Ortega-Aznar A, Romero-Vidal F J, Castellví J, Ferrer J M, Codina A
Department of Pathology (Neuropathology), Vail d'Hebrón University Hospital, Barcelona, Spain.
Clin Neuropathol. 2003 May-Jun;22(3):110-8.
Subacute sclerosing panencephalitis (SSPE), an uncommon disease usually affecting children and adolescents, is caused by persistent measles infection that progresses to chronic infection with fatal outcome. The debut of this disease in adults is rare, with a small number of cases in the medical literature. This article presents the clinical, radiologic and post-mortem neuropathologic findings in 2 new cases of women with SSPE (1 of them during pregnancy), which showed very atypical clinical characteristics, presentation and evolution. The influence of pregnancy on the course of the disease was unfavorable, in keeping with earlier reports. Our patients showed a very prolonged biphasal clinical course, with a period of disease-free remission that lasted several years. Histological study disclosed features of inflammatory disease associated with others of a neurodegenerative nature, such as the formation of neurofibrillary tangles, which would relate SSPE with other tauopathies.
亚急性硬化性全脑炎（SSPE）是一种罕见疾病，通常影响儿童和青少年，由持续性麻疹感染引起，这种感染会进展为慢性感染并导致致命后果。该疾病在成人中首次出现的情况罕见，医学文献中仅有少数病例报道。本文介绍了2例患有SSPE的女性（其中1例在孕期）的临床、放射学及尸检神经病理学发现，这些病例呈现出非常不典型的临床特征、表现及病程演变。与早期报道一致，妊娠对疾病进程产生了不利影响。我们的患者表现出非常漫长的双相临床病程，有一段持续数年的无病缓解期。组织学研究揭示了与神经退行性病变相关的炎症性疾病特征，如神经原纤维缠结的形成，这将SSPE与其他tau蛋白病联系起来。
