Progressive multifocal leukoencephalopathy

This code represents progressive multifocal leukoencephalopathy (PML), a rare and often fatal demyelinating disease of the central nervous system caused by the JC virus. It primarily affects individuals with compromised immune systems, leading to widespread destruction of myelin in the brain.

Use this code for patients diagnosed with PML, typically confirmed by neuroimaging (e.g., MRI showing characteristic white matter lesions) and detection of JC virus DNA in cerebrospinal fluid or brain biopsy. It is commonly seen in patients with HIV/AIDS, hematologic malignancies, or those on immunosuppressive therapies.

• Ensure documentation clearly states the diagnosis of progressive multifocal leukoencephalopathy.
• Always code any underlying immunocompromising condition (e.g., HIV, malignancy) as a primary or secondary diagnosis.
• Distinguish from other demyelinating diseases; PML is specifically linked to JC virus reactivation in immunocompromised hosts.