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Published on: October 19, 2014
[Progressive multifocal leukoencephalopathy: a complication in chronic B-cell lymphatic leukaemia]
Caroline R van Kernebeek1,2, Esra Ünal3, Cees G Schaar3
1Gelre ziekenhuizen, Apeldoorn. Afd. Neurologie.
Background:
Progressive multifocal leukoencephalopathy (PML) is a rare infectious cause of sub-acute neurological symptoms, and occurs predominantly in immunocompromised patients. PML is caused by reactivation of the JC virus.
Case Description:
A 79-year-old man with a history of chronic B-cell lymphatic leukaemia (B-CLL) presented at our hospital with a neurological deficit of the left side of his body. He was initially diagnosed with a right-hemisphere stroke. Two months later he returned with progressive paresis and on an MRI of the brain we saw an increase in abnormalities of the white matter. On suspicion of PML we conducted PCR for JC virus on cerebrospinal fluid (CSF), which was negative. Histopathological investigations of a brain biopsy confirmed the diagnosis of PML, four months after he first presented.
Conclusion:
PML is a rare cause of sub-acute neurological symptoms. PML can be difficult to diagnose as a PCR of CSF for JC virus in the early stages of PML can give a false negative result. If PML is suspected, histological investigation of a brain biopsy is necessary.
Insights
Progressive multifocal leukoencephalopathy (PML), a rare neurological disease caused by JC virus reactivation, can be challenging to diagnose. Early PCR testing of cerebrospinal fluid may yield false negatives, necessitating brain biopsy for confirmation.
Area of Science:
- Neurology
- Infectious Diseases
- Oncology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, opportunistic infection.
- It is caused by the reactivation of the JC virus, primarily affecting immunocompromised individuals.
- PML presents as sub-acute neurological deficits.
Observation:
- A 79-year-old male with chronic B-cell lymphatic leukemia (B-CLL) presented with left-sided neurological deficits.
- Initial diagnosis was stroke, but progressive symptoms and brain MRI abnormalities suggested PML.
- JC virus PCR on cerebrospinal fluid was negative.
Findings:
- Histopathological examination of a brain biopsy confirmed PML.
- This case highlights the diagnostic challenges of PML.
- JC virus PCR can produce false-negative results in early stages.
Implications:
- PML diagnosis requires a high index of suspicion in immunocompromised patients with neurological symptoms.
- Brain biopsy remains crucial for definitive PML diagnosis when PCR is inconclusive.
- Timely diagnosis is essential for appropriate patient management.
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