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A "cortico-basal degeneration"-like syndrome as first sign of progressive multifocal leukoencephalopathy
1Department of Neurology, AZ, St-Jan, Brugge, Belgium.
Abstract:
We report an unusual case of probable progressive multifocal leukoencephalopathy (PML), who initially presented with a right-sided movement disorder, including upper limb dystonia, tremor, and dyspraxia, reminiscent of corticobasal degeneration. In the further course, the patient developed disorientation, confusion, and bradyphrenia. The appearance of white matter MRI lesions as well as a positive PCR test result for JC-virus in the cerebrospinal fluid finally led to the correct diagnosis.
Insights
We describe a rare case of probable progressive multifocal leukoencephalopathy (PML) that initially mimicked corticobasal degeneration due to movement abnormalities. JC virus detection in cerebrospinal fluid confirmed the diagnosis.
Area of Science:
- Neuroscience
- Neurology
- Infectious Diseases
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, demyelinating disease affecting the white matter of the brain.
- It is caused by the opportunistic JC virus (JCV) and typically occurs in immunocompromised individuals.
Observation:
- The patient presented with a right-sided movement disorder, including dystonia, tremor, and dyspraxia, characteristic of corticobasal degeneration.
- Subsequently, the patient developed cognitive deficits such as disorientation, confusion, and bradyphrenia.
Findings:
- Brain MRI revealed characteristic white matter lesions.
- Cerebrospinal fluid analysis showed a positive PCR test for JC virus, confirming the diagnosis of probable PML.
Implications:
- This case highlights the importance of considering PML in the differential diagnosis of movement disorders with cognitive decline.
- Early and accurate diagnosis through neuroimaging and JCV detection is crucial for appropriate management and prognosis.