Wilhelm-Gössling C, Weckbecker K, Brabant E G, Dengler R
Klinik für Neurologie mit klinischer Neurophysiologie, Abteilung Klinische Psychiatrie und Psychotherapie, Hochschule Hannover.
Dtsch Med Wochenschr. 1998 Mar 6;123(10):279-84. doi: 10.1055/s-2007-1023949.
A 58-year-old woman, known for 10 years to have Hashimoto's thyroiditis, was admitted from another hospital where, after an initial period of unconsciousness, she had developed progressive severe dementia, abnormal arousal and generalized myoclonia. Jakob-Creutzfeldt disease (JCD) was suspected.
The electroencephalogram (EEG) showed marked slowing of the basic activity and episodes of triphasic waves. The titres of thyroid antibodies (TPO 764 kU/l, TgAk 398 kU/l) and of the antinuclear antibodies (ANA 1:1280) were raised, as was the erythrocyte sedimentation rate (80/120 mm and the cerebrospinal fluid albumin concentration (1 g/l).
The history and findings suggested autoimmune encephalitis (AIE) and treatment with prednisolone, 2 mg/kg body weight daily, was initiated, achieving lasting improvement of arousal within two days. 6 weeks later the EEG merely showed mild alteration of basic activity. The thyroid antibody titres were now within normal limits and the signs of inflammation were regressing.
In case of rapidly progressive dementia autoimmune antibodies should be looked for in the differential diagnosis, because autoimmune disease may be the treatable cause.
一名58岁女性，患桥本甲状腺炎10年，从另一家医院转入我院。她起初昏迷一段时间，之后逐渐发展为严重痴呆、异常觉醒及全身性肌阵挛。怀疑患有克雅氏病（JCD）。
脑电图（EEG）显示基础活动明显减慢及三相波发作。甲状腺抗体（TPO 764 kU/l，TgAk 398 kU/l）及抗核抗体（ANA 1:1280）滴度升高，红细胞沉降率（80/120 mm）及脑脊液白蛋白浓度（1 g/l）也升高。
病史及检查结果提示自身免疫性脑炎（AIE），开始使用泼尼松龙治疗，每日2 mg/kg体重，两天内觉醒持续改善。6周后，脑电图仅显示基础活动轻度改变。此时甲状腺抗体滴度已在正常范围内，炎症迹象逐渐消退。
对于快速进展性痴呆病例，鉴别诊断时应查找自身免疫抗体，因为自身免疫性疾病可能是可治疗的病因。
