TN 38 (09-20)

DI 23022.490 Progressive Multifocal Leukoencephalopathy

 

COMPASSIONATE ALLOWANCE INFORMATION

PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY

ALTERNATE NAMES

PML

DESCRIPTION

Progressive Multifocal Leukoencephalopathy (PML) is a fatal disease of the brain that can occur in individuals with immunocompromising conditions such as AIDS, transplant patients, individuals undergoing chronic corticosteroid or immunosuppressive therapy, and individuals with cancer such as Hodgkin’s disease, lymphoma and sarcoidosis. The disease is caused by the John Cunningham polyomavirus (also known as polyomavirus JC or JC virus) which results in the loss of white matter (myelin) in multiple areas of the brain. Without the protection of myelin, nerve signals can not travel successfully from the brain to the rest of the body

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

Diagnostic testing:

  • A positive diagnosis of PML can be made with a brain biopsy and by observing the progressionof the disease;

  • An MRI scan can determine if there are any white matter lesions anda spinal tap can detect the presence of the JC virus;

  • Lab testing may include: cytologyexam of the urine; and

  • EEG and CT scans are also diagnostic of the disease.

Physical findings: The effects associated with PML vary from patient to patient; specific symptoms are related to the location and amount of damage in the brain, and evolve over the course of several days to several weeks. The most common symptoms include:

  • Clumsiness;

  • Progressive weakness;

  • Impaired vision and speech; and

  • Personality changes.

ICD-9: 046.3

ICD-10: A81.2

PROGRESSION

The progression of deficits leads to life-threatening disability and death over a period of weeks or months. The mortality rates for those with HIV-PML have fallen dramatically from approximately 90 percent to between 30 and 50 percent. For non-AIDS individuals with PML, the prognosis remains grim; the disease usually lasts for months and 80 percent die within the first 6 months, although spontaneous improvement has been reported. Those who survive PML can be left with severe neurological disabilities.

TREATMENT

The best available therapy is reversal of the immune-deficient state. This can sometimes be accomplished by alteration of chemotherapy or immunosuppression, even at the expense of losing non-vital transplanted organs). In the case of HIV-associated PML, immediately beginning anti-retroviral therapy will benefit most individuals.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:
  • Clinical examination that includes a description of findings;

  • Urine analysis;

  • CT scan;

  • EEG;

  • MRI; and

  • HIV testing.

DETERMINATION

LISTING

REMARKS

Meets

14.11

114.11

Equals

* 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.


To Link to this section - Use this URL:
http://policy.ssa.gov/poms.nsf/lnx/0423022490
DI 23022.490 - Progressive Multifocal Leukoencephalopathy - 09/16/2020
Batch run: 09/16/2020
Rev:09/16/2020