Erturk Ozdem, Karslıgil Beyhan, Cokar Ozlem, Yapici Zuhal, Demirbilek Veysi, Gurses Candan, Yalcinkaya Cengiz, Gokyigit Aysen, Direskeneli Guher Saruhan, Yentur Sibel, Onal Emel, Yilmaz Gulden, Dervent Aysin
Department of Neurology, Cerrahpasa Faculty of Medicine, Istanbul University, 34098 Fatih, Istanbul, Turkey.
Childs Nerv Syst. 2011 Dec;27(12):2041-4. doi: 10.1007/s00381-011-1603-x. Epub 2011 Oct 15.
The typical clinical presentation of subacute sclerosing panencephalitis (SSPE) includes behavioral and intellectual changes followed by myoclonia. However, there are a considerable number of SSPE cases which present with distinct clinical features that can lead to a diagnostic difficulty. In this report, we summarize the clinical features of patients with SSPE who have uncommon presentations or features of the disease or coexisting medical conditions which may lead to diagnostic difficulties.
We studied 173 patients, all under the age of 17. Patients were included in the study group according to following criteria: onset of the disease before age 2 years, seizures occurring before the onset of myoclonia and/or behavioral symptoms, extrapyramidal or cerebellar signs and ocular manifestations as initial presenting symptoms, fulminant course including coma or death within 6 months. Additionally, patients with onset of SSPE at the setting of a known neurological disorder are defined as another group in the study.
Out of 173 patients with SSPE who were followed in two neurology centers, 31 (17.9%) met our criteria.
We found a relative high frequency of these clinical features. Our findings suggest that clinicians should be aware of this clinical characteristics and rule out the disease in cases were other common causes have been excluded, especially in countries with insufficient measles immunization.
亚急性硬化性全脑炎（SSPE）的典型临床表现包括行为和智力改变，随后出现肌阵挛。然而，有相当数量的SSPE病例具有独特的临床特征，可能导致诊断困难。在本报告中，我们总结了具有不常见表现、疾病特征或可能导致诊断困难的并存疾病的SSPE患者的临床特征。
我们研究了173名年龄均在17岁以下的患者。根据以下标准将患者纳入研究组：发病年龄在2岁之前，癫痫发作先于肌阵挛和/或行为症状出现，锥体外系或小脑体征以及眼部表现为初始症状，病程急骤，包括在6个月内出现昏迷或死亡。此外，在已知神经系统疾病背景下发病的SSPE患者被定义为研究中的另一组。
在两个神经科中心随访的173例SSPE患者中，31例（17.9%）符合我们的标准。
我们发现这些临床特征的出现频率相对较高。我们的研究结果表明，临床医生应了解这些临床特征，并在排除其他常见病因的情况下排除该疾病，尤其是在麻疹免疫接种不足的国家。
