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Updated: Aug 31, 2026

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
[Progressive multifocal leukoencephalitis complicating polymyositis]
Mohamed Elloumi1, Nada Ayadi, Chokri Mhiri
1Service de rhumatologie, Hôpital universitaire Hédi Chaker, Sfax, Tunisie. elloumu02@yahoo.fr
Introduction:
Progressive multifocal leukoencephalitis (PML) must be evoked in patients presenting with a systemic disease during which multiple neurological deficiencies rapidly worsen.
Observation:
A 17 year-old girl suffering from histologically confirmed polymyositis was treated with corticosteroids. Two years after the diagnosis she exhibited global signs of cerebral damage with fever and magnetic resonance imaging evoked leukoencephalitis.
Comments:
An affection of the central nervous system, PML is characterised by the existence of multiple areas of demyelination in the hemispheric white substance of the cerebral trunk and sometimes the cerebellum, whereas the grey substance is usually spared. This entity occurs more frequently in HIV-infected patients, but also in patients in whom the immunodeficiency may have other causes, such as the treatment for a systemic disease for example.
Insights
Progressive multifocal leukoencephalitis (PML) is a serious brain infection that can occur in patients with weakened immune systems, often due to treatments for systemic diseases. Early recognition is crucial for managing this neurological condition.
Area of Science:
- Neurology
- Immunology
- Infectious Diseases
Background:
- Progressive multifocal leukoencephalitis (PML) is a demyelinating disease of the central nervous system.
- PML must be considered in patients with systemic diseases experiencing rapid neurological decline.
Observation:
- A 17-year-old female with polymyositis, treated with corticosteroids, presented with neurological symptoms.
- Cerebral damage, fever, and MRI findings suggestive of leukoencephalitis were observed two years post-diagnosis.
Findings:
- PML is characterized by multifocal demyelination in the cerebral white matter, sparing grey matter.
- While common in HIV-infected individuals, PML can also arise from other causes of immunodeficiency, including iatrogenic immunosuppression.
Implications:
- This case highlights the importance of considering PML in immunocompromised patients with neurological deficits.
- Corticosteroid treatment for systemic diseases can increase the risk of opportunistic infections like PML.
- Prompt diagnosis and management are essential for patients presenting with symptoms suggestive of PML.
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