Program Operations Manual System (POMS)
TN 40 (10-20)
DI 23022.490 Progressive Multifocal Leukoencephalopathy
COMPASSIONATE ALLOWANCES INFORMATION
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PROGRESSIVE
MULTIFOCAL LEUKOENCEPHALOPATHY |
ALTERNATE NAMES
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PML
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DESCRIPTION
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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
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing:
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A positive diagnosis of PML can be made with a brain biopsy and by observing the progressionof
the disease;
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An MRI scan can determine if there are any white matter lesions anda spinal tap can
detect the presence of the JC virus;
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Lab testing may include: cytologyexam of the urine; and
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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:
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Impaired vision and speech; and
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ICD-9: 046.3
ICD-10: A81.2
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PROGRESSION
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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.
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TREATMENT
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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.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for
Evaluation:
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Clinical examination that includes a description of findings;
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DETERMINATION
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LISTING
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REMARKS
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Meets |
14.11
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114.11
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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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