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Adult-onset subacute sclerosing panencephalitis: clinico-pathological findings in 2 new cases
A Ortega-Aznar1, F J Romero-Vidal, J Castellví
1Department of Pathology (Neuropathology), Vail d'Hebrón University Hospital, Barcelona, Spain. aortega@cs.vhebron.es
Abstract:
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.
Insights
Subacute sclerosing panencephalitis (SSPE) is a rare, fatal measles complication. This study details two adult female cases with atypical presentations, including one during pregnancy, highlighting disease progression and neurodegenerative links.
Area of Science:
- Neurology
- Infectious Diseases
- Pathology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological complication of measles infection, typically affecting children and adolescents.
- Adult-onset SSPE is exceptionally uncommon, with limited documented cases in medical literature.
Observation:
- Presents two new cases of adult women with SSPE, one experiencing the disease during pregnancy.
- Both cases exhibited highly atypical clinical features, disease presentation, and progression.
- Pregnancy appeared to negatively influence the disease course, consistent with previous reports.
Findings:
- The patients displayed a prolonged biphasic clinical course, including extended periods of remission lasting several years.
- Neuropathological examination revealed inflammatory processes alongside neurodegenerative features, such as neurofibrillary tangle formation.
- These histological findings suggest a potential link between SSPE and other tauopathies.
Implications:
- Highlights the possibility of atypical SSPE presentations in adults, necessitating broader diagnostic considerations.
- Underscores the adverse impact of pregnancy on SSPE progression.
- Suggests a potential shared pathogenic mechanism between SSPE and tauopathies, warranting further investigation into neurodegenerative pathways.