Ishida Hiroshi, Hattori Hideji, Takaura Natsuko, Yoshida Toshiko, Tanaka Katsuji, Otani Sanae, Matsuoka Osamu, Takahashi Yukitoshi, Yamano Tsunekazu
Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka.
No To Hattatsu. 2006 Nov;38(6):443-7.
We experienced an 8-year-old-boy with non-herpetic acute limbic encephalitis (NHALE), who developed headache, convulsion, consciousness disturbance, and ataxia following cold like symptoms. Disturbance of short term memory and a change of character were recognized. Myoclonic seizures and generalized tonic clonic convulsions developed, that responded to antiepileptic agents. Although other symptoms resolved spontaneously, short term memory disturbance persisted. Brain MRI demonstrated the lesion involving the bilateral claustrum and right hippocampus. Three months later, the lesion in the claustrum disappeared, but the hippocampus still showed slight hyperintensity on FLAIR image of MRI. Autoantibodies against glutamine receptor were detected in the cerebrospinal fluid and plasma, which suggested the involvement of immunologic disturbances in this disease. In NHALE, many cases have been reported in adults but not in children, and the further attentions should be paid to childhood-onset NHALE.
我们接诊了一名8岁男孩,患有非疱疹性急性边缘叶脑炎(NHALE),他在出现类似感冒症状后出现头痛、惊厥、意识障碍和共济失调。发现存在短期记忆障碍和性格改变。出现了肌阵挛发作和全身性强直阵挛惊厥,对抗癫痫药物有反应。尽管其他症状自行缓解,但短期记忆障碍持续存在。脑部MRI显示病变累及双侧屏状核和右侧海马体。三个月后,屏状核的病变消失,但海马体在MRI的FLAIR图像上仍显示轻度高信号。在脑脊液和血浆中检测到抗谷氨酰胺受体自身抗体,这表明免疫紊乱参与了该疾病。在NHALE中,成人病例已有很多报道,但儿童病例较少,应进一步关注儿童期发病的NHALE。