ID

Progressive Multifocal Leukoencephalopathy

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Progressive Multifocal Leukoencephalopathy, PML

  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
  • Pathophysiology
  1. JC Virus infection (Polyomavirus JC) is common organism present without consequence in most patients
  2. Suppressed Immunity allows for Encephalitis
  • Signs
  1. Cognitive Decline
  2. Cranial Nerve Palsy
  3. Aphasia
  4. Ataxia
  5. Weakness
  6. Sensory loss
  7. Seizure
  1. Single or multiple white matter lesions
  2. Minimal to no enhancement, edema, or mass effect
  • Diagnosis
  1. JC Virus PCR detection from CSF
  2. Brain Biopsy (rarely done)
  • Management
  1. Reverse immune deficiency
  • Prognosis
  1. Mortality: 30-50% over months following onset
  2. Morbidity: Debilitating residual cognitive deficits are common in those who survive