Program Operations Manual System (POMS)
TN 54 (09-22)
DI 23022.505 Subacute Sclerosing Panencephalitis
COMPASSIONATE ALLOWANCES INFORMATION
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SUBACUTE SCLEROSING PANENCEPHALITIS
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ALTERNATE NAMES
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SSPE; Dawson Disease; Dawson’s Encephalitis; Panencephalitis Subacute Sclerosing;
Subacute Inclusion Body Encephalitis
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DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING
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Diagnostic testing: Testing includes:
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Electroencephalogram (EEG);
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Physical findings: Physical symptoms include:
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Involuntary jerks and spasms;
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Impairment or loss of ambulation; and
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Coma or vegetative state.
ICD-9: 046.2
ICD-10: A81.1
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PROGRESSION
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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.
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TREATMENT
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There is no cure for SSPE. Anticonvulsant and antispasmodic drugs may control seizures
and myoclonic jerks.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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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.
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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111.02
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111.17
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Equals
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111.21
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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