Program Operations Manual System (POMS)
TN 30 (08-20)
COMPASSIONATE ALLOWANCES INFORMATION
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FATAL FAMILIAL INSOMNIA
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ALTERNATE NAMES
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Insomnia Fatal Familial; FFI
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DESCRIPTION
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Fatal Familial Insomnia (FFI) is a rare, genetic sleep disorder caused by prion mutation of the PRNP gene. These
mutations lead to degeneration in the thalamus, the part of the brain responsible
for sensory perception and regulation of motor function. FFI is characterized by by
subacute onset of insomnia showing as a reduced overall sleep time, autonomic dysfunction,
and motor disturbances.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING
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Diagnostic testing:
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Electroencephalography (EEG);
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Magnetic resonance imaging (MRI) of the brain;
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Evidence of mutations in the PRNP gene.
Physical findings:
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High pulse and blood pressure;
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Decline in coordination and motor ability.
ICD-9: 046.72
ICD-10:
A81.83
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PROGRESSION
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FFI is a progressive sleep disorder that usually begins in adulthood and leads to
death within 6 to 32 months.
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TREATMENT
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There is no cure for FFI. Treatment is symptomatic and palliative.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of this impairment;
and
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Appropriate laboratory testing, including genetic testing showing mutations in the
PRNP gene.
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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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11.17
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Equals
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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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