Adult-onset subacute sclerosing panencephalitis: clinico-pathological findings in 2 new cases.
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.
Ortega-Aznar
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A
Department of Pathology (Neuropathology), Vail d'Hebrón University Hospital, Barcelona, Spain. aortega@cs.vhebron.es
Romero-Vidal
F J
FJ
Castellví
J
J
Ferrer
J M
JM
Codina
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A
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology*; D001932:Brain Neoplasms / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008459:Measles virus / Q000276:immunology; D008590:Meningoencephalitis / Q000473:pathology; D016874:Neurofibrillary Tangles / Q000473:pathology; D011247:Pregnancy; D011859:Radiography; D013344:Subacute Sclerosing Panencephalitis / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000473:pathology*