Subacute sclerosing panencephalitis: an update.
Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis occurring after infection with measles virus. The prevalence of the disease varies depending on uptake of measles vaccination, with the virus disproportionally affecting regions with low vaccination rates. The physiopathology of the disease is not fully understood; however, there is evidence that it involves factors that favour humoral over cellular immune response against the virus. As a result, the virus is able to infect the neurons and to survive in a latent form for years. The clinical manifestations occur, on average, 6 years after measles virus infection. The onset of SSPE is insidious, and psychiatric manifestations are prominent. Subsequently, myoclonic seizures usually lead to a final stage of akinetic mutism. The diagnosis is clinical, supported by periodic complexes on electroencephalography, brain imaging suggestive of demyelination, and immunological evidence of measles infection. Management of the disease includes seizure control and avoidance of secondary complications associated with the progressive disability. Trials of treatment with interferon, ribavirin, and isoprinosine using different methodologies have reported beneficial results. However, the disease shows relentless progression; only 5% of individuals with SSPE undergo spontaneous remission, with the remaining 95% dying within 5 years of diagnosis.
Gutierrez
Jose
J
Department of Neurology, Miller School of Medicine, University of Miami, Miami, FL 33136, USA. drjosegc@hotmail.com
Issacson
Richard S
RS
Koppel
Barbara S
BS
D000914:Antibodies, Viral / Q000302:isolation & purification; D000998:Antiviral Agents / Q000627:therapeutic use*; D017209:Apoptosis; D001921:Brain / Q000473:pathology* / Q000503:physiopathology / Q000821:virology*; D003711:Demyelinating Diseases / Q000821:virology; D018450:Disease Progression; D004359:Drug Therapy, Combination; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000821:virology; D005260:Female; D005911:Gliosis / Q000821:virology; D006801:Humans; D007542:Inosine Pranobex / Q000627:therapeutic use; D016898:Interferon-alpha / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008457:Measles / Q000150:complications*; D008458:Measles Vaccine / Q000008:administration & dosage; D008459:Measles virus / Q000276:immunology / Q000302:isolation & purification*; D012254:Ribavirin / Q000627:therapeutic use; D012720:Severity of Illness Index; D012737:Sex Factors; D013344:Subacute Sclerosing Panencephalitis / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000517:prevention & control / Q000821:virology; D013997:Time Factors; D014771:Virion / Q000187:drug effects
D000914:Antibodies, Viral; D000998:Antiviral Agents; D016898:Interferon-alpha; D008458:Measles Vaccine; D012254:Ribavirin; D007542:Inosine Pranobex