II. Risk Factors: Immunosuppression

  1. AIDS Defining Illness (CD4 <200/mm3)
  2. Chemotherapy for Lymphoma
  3. Immunosuppressants post-organ transplant
  4. Multiple Sclerosis medications
  5. Rheumatoid Arthritis medications
  6. Systemic Lupus Erythematosus medications

III. Pathophysiology

  1. JC Virus infection (Polyomavirus JC) is common organism present without consequence in most patients
  2. Suppressed Immunity allows for Encephalitis

IV. Signs

  1. Cognitive Decline
  2. Cranial Nerve Palsy
  3. Aphasia
  4. Ataxia
  5. Weakness
  6. Sensory loss
  7. Seizure

V. Imaging: MRI Brain

  1. Single or multiple white matter lesions
  2. Minimal to no enhancement, edema, or mass effect

VI. Diagnosis

  1. JC Virus PCR detection from CSF
  2. Brain Biopsy (rarely done)

VII. Management

  1. Reverse immune deficiency

VIII. Prognosis

  1. Mortality: 30-50% over months following onset
  2. Morbidity: Debilitating residual cognitive deficits are common in those who survive

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Related Studies

Ontology: Leukoencephalopathy, Progressive Multifocal (C0023524)

Definition (NCI) A progressive demyelination within the central nervous system associated with reactivation of a latent JC virus infection.
Definition (MSH) 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)
Definition (CSP) demyelination in various part of the central nervous system, usually secondary to neoplastic disease; JC virus has been recovered from brain tissue.
Concepts Disease or Syndrome (T047)
MSH D007968
ICD9 046.3
ICD10 A81.2
SnomedCT 22255007
English Leukoencephalopathies, Progressive Multifocal, Multifocal Leukoencephalopathies, Progressive, Multifocal Leukoencephalopathy, Progressive, Progressive Multifocal Leukoencephalopathies, Progressive Multifocal Leukoencephalopathy, PML - Prog multifo leukoenceph, PMLE - Prog multi leucoenceph, Prog mltifoc lucoencephalopath, ENCEPH JC POLYOMAVIRUS, JC POLYOMAVIRUS ENCEPH, LEUKOENCEPH PROGRESSIVE MULTIFOCAL, PROGRESSIVE MULTIFOCAL LEUKOENCEPH, progressive multifocal leukoencephalopathy (diagnosis), progressive multifocal leukoencephalopathy, Encephalitis, JC Polyomavirus, JC Polyomavirus Encephalitis, Encephalopathies, JC Polyomavirus, Encephalopathy, JC Polyomavirus, JC Polyomavirus Encephalopathy, Prog multifoc leukoencep, Multifocal leukoencephalopathy NOS, Leukoencephalopathy, Progressive Multifocal [Disease/Finding], multifocal leukoencephalopathy, pml, Progressive multifocal leukoencephalopathy, PML - Progressive multifocal leukoencephalopathy, PMLE - Progressive multifocal leucoencephalopathy, Progressive multifocal leucoencephalopathy, Multifocal leucoencephalopathy, Multifocal leukoencephalopathy, PML - Progressive multifocal leucoencephalopathy, PMLE - Progressive multifocal leukoencephalopathy, Progressive multifocal leukoencephalopathy (disorder), leukoencephalopathy; multifocal, multifocal; leukoencephalopathy, Multifocal leukoencephalopathy, NOS, Leukoencephalopathy, Progressive Multifocal, PML
Italian Leucoencefalopatia multifocale progressiva, Encefalopatia da poliomavirus JC, Encefalite da poliomavirus JC, Leucoencefalopatia progressiva multifocale
French Leucoencéphalopathie mutlifocale progressive, Leucoencéphalopathie progressive multifocale, Leuco-encéphalopathie multifocale progressive, Leucoencéphalopathie multifocale progressive, Encéphalite à polyomavirus JC, LEMP
Swedish Leukoencefalopati, progressiv multifokal
Japanese シンコウセイタソウセイハクシツノウショウ, 白質脳症-進行性多巣性, 進行性多巣性白質エンセファロパシー, 進行性多巣性白質脳症, 進行性多病巣性白質エンセファロパシー, 進行性多病巣性白質脳症, 進行性多病巣性脳症, 白質エンセファロパシー-進行性多巣性, 白質エンセファロパシー-進行性多病巣性, 白質脳症-進行性多病巣性
Czech leukoencefalopatie progresivní multifokální, Progresivní multifokální leukoencefalopatie
Finnish Progressiivinen multifokaalinen leukoenkefalopatia
German Progressive multifokale Leukenzephalopathie, progressive multifokale Leukoenzephalopathie, Leukoenzephalopathie, progressive multifokale, Enzephalitis, JC-Polyomavirus, Progressive multifokale Leukoenzephalopathie
Korean 진행성 다발성 백질뇌병증
Polish Leukoencefalopatia wieloogniskowa postępująca, Zwyrodnienie istoty białej postępujące wieloogniskowe, Choroba Astroma
Hungarian progressiv multifocalis leucoencephalopathy, multifocalis progresszív leukoencephalopathia
Norwegian Progressiv mulitfokal leukoencefalopati
Dutch leuko-encefalopathie; multifocaal, multifocaal; leuko-encefalopathie, progressieve multifocale leuko-encefalopathie, Encefalopathie, progressieve multifocale leuko-, Leuko-encefalopathie, progressieve multifocale, Multifocale leuko-encefalopathie, progressieve, Polyomavirusencefalitis, Progressieve multifocale leuko-encefalopathie
Spanish leucoencefalopatía multifocal progresiva (trastorno), leucoencefalopatía multifocal progresiva, leucoencefalopatía multifocal, Leucoencefalopatía multifocal progresiva, Encefalitis por JC Poliomavirus, Leucoencefalopatía Multifocal Progresiva, Leucoencefalopatía Progresiva Multifocal
Portuguese Leucoencefalopatia multifocal progressiva, Encefalite por Polyomavirus JC, Leucoencefalopatia Multifocal Progressiva