[Focal presentation of progressive multifocal leukoencephalopathy: first manifestation of human immunodeficiency

L Martínez1, J Cuesta, S Santos

  • 1Servicios de Neurologia, Hospital Clínico Universitario Lozano Blesa, Zaragoza. lulicazgz@hotmail.com

Abstract

Insights

Diagnosing progressive multifocal leukoencephalopathy (PML) in immunocompromised patients can be challenging. Invasive methods like brain biopsy, alongside PCR testing, are crucial for differentiating PML from tumors and guiding treatment with HAART.

Area of Science:

  • Neuroimmunology
  • Infectious Diseases
  • Neuropathology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a demyelinating CNS disorder caused by JC virus reactivation in immunocompromised individuals.
  • JC virus remains latent in most adults, with reactivation linked to impaired cellular immunity from diseases or therapies.
  • Understanding PML pathogenesis is crucial for diagnosing and managing opportunistic infections in vulnerable populations.

Observation:

  • A 35-year-old man with HIV presented with facial palsy and hand weakness.
  • Cerebral MRI revealed a solitary lesion, posing a diagnostic challenge between PML and glial tumor.
  • Initial JC virus PCR in cerebrospinal fluid was negative, necessitating further investigation.

Findings:

  • Stereotactic brain biopsy confirmed PML with a positive JC virus PCR.
  • The patient's CD4/CD8 ratio indicated significant cellular immune deficiency.
  • Despite initial negative CSF PCR, biopsy provided definitive diagnosis.

Implications:

  • Invasive diagnostic methods, such as brain biopsy, are vital for accurate PML diagnosis and prognosis, especially when non-invasive tests are inconclusive.
  • Highly active antiretroviral therapy (HAART) shows promise in managing PML, potentially leading to longer survival and improved patient outcomes.
  • This case highlights the importance of a multidisciplinary approach in diagnosing and treating opportunistic neurological infections in HIV patients.

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