Adult-onset hypothalamic hamartoma: origin of epilepsy?

IF 1.2 Q4 CLINICAL NEUROLOGY Acta Epileptologica Pub Date : 2023-04-28 DOI:10.1186/s42494-023-00120-9
Wenjie Han, Che Jiang, Zijuan Qi, Wei Xiang, Jian Lin, Youtian Zhou, Zhensheng Li, Bingmei Deng
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Abstract

Background: Hypothalamic hamartoma (HH) is a congenital non-progressive lesion of hypothalamus during fetal development. Mass-like lesions in different anatomical locations often develop a variously disabling course presenting with cognitive decline, psychiatric symptoms, as well as multiple seizure types. As a rare disease, HH is relatively common in infants and children, but it is extremely rare in adults.

Case presentation: We reported a case of adult-onset hypothalamic hamartoma, and summarized and analyzed relevant reports and studies of HH worldwide. The patient had clinical manifestations characterized by multiple seizure forms. After stereotactic radiofrequency thermocoagulation and drug treatment, the condition was effectively controlled. The patient was followed up till October 2022, with no recurrence of seizures.

Conclusions: Epilepsy caused by HH can resemble that of temporal lobe seizures, as HH forms a complex epileptogenic network with other regions of the brain through anatomical and functional connections. Early treatment of HH can provide better control of the symptoms of epilepsy, and patients with longer disease courses may have more complications.

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成人发病的下丘脑错构瘤:癫痫的起源?
背景:下丘脑错构瘤(HH)是胎儿发育期间下丘脑的一种先天性非进行性病变。不同解剖位置的肿块样病变通常发展为各种致残过程,表现为认知能力下降、精神症状以及多种癫痫发作类型。HH是一种罕见的疾病,在婴儿和儿童中比较常见,但在成人中极为罕见。病例介绍:我们报道了1例成人起病的下丘脑错构瘤,并对国内外有关HH的报道和研究进行了总结和分析。患者临床表现为多种发作形式。经立体定向射频热凝及药物治疗,病情得到有效控制。随访至2022年10月,无癫痫复发。结论:HH引起的癫痫可能类似于颞叶癫痫发作,HH通过解剖学和功能连接与大脑其他区域形成复杂的致痫网络。早期治疗HH可以更好地控制癫痫症状,病程较长的患者可能有更多的并发症。
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来源期刊
Acta Epileptologica
Acta Epileptologica Medicine-Neurology (clinical)
CiteScore
2.00
自引率
0.00%
发文量
38
审稿时长
20 weeks
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