Wölfle J, Schmidt J, Kreft B, Albert F, Haverkamp F
Abteilung Allgemeine Pädiatrie, Friedrich Wilhelms-Universität Bonn.
Klin Padiatr. 1997 May-Jun;209(3):111-5. doi: 10.1055/s-2008-1043938.
An 8 year old girl presented with progressive change of personality and spastic ataxia since 4 weeks. A year before she had developed focal grand-mal-seizures; at this time laboratory and radiologic findings were normal. The EEG on admission demonstrated marked changes with partially focal, partially generalized hypersynchronic activity, but no SSPE-typical Radermecker-complexes. There were no cells in the cerebrospinal fluid (CSF), a slightly increased level of protein and a normal glucose. Isoelectric focusing showed predominantly measles-specific oligoclonal IgG bands in the CSF. In the magnetic resonance tomography multiple focal white matter lesions in the basal ganglia as well as in cortical and occipitoparietal regions could be seen. At the age of two the girl had suffered from measles, the child didn't receive any vaccination. The combination of history, CSF-, MRI-results and EEG lead to the diagnosis of subacute sclerosing panencephalitis (SSPE). After 3 months the clinical and radiological abnormalities had markedly increased. On the background of this history SSPE should be considered as differential diagnosis in patients with changes of personality.
一名8岁女孩自4周前起出现性格逐渐改变和痉挛性共济失调。1年前她出现局灶性大发作癫痫;此时实验室和影像学检查结果均正常。入院时脑电图显示有明显变化,部分为局灶性、部分为全身性超同步活动,但无亚急性硬化性全脑炎(SSPE)典型的拉德梅克复合体。脑脊液(CSF)中无细胞,蛋白水平略有升高,葡萄糖正常。等电聚焦显示CSF中主要为麻疹特异性寡克隆IgG带。在磁共振断层扫描中,可见基底神经节以及皮质和枕顶叶区域有多个局灶性白质病变。该女孩两岁时患过麻疹,未接种过任何疫苗。结合病史、CSF、MRI结果和脑电图,诊断为亚急性硬化性全脑炎(SSPE)。3个月后,临床和放射学异常明显加重。鉴于此病史,对于性格改变的患者,应将SSPE视为鉴别诊断之一。