Program Operations Manual System (POMS)
TN 34 (08-20)
DI 23022.281 Progressive Bulbar Palsy
COMPASSIONATE ALLOWANCES INFORMATION
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PROGRESSIVE
BULBAR PALSY |
ALTERNATE NAMES
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Progressive Bulbar Atrophy; Bulbar Paralysis; Bulbar Palsy; Fazio-Londe Syndrome;
Fazio-Londe Disease; Infantile Progressive Bulbar Palsy
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DESCRIPTION
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Progressive Bulbar Palsy (PBP) is a motor neuron disease that involves the brain stem—the bulb-shaped region containing
lower motor neurons needed for swallowing, speaking, chewing, and other functions.
Symptoms include pharyngeal muscle weakness (involved with swallowing), weak jaw
and facial muscles, progressive loss of speech, and tongue muscle atrophy. Limb weakness
with both lower and upper motor neuron signs is almost always evident but less prominent.
Individuals are at increased risk of choking and aspiration pneumonia, which is caused
by the passage of liquids and food through the vocal folds and into the lower airways
and lungs. Affected persons may have unusual outbursts of laughing or crying (called
emotional lability). Stroke and myasthenia gravis may have certain symptoms that are similar to those
of progressive bulbar palsy and must be ruled out prior to diagnosing this disorder. The
exact cause of PBP is unknown.
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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 PBP is based on:
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History and neurological examination;
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Electrophysiological and neuroimaging testing to rule out other impairments;
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Needle electromyogram (EMG);
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Nerve conduction study; and
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Analysis of the cerebral spinal fluid to rule out other causes of symptoms.
Physical findings:
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Spastic dysarthria (difficulties with articulation, stammering or stuttering);
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Pseudobulbar reflexes; and
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Pseudobulbar affect (uncontrollable crying or laughing).
ICD-9:
335.22
ICD-10: G12.22
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PROGRESSION
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The prognosis for PBP is poor. The symptoms of PBP slowly worsen with onset beginning
between the ages of 50-70 years. Most people with PBP die from respiratory failure,
usually within ten years from the onset of symptoms.
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TREATMENT
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Treatment of PBP is symptoms specific and supportive. Medications such as riluzole
are prescribed to prolong survival. Other medications may be prescribed to help reduce
fatigue, ease muscle cramps, control spasticity and reduce excess saliva and phlegm.
A multidisciplinary team of health care professionals can design an individualized
plan for medical and physical therapy and provide special equipment aimed at keeping
the individual as mobile and comfortable as possible. As swallowing, tongue control,
and pharyngeal muscles weaken, a speech therapist and nutritionist may be consulted.
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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 disorder;
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Full neurological examination with emphasis on motor function and coordination, gait
and balance, eye movements and gaze, and cognitive function; and
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Brain imaging may provide supporting evidence.
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Suggested Listings for
Evaluation: |
DETERMINATION
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LISTING
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REMARKS
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Meets |
11.17
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11.22
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111.17
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111.22
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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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