Kunika N, Yasaki S, Oshima J, Ino M, Saito N
Third Department of Internal Medicine, St. Marianna University School of Medicine, Japan.
Rinsho Shinkeigaku. 1995 Nov;35(11):1214-20.
A 20-year-old man who had developed involuntary movement of his left hand and memorial disturbance visited our hospital in December, 1991. On admission, myoclonus, dementia and speech disturbance were recognized. He was diagnosed as subacute sclerosing panencephalitis (SSPE) based on a high titer of serum anti-measles antibody (1/256), serum anti-measles-IgG antibody (> 1/4,800) and typical EEG fiding of periodic synchronus discharge (PSD). Inosine pranobex was administrated orally (4,800mg per day). Serial cranial magnetic resonance imagings (MRI) were taken since January, 1992 to June, 1994. No abnormal finding was demonstrated until April 16, 1992 in MRI, but 123I-IMP SPECT detected decreased accumulation in parietal to occipital lobes on early image in February 5, 1992. Marked high signal area on T2 weighted image in right temporal lobe and parieto-occipital lobe were firstly demonstrated in June 22, 1992 on MRI. These high signal lesions alternated the areas and locations, but the changes were not related to his clinical symptom. These findings may suggest ischemic changes after demyelination. His symptoms have been improving gradually since June, 1994. To our knowledge, 42 cases of adult-onset SSPE were reported so far (5 were in Japan). This case is the first report in the world on adult-onset SSPE serially observed with MRI and SPECT since early stage.
一名20岁男性，出现左手不自主运动和记忆障碍，于1991年12月就诊于我院。入院时，发现有肌阵挛、痴呆和言语障碍。根据高滴度血清抗麻疹抗体（1/256）、血清抗麻疹IgG抗体（>1/4800）以及典型的脑电图周期性同步放电（PSD）表现，他被诊断为亚急性硬化性全脑炎（SSPE）。口服给予异丙肌苷（每日4800mg）。自1992年1月至1994年6月进行了系列头颅磁共振成像（MRI）检查。直到1992年4月16日MRI均未发现异常，但1992年2月5日的早期图像上，123I-IMP单光子发射计算机断层显像（SPECT）检测到顶叶至枕叶放射性摄取减少。1992年6月22日MRI首次显示右侧颞叶和顶枕叶T2加权像上有明显高信号区。这些高信号病变在区域和位置上交替出现，但变化与他的临床症状无关。这些发现可能提示脱髓鞘后的缺血性改变。自1994年6月以来，他的症状逐渐改善。据我们所知，迄今为止共报道了42例成人起病的SSPE（其中5例在日本）。该病例是世界上首例对成人起病的SSPE从早期开始进行MRI和SPECT系列观察的报道。
