TN 6 (12-11)

DI 23022.660 The ALS Parkinsonism Dementia Complex

COMPASSIONATE ALLOWANCE INFORMATION
 

THE ALS/PARKINSONISM DEMENTIA COMPLEX

ALTERNATE NAMES

The 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

DESCRIPTION

The 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.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

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.

ICD-9: 331.0

ONSET AND PROGRESSION

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.

TREATMENT

There is currently no cure for ALS/PDC. Treatment involves the management of symptoms.

Management of symptoms may include:

  • Cholinesterase inhibitors for the treatment of neuropsychiatric symptoms;

  • Levodopa-carbidopa combinations to treat movement disorders;

  • Physical therapy for cardiovascular and skeletal muscle strengthening; flexibility, as well as for gait training;

  • Occupational therapy helps to maintain skills and promote function and independence; and,

  • 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.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

  • Clinical information from the treating primary physician, neurologist, or psychiatrist documenting a progressive physical, neurological finding of PD and ALS. Dementia is also critical and required for disability evaluation of ALS/PDC.

  • Activities of daily living report or a similar report completed by relative or caregiver

Suggested Listings for Evaluation:

DETERMINATION

 LISTING 

REMARKS

Meets Listing

11.06

11.10

12.02 A and B

12.02 A and C

 

Medical Equals

  

* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.


To Link to this section - Use this URL:
http://policy.ssa.gov/poms.nsf/lnx/0423022660
DI 23022.660 - The ALS Parkinsonism Dementia Complex - 12/09/2011
Batch run: 12/09/2011
Rev:12/09/2011