ALS/PARKINSONISM
DEMENTIA COMPLEX
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ALTERNATE NAMES
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Amyotrophic Lateral Sclerosis/Parkinsonism Dementia Complex; ALS/PDC; ALS/PDC syndrome
of Guam; Kii ALS-PDC; Lytico-Bodig Motor Neuron Disease; Guam Disease; PDALS; Parkinsonism
Dementia-ALS complex
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DESCRIPTION
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Amyotrophic
Lateral Sclerosis/Parkinsonisim Dementia Complex (ALS/PDC) is a rare malignant form of amyotrophic lateral sclerosis (ALS). ALS/PDC is an endemic
neurodegenerative disorder, known to occur primarily on the island of Guam and the
Kii peninsula of Japan. It is characteristic of classical ALS, parkinsonism, and dementia.
The cause of this disorder is widespread neurofibrillary degeneration in the brain
and spinal cord. This type of damage results in bradykinesia (abnormal slowness of
physical movement), rigidity, tremor, forgetfulness, and dementia.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: There is no specific test or procedure to establish the diagnosis of ALS/PDC. ALS/PDC
diagnosis is based on history, neurological findings consistent with the diagnosis
of ALS/PDC.
Electrophysiological and neuroimaging testing is used to rule out other impairments
that may cause similar signs and symptoms. There are no definitive biomarkers that
clearly distinguish ALS/PDC from other degenerative neurological disorders.
Physical findings:
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Bradykinesia (abnormal slowness of physical movement);
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ICD-9: 331.0
ICD-10: F02
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PROGRESSION
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The average onset of the disease is in the 40s. As the disease progresses, people
experience increasing cognitive deficits, spasticity, muscle atrophy, and weakness.
These symptoms progressively worsen to a vegetative state with death occurring within
4 -6 years after diagnosis.
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TREATMENT
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There is currently no cure for ALS/PDC. Treatment involves the management of symptoms.
Management of symptoms may include:
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Cholinesterase inhibitors for the treatment of neuropsychiatric symptoms;
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Levodopa-carbidopa combinations to treat movement disorders;
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Physical therapy for cardiovascular and skeletal muscle strengthening; flexibility,
as well as for gait training;
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Occupational therapy helps to maintain skills and promote function and independence;
and,
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The primary bulbar muscles affected by this disorder include the pharynx, tongue,
and part of the larynx. Issues such as low voice volume, poor enunciation, poor bulbar
muscular strength, and swallowing difficulties are treated with speech therapy.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical information from the claimant’s medical source(s) documenting a progressive
physical, neurological finding of PD and ALS. Dementia is also critical and required
for disability evaluation of ALS/PDC; and
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Activities of daily living report or a similar report completed by relative or caregiver.
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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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11.06
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11.10
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11.22
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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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