TN 6 (12-11)
DI 23022.620 Malignant Multiple Sclerosis
COMPASSIONATE ALLOWANCE INFORMATION
MALIGNANT MULTIPLE SCLEROSIS
Malignant MS; Marburg Variant Multiple Sclerosis, Marburg Variant MS; Fulminant Multiple Sclerosis; Aggressive MS; Advanced MS
Malignant Multiple Sclerosis (Malignant MS) is an aggressive and rare form of MS. It is characterized by rapidly progressive inflammation and destruction of myelin (protective covering surrounding the nerves) and increased formation of lesions and plaque in the brain and spine. The loss of myelin affects the brain’s ability to transmit electrochemical impulses between the nerve cells of the brain, and the spinal cord, resulting in diminished or loss of neurological functioning. People with this form of MS experience weakness in their extremities, difficulties with coordination and balance, spasticity, and paresthesias (abnormal sensory feelings of numbness and prickling sensations). Speech impediments, tremors, dizziness, hearing loss, changes in vision, bowel and bladder difficulties, falls, and cognitive impairments are other frequent complaints. As the disease progresses lesions develop in the areas of the brain responsible for information processing, resulting in cognitive impairments such as difficulties with concentration, attention, memory, language, and judgment. People with malignant MS can have damage to regions of the brain responsible for behavior and emotions resulting in psychotic disorders such as manic depression and paranoia.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
There is no single test to detect malignant MS. It may be difficult to distinguish between a diagnosis of malignant MS and acute disseminated encephalomyelitis (ADEM) because of the timing of the occurrence of plaques in the brain tissue. A neurological exam is performed to assess symptoms and to rule out other possible disorders. Analysis of the cerebrospinal fluid is also helpful for the diagnosis of malignant MS. Neuro-imaging technologies, such as magnetic resonance imaging (MRI), diffusion-tensor magnetic resonance imaging (DT-MRI), and computerized brain tomography are used to detect central nervous system lesions, myelin loss, white matter abnormalities, and other physical changes in the brain.
ICD 9: 340 AL, 340 AZ, 341.8 and 341.9
ONSET AND PROGRESSION
People with malignant MS experience a rapid decline in functioning. They require assistance with ambulation within five years from symptom onset due to the loss of the ability of the nerve cell (neurons) to transmit impulses to muscles that control motor functioning. Assistance with ADL’s is required.
There is currently no cure for malignant MS. Treatment generally consists of immunomodulatory therapy and the management of symptoms. Physical and occupational therapies can help the person perform daily activities such as handwriting, buttoning, and using eating utensils. Ambulatory aides such as canes, walkers and wheel chairs are prescribed for gait and ataxia
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation:
Clinical records from the treating physician, neurologist, or psychiatrist documenting the progression of neurological and cognitive decline.
A report completed by a family member or caregiver is needed to document the individual’s ability to perform daily activities independently.
Neuroimaging studies, such as MRI and other tests used to detect central nervous system lesions, myelin loss, white matter abnormalities, and other physical changes in the brain.
Suggested Listings for Evaluation:
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* 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.