Bilateral Rasmussen encephalitis: Postmortem documentation in a five-year-old

Bilateral Rasmussen encephalitis: Postmortem documentation in a five-year-old
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DOI:
10.1046/j.1528-1157.2003.36602.x
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发表时间:
2003-01-01
期刊:
影响因子:
5.6
通讯作者:
Andermann, T
Andermann, T
中科院分区:
医学1区
文献类型:
--
作者:
Tobias, SM;Robitaille, Y;Andermann, T

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本文回顾1例2岁发病的双侧累及拉斯穆森脑炎病例。拉斯穆森脑炎是一种罕见的进行性疾病,可引起顽固性癫痫发作、认知能力下降和脑部炎症变化。神经受累是单侧的特征。该病例在发病后5个月内怀疑双侧受累,并通过双侧额叶活检得到证实。在这个病例中,疾病的严重程度和进展是显著的,尽管多次尝试治疗干预,但最终导致患者在5岁时死亡。尸检结果证实诊断为双侧累及拉斯穆森脑炎。
A case of Rasmussen encephalitis with bilateral involvement and onset at age 2 years is reviewed. Rasmussen encephalitis is a rare progressive disease that causes intractable seizures, cognitive decline, and inflammatory changes in the brain. The neurologic involvement is characteristically unilateral. Bilateral involvement in this case was suspected within 5 months of presentation and was confirmed by bilateral frontal lobe biopsies. The severity and progression of the disease in this case was remarkable and resulted in the patient's death by age 5 years, despite numerous attempted therapeutic interventions. Autopsy findings confirmed the diagnosis of Rasmussen encephalitis with bilateral involvement.