SSPE; Dawson Disease; Dawson’s Encephalitis; Panencephalitis Subacute Sclerosing; Subacute Inclusion Body Encephalitis

Subacute Sclerosing Panencephalitis (SSPE) is a progressive neurological disorder of children and young adults that affects the central nervous system (CNS). It is a slow, but persistent, viral infection caused by defective measles virus and is universally fatal. The rate of occurrence is much higher (3:1) among males than females.

The initial symptoms of SSPE are subtle and include mild mental deterioration (such as memory loss) and changes in behavior (such as irritability) followed by disturbances in motor function. Seizures may also occur. Some people may become blind. In advanced stages of the disease, individuals may lose the ability to walk.

Diagnostic testing: Testing includes:

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  Electroencephalogram (EEG);

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  Blood tests; and

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  Brain CT or MRI scans.

Physical findings: Physical symptoms include:

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  Involuntary jerks and spasms;

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  Seizures;

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  Vision loss;

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  Impairment or loss of ambulation; and

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  Coma or vegetative state.

ICD-9: 046.2

Most children with SSPE have a history of measles infection at an early age, usually younger than 2 years, followed by a latent period of 6 to 8 years before neurological symptoms begin. Despite the long interval between the measles infection and the onset of SSPE, researchers believe the initial infection of the brain occurs soon after the primary bout with measles and progresses slowly.

There is progressive deterioration to a comatose state, and then to a persistent vegetative state. The disease is always fatal, usually within 2 years of diagnosis. Death is usually the result of fever, heart failure, or the brain's inability to continue controlling the autonomic nervous system.

There is no cure for SSPE. Anticonvulsant and antispasmodic drugs may control seizures and myoclonic jerks.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical examination that describes diagnostic features of the impairment and laboratory studies are needed to confirm the diagnosis;EEG showing widespread cortical dysfunction;

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  Lab tests showing eosinophilic inclusion bodies in the cytoplasm and nuclei of neurons and glial cells;

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  Lab tests showing marked elevation in CSF immunoglobulin;

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  Lab tests showing high Rubeola Ig G Antibody Titres in serum, elevated measles antibody titers in the CSF; and

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  Brain CT or MRI scan showing cortical atrophy and white matter lesions.

REMARKS