Program Operations Manual System (POMS)
TN 30 (08-20)
COMPASSIONATE ALLOWANCES INFORMATION
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PERRY
SYNDROME |
ALTERNATE NAMES
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Parkinsonism with alveolar hypoventilation and mental depression
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DESCRIPTION
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Perry Syndrome is a rare inherited brain disease with gradual loss of neurons that regulate movement,
emotion, and breathing.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING
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Diagnostic testing: The diagnosis of Perry syndrome is made by molecular genetic testing for DCTN1,the
only gene known to be associated with Perry syndrome; and sleep studies to document
abnormally slow breathing (hypoventilation)/hypoxia.
Physical
findings: Four major features characterize Perry syndrome movement abnormalities:
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Signs of parkinsonism include:
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Unusually slow movements (bradykinesia);
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Psychiatric changes include:
Significant, unexplained weight loss affects many individuals early in the disease.
Hypoventilation occurs in the later stages of the disease, and can result in a life-threatening
lack of oxygen and respiratory failure.
ICD-9: 294.11
ICD-10: F03.91
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PROGRESSION
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Perry syndrome commonly presents around age 48, with the average survival of 5 years
after symptoms first appear. Death is common from respiratory failure or pneumonia;
suicide is another cause.
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TREATMENT
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There is no cure for Perry syndrome. Treatment involves the management of symptoms,
including medications for movement disorders and psychiatric symptoms; ventilatory
support; and nutritional supplements.
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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 the impairment
from the claimant’s medical source(s) documenting progressive physical, neurological
findings of Perry syndrome; and
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Suggested Listings for
Evaluation: |
DETERMINATION |
LISTING
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REMARKS
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Meets |
11.06
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11.17
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12.02
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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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