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Published on: September 12, 2016
[Subacute sclerosing panencephalitis (SSPE)]
1Klinik für Kinder- und Jugendmedizin, Städtisches Klinikum Kemperhof, Koblenz. thomas.hoppen@dgn.de
Abstract:
Subacute sclerosing panencephalitis is a rare progressive neurological disorder of childhood and early adolescence caused by persistent measles virus. The diagnosis is based upon characteristic clinical manifestations, periodic EEG discharges, raised antibody titre against measles/SSPE in the plasma and cerebrospinal fluid and increase of gamma-globulins in the cerebrospinal fluid. Histopathological examination shows neuronal loss, astrogliosis, demyelination, infiltration of inflammatory cells, and intranuclear inclusions in neurons, oligodendrocytes and astrocytes. In most cases nucleocapsids are detected by electron microscopy. Although treatment is still undetermined, combination of intrathecal high-dose interferon-alpha and intravenous ribavirin administered at an early stage of SSPE seems to be effective.
Insights
Subacute sclerosing panencephalitis (SSPE) is a rare, progressive measles virus infection affecting children. Early diagnosis and treatment with interferon-alpha and ribavirin show potential effectiveness.
Area of Science:
- Neurology
- Virology
- Pathology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder.
- It is caused by persistent measles virus infection in children and adolescents.
Observation:
- Diagnosis relies on clinical signs, EEG, elevated measles antibodies, and increased cerebrospinal fluid gamma-globulins.
- Histopathology reveals neuronal loss, inflammation, demyelination, and intranuclear inclusions.
- Electron microscopy often detects viral nucleocapsids.
Findings:
- Combination therapy of intrathecal interferon-alpha and intravenous ribavirin may be effective.
- Early intervention is crucial for potential treatment success.
Implications:
- This research highlights a potential therapeutic strategy for SSPE.
- Further studies are warranted to confirm the efficacy and optimize treatment protocols for this devastating neurological condition.
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