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[Progressive multifocal leukoencephalopathy in a patient with prolonged survival: a case report]
Aquiles Antonio-Valdiviezo1, Sergio Gómez-Rivera, Jorge Martínez-Torres
1Unidad de Medicina Familiar 11, Instituto Mexicano del Seguro Social, Tapachula, Chiapas.
Background:
The progressive multifocal leukoencephalopathy (PML) is a disease histological anatomy characterized by the presence of multifocal areas of demyelization spread out over the central nervous system.The PML is caused by the polyomavirus, a papovavirus.
Clinical Case:
A female patient with 27 years aged began with malaise, musculosqueletal pain, paresthetic, hyperthermia and diaphoresis; 72 hours later she presented quadriplegic with neurological damage that progressed to stupor and generalized clonic-tonic seizures. The spinal fluid showed no infection signs. The simple and contrasted computed tomography (CT) was normal. The magnetic resonance imaging (MRI) from encephalon reported lesion in white substance in hemispheres, callosal corpus, cerebellum and tallus. Angioresonance showed an increased number of hyperdense areas in the white substance. Final pathological diagnosis was: progressive multifocal leukoencephalopathy. There was no confirmation of immunological disease after the exhaustive studies realized. The patient's survival was two years after the diagnosis was made.
Insights
Progressive multifocal leukoencephalopathy (PML), a rare brain disease caused by a papovavirus, presents with demyelination. This case highlights a rapid, severe presentation in a young adult, emphasizing the need for advanced imaging for diagnosis.
Area of Science:
- Neurology
- Neurovirology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system.
- PML is caused by the human polyomavirus, a type of papovavirus.
Observation:
- A 27-year-old female presented with acute neurological decline including malaise, pain, paresthesia, hyperthermia, and diaphoresis.
- Neurological status rapidly worsened to quadriplegia, stupor, and seizures within 72 hours.
- Initial diagnostics, including spinal fluid analysis and CT scans, were unremarkable, necessitating advanced neuroimaging.
Findings:
- Magnetic Resonance Imaging (MRI) revealed white matter lesions in the brain hemispheres, corpus callosum, cerebellum, and brainstem.
- Angiography-MRI showed increased hyperdense areas within the white matter.
- Pathological diagnosis confirmed progressive multifocal leukoencephalopathy, with no underlying immunological disease identified.
Implications:
- This case underscores the importance of MRI in diagnosing PML, especially when initial tests are inconclusive.
- The rapid and severe presentation in a young adult highlights the varied clinical spectrum of PML.
- Accurate diagnosis is crucial for patient management and understanding disease progression.
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