亞急性硬化性汎腦炎：兩病例報告

Subacute Sclerosing Panencephalitis: Report of Two Cases

林美慧(Mei-Hwey Lin) ； 李慶雲(Chin-Yun Lee) ； 沈友仁(Yu-Zen Shen)

《Acta Paediatrica Sinica》 22卷4期 (1981/12) Pp. 212-218

摘要

臺大醫院於1980年發現兩例亞急性硬化性汎腦炎。第1例為8歲男孩，主要症狀為漸進性的肌無力，肌陣攣及智力減退，此例曾於1歲多時患麻疹，住院時發現血清中麻疹抗體及腦脊髓液中麻疹抗體較高。雖然其他於亞急性硬化性腦炎可見的特徵在此例並不完全出現，但臨床上進行性的運動障礙智力退化及肌陣攣的表現加上血清及腦脊髓液的麻疹補體結合抗體一次高到1:128及1:8極似亞急性硬化性汎腦炎。第2例為11歲男孩，主要症狀為異常行為，智力減退及肌陣攣，此例亦曾於2歲時感染麻疹。血清及腦脊髓液中的麻疹抗體昇高到1:256及1:8，腦脊髓液中的γ球蛋白增加，並有典型的腦波變化，雖無腦部活體檢視的組織學變化證實，但其他的表現已足以確定亞急性硬化性汎腦炎的診斷。因此症為罕見病例，故提出報告且綜合有關文獻加以討論。

並列摘要

Subacute sclerosing panencephalitis is a chronic inflammatory disease of central nervous system caused by measles virus. The clinical picture of mental deterioration associated with myoclonic seizures is always strongly suggestive of this disease. The diagnosis is confirmed by the presence of suppression bursts on electroencephalographic examination, and the strikingly elevated levels of measles antibodies in the serum and cerebrospinal fluid. Microscopic examination of brain biopsy, and viral isolation from the tissue provide a final diagnostic proof. This article presents two such cases, which were diagnosed at the National Taiwan University Hospital in 1980. Case 1 was an eight-year-old boy who suffered from weakness of lower extremities, myoclonus and progressive deterioration of speech and mentality. He got measles at the age of one. Elevated titer of measles complement fixation antibody in serum (1:128) and CSF (1:8) (but only in one occasion) was detected. Although other criteria were not all fulfilled in this case, the diagnosis of SSPE was most probable due to the typical clinical picture of mental deterioration and myoclonus. Case 2 was a eleven-year-old boy, who had measles at the age of 2. He had been manifesting from queer behavior, silly laughter, myoclonus and mental deterioration during the month prior to the admission. Complement fixation antibody in the serum was 1:256, while that in cerebrospinal fluid was 1:8 in one occasion and 1:16 in another. The electroencephalogram showed classic pictures of diffuse slow dysrhythmia with periodic burst-suppression complexes. Neurological conditions of both patients deteriorated in spite of symptomatic treatment. None of them accepted brain biopsy. The literature related to this disease was reviewed and discussed.

並列關鍵字

subacute sclerosing panencephalitis (SSPE) ； measles