Takahashi Sunao, Ono Daisuke, Shintaku Hiroshi, Oyama Jun, Nishida Yoichiro, Ishikawa Kinya, Yokota Takanori
Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Japan.
Division of Surgical Pathology, Tokyo Medical and Dental University Hospital, Japan.
Intern Med. 2025 May 1;64(9):1412-1418. doi: 10.2169/internalmedicine.4217-24. Epub 2024 Sep 27.
We present a 76-year-old man with cryptogenic new-onset refractory status epilepticus (C-NORSE) with an initial abnormal signal in the nucleus accumbens and a remarkable hyperintense signal on T1-weighted magnetic resonance imaging in the bilateral basal ganglia (BG). His status epilepticus did not respond to most anti-epileptic therapies or immunotherapies, and he died of sepsis. An autopsy revealed severe neuronal loss and hypertrophic astrocytes in the BG and limbic system, with no signs of inflammation or malignancy. This case suggests that lesions in the BG may reflect secondary degeneration and predict poor outcomes in C-NORSE.
我们报告了一名76岁男性,患有隐源性新发难治性癫痫持续状态(C-NORSE),伏隔核最初出现异常信号,双侧基底神经节(BG)在T1加权磁共振成像上有明显的高信号。他的癫痫持续状态对大多数抗癫痫治疗或免疫治疗均无反应,最终死于败血症。尸检显示BG和边缘系统存在严重的神经元丢失和肥大星形胶质细胞,无炎症或恶性肿瘤迹象。该病例表明,BG中的病变可能反映继发性变性,并预示C-NORSE的预后不良。