Subacute sclerosing leukoencephalitis (SSPE) is a rare and chronic progressive neurological disorder that affects primarily children and young adults. It is characterized by the inflammation, degeneration, and subsequent scarring (sclerosis) of the myelin sheath, which is the protective covering surrounding nerve fibers in the central nervous system.
This condition is believed to be caused by an aberrant measles virus infection that occurs several years after the initial measles infection. The underlying mechanism involves the persistence of the virus in the brain, leading to slow and progressive damage of the white matter. The exact reason behind the reactivation of the dormant measles virus is not well understood; however, it is thought to be linked to an impaired immune response.
Early symptoms of SSPE may include personality changes, behavioral abnormalities, decline in school performance, and subtle motor abnormalities. As the disease progresses, individuals may develop myoclonic jerks (involuntary muscle twitches), seizures, loss of coordination, and eventually dementia or coma. The prognosis for SSPE is generally poor, with the disease being fatal in most cases.
Diagnosis of SSPE involves neurological examination, a thorough medical history, evaluation of symptoms, and various tests including electroencephalogram (EEG) to assess brain function, cerebrospinal fluid (CSF) analysis, and brain imaging studies.
Management of SSPE primarily focuses on controlling symptoms, reducing seizures with anticonvulsant medications, and providing supportive care. There is currently no cure for SSPE, and treatment mainly aims at improving the quality of life and prolonging survival. In some cases, an experimental antiviral drug may be used to slow down the disease progression. Vaccination against measles is considered the most effective preventive measure for this condition.
