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[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
Introduction:
Progressive multifocal leukoencephalopathy (PML) is a subacute demyelinating disorder of central nervous system caused by an opportunistic papovavirus infection of oligodendroglial cells, the JC virus. This virus remains latent in the human host. Their antibodies are detectable in 70% of healthy adult subjects. It is reactivated by different diseases and medical therapies impairing cellular immunity.
Clinical Case:
A 35 year-old man, smoker, allergic to penicillin and derivatives, with a history of endovenous drug abuse, was admitted to Neurology Department due to a progressive left facial palsy and hand weakness. An alteration in the cellular immune ratio (CD4/CD8 0.07) and a positive human immunodeficiency virus serology were demonstrated. Highly active antiretroviral therapy (HAART) was started. A unique wide hyperintense subcortical right temporoparietal lobe lesion was shown by a cerebral magnetic resonance imaging (MRI), suggesting a differential diagnosis between PLM and low grade glial tumor. Cerebrospinal fluid study was normal and the polymerase chain reaction (PCR) was negative for JC virus. A steroataxic cerebral biopsy was made in order to establish a correct diagnosis due to patient's age and the good response to treatment. The pathology study showed changes suggestive of PLM and a positive-PCR test for JC virus.
Discussion:
We report this case to suggest that inva- sive diagnosis methods may be useful to differentiate between PLM and low grade glial tumors and to establish a correct prognosis. HAART is becoming a good treatment option because it seems to make long term survival longer, as is the case of this patient who is practically asymptomatic after more than 2 years of evolution.
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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