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[Subacute sclerosing panencephalitis (SSPE)]
1Department of Neurology, National Children's Hospital.
Abstract:
Subacute sclerosing panencephalitis (SSPE) is one of the most important slow virus infection caused by measles virus in human. It have passed over 20 years since measles virus was found in the brain of SSPE patients. However, definite etiological mechanism is still unknown. In Japan, number of SSPE patients are gradually decreasing, but over 5 patients are reported every year. Recently, the age of onset of SSPE became higher, incubation period from measles to SSPE onset became to be more prolonged, and serum measles antibody titers became lower than before. From typical clinical findings, serum and CSF measles antibody titers and periodic complex high voltage slow waves with suppression burst, SSPE is suggested. It is not so difficult to make diagnosis. As the differential diagnosis, epilepsy, brain tumor, metabolic diseases, or psychological disorders are mentioned. Treatment for SSPE is still undetermined. At present, it is thought that combined treatment of oral inosinpranobex (isoprinosine) and intratechal or intraventricular interferon (IFN) administration is the most effective.
Insights
Subacute sclerosing panencephalitis (SSPE) is a rare slow measles virus infection. Diagnosis involves clinical signs and antibody titers, though treatment remains challenging.
Area of Science:
- Neurology
- Virology
- Infectious Diseases
Context:
- Subacute sclerosing panencephalitis (SSPE) is a severe neurological complication of measles virus infection.
- Despite decades of research, the exact etiological mechanisms of SSPE remain elusive.
- While SSPE incidence is declining in Japan, cases persist, with trends showing later onset and altered immune responses.
Purpose:
- To review the current understanding of Subacute Sclerosing Panencephalitis (SSPE), including its diagnosis and treatment.
- To highlight the changing epidemiological and clinical features of SSPE.
- To discuss diagnostic criteria and differential diagnoses for SSPE.
Summary:
- SSPE is a slow viral infection caused by the measles virus, characterized by specific clinical and laboratory findings.
- Diagnostic indicators include clinical presentation, elevated serum and cerebrospinal fluid (CSF) measles antibody titers, and characteristic EEG patterns.
- Differential diagnoses encompass epilepsy, brain tumors, metabolic disorders, and psychological conditions.
Impact:
- This review provides insights into the evolving nature of SSPE, aiding clinicians in diagnosis and management.
- Understanding these changes is crucial for effective patient care and public health strategies.
- The current most effective treatment approach involves a combination of oral inosinpranobex and intrathecal or intraventricular interferon administration.