SUBACUTE SCLEROSING PANENCEPHALITIS

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. There is a higher incidence among males than females (male/female: 3/1). 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, including uncontrollable involuntary jerking movements of the head, trunk or limbs called myoclonic jerks. Seizures may also occur. Some people may become blind. In advanced stages of the disease, individuals may lose the ability to walk, as their muscles stiffen or spasm. There is progressive deterioration to a comatose state, and then to a persistent vegetative state. Death is usually the result of fever, heart failure, or the brain's inability to continue controlling the autonomic nervous system.

EEG showing widespread cortical dysfunction.

Lab tests showing eosinophilic inclusion bodies in the cytoplasm and nuclei of neurons and glial cells. Lab tests showing marked elevation in CSF immunoglobulin. Lab tests showing high Rubeola Ig G Antibody Titres in serum, elevated measles antibody titers in the CSF, brain CT or MRI scan may show cortical atrophy and white matter lesions.

ICD-9: 046.2 Subacute Sclerosing Panencephalitis

Most youngsters 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 think that the infection of the brain occurs soon after the primary bout with measles and progresses slowly. Persons with this disease frequently die 1 to 2 years after diagnosis, but some may survive for longer periods. The condition is always deadly.

Currently, there is no cure for SSPE. Anticonvulsant and antispasmodic drugs are used to control the seizures and myoclonic jerks.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation: Clinical examination that describes diagnostic features of the impairment and laboratory studies are needed to confirm the diagnosis. Laboratory tests showing results of genetic testing, enzyme study tests, blood and urine tests, and MRI or CT scan.

Suggested Listings for Evaluation:

LISTING

111.02

111.17