Progressive Multifocal Leukoencephalopathy

Progressive multifocal leukoencephalopathy  is an infrequent disorder of the nervous system that primarily affects individuals with suppressed immune systems (including, allograft recipients such as kidney transplant patients; patients with cancers such as leukemia or lymphoma; and nearly 10% of patients with acquired immune deficiency syndrome — AIDS. The disorder, which is caused by a common human polyomavirus, JC virus, is characterized by demyelination or destruction of the myelin sheath that covers nerve cells. The myelin sheath is the fatty covering - which acts as an insulator - on nerve fibers in the brain. Symptoms of Progressive Multifocal Leukoencephalopathy include mental deterioration, vision loss, speech disturbances, ataxia (inability to coordinate movements), paralysis, and, ultimately, coma reflecting the multifocal distribution of brain lesions. In rare cases, seizures may occur.

Is there any treatment for Progressive Multifocal Leukoencephalopath?

There is no cure for Progressive Multifocal Leukoencephalopathy, nor is there currently an effective treatment for the disorder. Treatment is symptomatic and supportive.

What is the prognosis of Progressive Multifocal Leukoencephalopath?

The course of Progressive Multifocal Leukoencephalopathy is relentlessly progressive. Death usually occurs between 1 and 4 months after onset, but there have been a number of reported cases with survival for months to years.

What research is being done for Progressive Multifocal Leukoencephalopath?

The NINDS supports research on demyelinating disorders of the nervous system such as Progressive Multifocal Leukoencephalopathy aimed at increasing scientific understanding of these disorders and finding ways to prevent, treat, and cure the disease utilizing state of the art molecular diagnostic techniques.

Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892