progressive multifocal leukoencephalopathy

E133122

Progressive multifocal leukoencephalopathy is a rare, often fatal demyelinating brain disease caused by reactivation of the JC virus in immunocompromised individuals, leading to rapidly progressive neurological decline.

All labels observed (1)

Label Occurrences
progressive multifocal leukoencephalopathy canonical 1

How this entity was disambiguated

Statements (58)

Predicate Object
instanceOf demyelinating disease
neurological disorder
opportunistic infection
viral disease
white matter disease
affects brain white matter
brainstem
cerebellum
cerebral hemispheres
oligodendrocytes
cause aphasia
ataxia
cognitive impairment
demyelination of central nervous system white matter
focal neurological deficits
motor weakness
progressive neurological decline
seizures
visual disturbances
diagnosedBy PCR for JC virus
brain MRI
brain biopsy
detection of JC virus DNA in cerebrospinal fluid
epidemiology occurs predominantly in immunocompromised individuals
rare disease
hasCause HIV/AIDS
surface form: AIDS

HIV/AIDS
surface form: HIV infection

chronic corticosteroid use
hematologic malignancy
immunosuppressive therapy
infection with JC polyomavirus
natalizumab therapy
organ transplantation
other monoclonal antibody therapies
reactivation of JC virus
rituximab therapy
severe immunosuppression
hasCourse multifocal lesions in the brain
rapidly progressive
hasHistopathology demyelination with enlarged oligodendrocyte nuclei
presence of viral inclusions in oligodendrocytes
hasImagingFinding lesions without mass effect
non-enhancing white matter lesions on MRI
hasPathogen JC virus
John Cunningham virus
hasPrognosis high mortality rate
often fatal
poor prognosis without immune reconstitution
hasSymptom dysarthria
gait disturbance
hemianopia
hemiparesis
memory loss
personality changes
subacute onset of neurological deficits
treatedBy antiretroviral therapy in HIV-positive patients
discontinuation of causative immunosuppressive drugs
restoration of immune function

How these facts were elicited

Referenced by (1)

Full triples — surface form annotated when it differs from this entity's canonical label.

Tysabri blackBoxWarningFor progressive multifocal leukoencephalopathy