanford University School of Medicine, Palo Alto, California.

Departments of Neurosurgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.

出版信息

J Neurosurg Case Lessons. 2024 Jul 29;8(5). doi: 10.3171/CASE2452.

Abstract

BACKGROUND

Focal epilepsy caused by a posterior fossa lesion is a rare phenomenon. In these cases, seizure onset typically occurs during the first few months of life, with episodes of epileptic hemifacial spasms and abnormal eye movements. Patients often present with drug-resistant epilepsy and often require resection for the best chance of seizure freedom.

OBSERVATIONS

The authors present the case of a 19-month-old male with intractable epileptic hemifacial spasms and a dorsally exophytic right brainstem and middle cerebellar peduncle hamartoma, following 2 prior subtotal resections. The authors recommended a third suboccipital craniotomy with intraoperative electrocorticography, which revealed interictal spiking from an intralesional depth electrode. Near-total resection led to durable seizure freedom.

LESSONS

Although posterior fossa lesions are rarely associated with epileptiform activity, this case demonstrates that pediatric patients with epileptic hemifacial spasms associated with a posterior fossa lesion may respond favorably to resection. Furthermore, this case demonstrates that intralesional electrocorticography can detect epileptic activity in posterior fossa lesions, which may predict postoperative seizure outcomes. https://thejns.org/doi/10.3171/CASE2452.

摘要

背景

后颅窝病变引起的局灶性癫痫是一种罕见现象。在这些病例中,癫痫发作通常在生命的最初几个月出现,伴有癫痫性半面痉挛发作和异常眼动。患者常表现为难治性癫痫,通常需要进行切除术以获得最佳的无癫痫发作机会。

观察结果

作者报告了一例19个月大的男性患者,患有顽固性癫痫性半面痉挛,存在一个向背侧外生性生长的右脑桥和小脑中脚错构瘤,此前已进行过2次次全切除术。作者建议进行第三次枕下开颅手术并术中进行皮层脑电图监测,结果显示病变内深部电极在发作间期出现棘波。近全切除术后患者实现了持久的无癫痫发作。

经验教训

尽管后颅窝病变很少与癫痫样活动相关,但该病例表明,患有与后颅窝病变相关的癫痫性半面痉挛的儿科患者可能对切除术反应良好。此外,该病例表明病变内皮层脑电图可检测后颅窝病变中的癫痫活动,这可能预测术后癫痫发作结果。https://thejns.org/doi/10.3171/CASE2452

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4ad/11301596/3da7a12d017b/CASE2452_figure_1.jpg

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