Subacute sclerosing panencephalitis
| Subacute sclerosing panencephalitis | |
|---|---|
| Classification and external resources | |
| Specialty | infectious disease |
| ICD-10 | A81.1 |
| ICD-9-CM | 046.2 |
| OMIM | 260470 |
| DiseasesDB | 12597 |
| MedlinePlus | 001419 |
| MeSH | C02.182.500.300.600 |
Subacute sclerosing panencephalitis (SSPE) also known as Dawson Disease, Dawson encephalitis, and measles encephalitis is a rare and chronic form of progressive brain inflammation caused by a persistent infection with measles virus (which can be a result of a mutation of the virus itself). The condition primarily affects children and young adults. It has been estimated that about 1 in 10,000 people infected with measles will eventually develop SSPE. However, a 2016 study estimated that the rate for babies who contracted measles was as high as 1 in 609. No cure for SSPE exists and the condition is often fatal. However, SSPE can be managed by medication if treatment is started at an early stage. Much of the work on SSPE has been performed by the National Institute of Neurological Disorders and Stroke (NINDS).
SSPE should not be confused with acute disseminated encephalomyelitis which has a similar etiology but very different timing and course.
Characterized by a history of primary measles infection usually before the age of 2 years, followed by several asymptomatic years (6–15 on average), and then gradual, progressive psychoneurological deterioration, consisting of personality change, seizures, myoclonus, ataxia, photosensitivity, ocular abnormalities, spasticity, and coma.
A large number of nucleocapsids are produced in the neurons and the glial cells. In these cells the viral genes that encode envelope proteins have restricted expression. As a result, infectious particles like the M protein are not produced, and the virus is able to survive persistently without evoking an immune response. Eventually the infection will lead to SSPE.
The progression of symptoms begins with stage 1—in this stage the individual's behaviour becomes more abnormal and erratic: they can be irritable and personality alterations can occur, often accompanied by dementia. As the nervous system begins to lose control of movement, the person develops myoclonic spasms/jerks (these being involuntary motions and spasms in extremities). As the disease progresses towards stage 2, the intensity of the spasms and the mental deterioration increases. The spasms can grow to such an extent that loss of the ability to walk can be a common sign. Also, the person will suffer speech impairment and increasingly deteriorated comprehension coupled with difficulty swallowing. The final, advanced stages of SSPE include the steady decline in body function with increased intensity of the stage 2 symptoms/signs and also blindness. At the end of the final stages the person is likely to be mute, in a vegetative state, and/or comatose.
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