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Progressive Multifocal Leukoencephalopathy

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Progressive Multifocal Leukoencephalopathy, PML

  • 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
  • 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
  • Imaging
  • MRI Brain
  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