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Progressive Multifocal Leukoencephalopathy
Aka: Progressive Multifocal Leukoencephalopathy, PML- Risk Factors: Immunosuppression
- AIDS Defining Illness (CD4 <200/mm3)
- Chemotherapy for Lymphoma
- Immunosuppressants post-organ transplant
- Multiple Sclerosis medications
- Rheumatoid Arthritis medications
- Systemic Lupus Erythematosus medications
- Pathophysiology
- JC Virus infection (Polyomavirus JC) is common organism present without consequence in most patients
- Suppressed Immunity allows for Encephalitis
- Signs
- Cognitive Decline
- Cranial Nerve Palsy
- Aphasia
- Ataxia
- Weakness
- Sensory loss
- Seizure
- Imaging: MRI Brain
- Single or multiple white matter lesions
- Minimal to no enhancement, edema, or mass effect
- Diagnosis
- JC Virus PCR detection from CSF
- Brain Biopsy (rarely done)
- Management
- Reverse immune deficiency
- Prognosis
- Mortality: 30-50% over months following onset
- Morbidity: Debilitating residual cognitive deficits are common in those who survive
Leukoencephalopathy, Progressive Multifocal (C0023524) |
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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 |
Russian | LEIKOENTSEFALOPATIIA PROGRESSIRUIUSHCHAIA MNOGOOCHAGOVAIA, ЛЕЙКОЭНЦЕФАЛОПАТИЯ ПРОГРЕССИРУЮЩАЯ МНОГООЧАГОВАЯ |
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 |
Sources |
Derived from the NIH UMLS (Unified Medical Language System) |
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