ICD-10-CM Code A81.2

Progressive multifocal leukoencephalopathy

Version 2020 Billable Code

Valid for Submission

A81.2 is a billable code used to specify a medical diagnosis of progressive multifocal leukoencephalopathy. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code A81.2 might also be used to specify conditions or terms like central nervous system demyelinating disease associated with aids, cerebral degeneration due to progressive multifocal leukoencephalopathy, dementia due to infectious disease, dementia with progressive multifocal leukoencephalopathy, demyelinating disease of central nervous system co-occurrent with human immunodeficiency virus infection, disease due to jc polyomavirus, etc

Short Description:Progressive multifocal leukoencephalopathy
Long Description:Progressive multifocal leukoencephalopathy

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code A81.2:

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Multifocal leukoencephalopathy NOS

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code A81.2 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Central nervous system demyelinating disease associated with AIDS
  • Cerebral degeneration due to progressive multifocal leukoencephalopathy
  • Dementia due to infectious disease
  • Dementia with progressive multifocal leukoencephalopathy
  • Demyelinating disease of central nervous system co-occurrent with human immunodeficiency virus infection
  • Disease due to JC polyomavirus
  • Disease due to JC polyomavirus
  • Disease due to JC polyomavirus
  • Encephalopathy associated with AIDS
  • Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation
  • Progressive multifocal leukoencephalopathy
  • Progressive multifocal leukoencephalopathy
  • Progressive multifocal leukoencephalopathy
  • Progressive multifocal leukoencephalopathy associated with AIDS
  • Progressive multifocal leukoencephalopathy co-occurrent with human immunodeficiency virus infection
  • White matter disorder caused by infection
  • White matter disorder caused by infection
  • White matter disorder caused by infection

Clinical Information

  • LEUKOENCEPHALOPATHY PROGRESSIVE MULTIFOCAL-. an opportunistic viral infection of the central nervous system associated with conditions that impair cell mediated immunity e.g. acquired immunodeficiency syndrome and other immunologic deficiency syndromes; hematologic neoplasms; immunosuppression; and collagen diseases. the causative organism is jc polyomavirus jc virus which primarily affects oligodendrocytes resulting in multiple areas of demyelination. clinical manifestations include dementia; ataxia; visual disturbances; and other focal neurologic deficits generally progressing to a vegetative state within 6 months. from joynt clinical neurology 1996 ch26 pp36 7

Convert A81.2 to ICD-9

  • 046.3 - Prog multifoc leukoencep (Approximate Flag)

Code Classification

  • Certain infectious and parasitic diseases (A00–B99)
    • Viral and prion infections of the central nervous system (A80-A89)
      • Atypical virus infections of central nervous system (A81)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

Information for Patients

Degenerative Nerve Diseases

Degenerative nerve diseases affect many of your body's activities, such as balance, movement, talking, breathing, and heart function. Many of these diseases are genetic. Sometimes the cause is a medical condition such as alcoholism, a tumor, or a stroke. Other causes may include toxins, chemicals, and viruses. Sometimes the cause is not known.

Degenerative nerve diseases include

  • Alzheimer's disease
  • Amyotrophic lateral sclerosis
  • Friedreich's ataxia
  • Huntington's disease
  • Lewy body disease
  • Parkinson's disease
  • Spinal muscular atrophy

Degenerative nerve diseases can be serious or life-threatening. It depends on the type. Most of them have no cure. Treatments may help improve symptoms, relieve pain, and increase mobility.

[Learn More]


Encephalitis is an inflammation of the brain. Usually the cause is a viral infection, but bacteria can also cause it. It can be mild or severe. Most cases are mild. You may have flu-like symptoms. With a mild case, you may just need rest, plenty of fluids, and a pain reliever.

Severe cases need immediate treatment. Symptoms of severe cases include

  • Severe headache
  • Sudden fever
  • Drowsiness
  • Vomiting
  • Confusion
  • Seizures

In babies, additional symptoms may include constant crying, poor feeding, body stiffness, and bulging in the soft spots of the skull.

Severe cases may require a stay in the hospital. Treatments include oral and intravenous (IV) medicines to reduce inflammation and treat infection. Patients with breathing difficulties may need artificial respiration. Some people may need physical, speech, and occupational therapy once the illness is under control.

NIH: National Institute of Neurological Disorders and Stroke

[Learn More]

Viral Infections

Viruses are very tiny germs. They are made of genetic material inside of a protein coating. Viruses cause familiar infectious diseases such as the common cold, flu and warts. They also cause severe illnesses such as HIV/AIDS, smallpox, and Ebola.

Viruses are like hijackers. They invade living, normal cells and use those cells to multiply and produce other viruses like themselves. This can kill, damage, or change the cells and make you sick. Different viruses attack certain cells in your body such as your liver, respiratory system, or blood.

When you get a virus, you may not always get sick from it. Your immune system may be able to fight it off.

For most viral infections, treatments can only help with symptoms while you wait for your immune system to fight off the virus. Antibiotics do not work for viral infections. There are antiviral medicines to treat some viral infections. Vaccines can help prevent you from getting many viral diseases.

[Learn More]