PROGRESSIVE SUPRANUCLEAR PALSY
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ALTERNATE
NAMES |
Richardson-Steele-Olszewski syndrome; Steele-Richardson-Olszewski syndrome; Richardson
Syndrome; Nuchal Dystonia Dementia Syndrome; Progressive Supranuclear Ophthalmoplegia;
Supranuclear Palsy-Progressive; PSP
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DESCRIPTION
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Progressive supranuclear palsy (PSP) is a rare progressive brain disorder that affects movement, gait, eye movements,
speech, and cognition. The signs and symptoms are very different in each person, but
may include personality changes, difficulty swallowing, jaw or face spasms, vision
problems, loss of balance, frequent falls, problems with walking, poor coordination,
and an unsteady, lurching gait. PSP primarily involves damage to the brain stem (the
area of the brain that controls breathing, heartbeat, blood pressure, eye movement,
and other autonomic functions) and the frontal lobes of the brain (the area of the
brain that is associated with reasoning, planning, speech, movement, emotions, and
problem solving). The most obvious sign of PSP is an inability to focus the eyes properly,
which occurs because of lesions in the area of the brain that coordinates eye movements.
People with PSP often show alterations of mood and behavior, including depression
and apathy as well as progressive mild dementia. PSP usually occurs in people over
60 years of age, and men are affected more often than women are. Additional features
of the disorder includes a general loss of interest and enthusiasm (apathy) an increasing
need for assistance with personal care and other activities of daily living.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING |
Diagnostic testing: The diagnosis of PSP involves:
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Assessment of balance and gait;
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Evaluation of ocular movements with attention on the ability to move the eyes up and
down;
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Hearing assessment and evaluation of voluntary muscle movement; and
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PSP is often misdiagnosed because some of its symptoms are similar to Parkinson’s
disease, Alzheimer disease and Creutzfeldt-Jakob disease. The key to properly diagnosing
PSP is identifying early gait instability and difficulty moving the eyes. Neurological
examinations may show uncontrollable eye movement, mild dementia, and visual difficulties
with the inability to look up or down without bending the neck.
Physical
findings: Signs and symptoms of PSP include:
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An unsteady, lurching gait.
ICD-9: 333.0
ICD-10: G23.1
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PROGRESSION |
PSP is slowly progressive with a decline in brain dysfunction over time. Initially,
the motor and eye movement abnormalities are the major impairments, but as the disease
progresses, cognitive and behavioral manifestations may become significant. In advance
cases, the person with PSP may become wheel chair dependent or bedridden. Individuals
with PSP are also at risk of pneumonia, choking from swallowing problems and injuries
from falling.
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TREATMENT
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There is no effective cure for this disorder. Drugs such as L-dopa, Amantadine, Amitriptyline,
and Desipramine are prescribed to control symptoms. Speech therapy can provide instruction
in swallowing techniques. Communication devices such as pointing boards or computer-based
systems can be used when speech becomes ineffective. Walking devices can help to minimize
falls.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination describing symptoms, signs, laboratory findings,
and response to treatment;
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Full neurological examination with emphasis on motor function and coordination, gait
and balance, eye movements and gaze, and cognitive function;
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Speech and language examination describing speech difficulties, low voice volume,
slow speech, and poor ability to speak clearly (enunciate); and
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Assessment of swallowing abilities.
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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 |
11.17
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Equals |
11.06
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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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