PML and Severe Immune Deficiency

PML is an abbreviation for progressive multifocal leukoencephalopathy, a severe opportunistic viral infection of the brain. PML is called an opportunistic infection because it takes advantage of a weakness in its patients — a seriously compromised immune system.

Named for its first identified victim, John Cunningham, the JC virus is a latent organism that exists in 80 to 90 percent of adults. It becomes dangerous when a person’s immune system is weakened. At that point, the virus awakens from its latent state and travels to the brain where it attacks the myelin, the protective substance covering the nerve sheath.

The myelin, which is destroyed by the JC virus, helps nerves transmit their messages through the body. When the myelin is destroyed, the nerves can no longer transmit messages to the muscles and other body parts. This is when the symptoms of PML become apparent.

Symptoms of PML

The first symptom of PML is usually clumsiness. Other symptoms follow, determined by which portions of the brain are being destroyed. These can include:

  • Difficulty moving the arms and legs, often on one side of the body at first
  • Headaches
  • Weakness
  • Decreased vision in half of the visual field
  • Aphasia (problems with language: speaking, understanding speech, reading and writing)
  • Slow, distorted speech
  • Problems in feeling parts of the body
  • Seizures
  • Compromised mental state
  • Personality changes

Who Gets PML?

Victims of PML are patients with severely compromised immune systems. The disease is an immunodeficiency disorder. People who suffer from this rare disease include the following types of patients whose immune systems are weakened:

Sometimes MS patients, who are in remission, think their MS symptoms have returned, when in fact they are experiencing the beginning of PML.

PML is usually fatal within one to nine months, with the average survival time being about six months, during which the patient steadily declines. Symptoms may occur slowly in the beginning, but appear more rapidly as time passes. Those who survive often are left with severe neurological damage.

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