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Comprehensive Autopsy Program for Individuals with Multiple Sclerosis
Published on: July 19, 2019
Progressive multifocal leukoencephalopathy in patients with multiple sclerosis
Kelly J Baldwin1, Jeffery P Hogg
1Department of Neurology, West Virginia University School of Medicine, Morgantown, West Virginia, USA. kbaldwin@hsc.wvu.edu
Purpose Of Review:
Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection of the central nervous system. It has been recently associated with selective immunosuppression in patients with multiple sclerosis. This review describes the pathogenesis, clinical presentation, diagnosis, and treatment of natalizumab-associated PML.
Recent Findings:
Treatment of multiple sclerosis with natalizumab first involves risk stratifying patients. Clinicians can employ new tools for risk stratification including JC-virus antibody status, prior immunosuppression, and length of natalizumab treatment. These tools can help minimize the risk of developing PML. Identifying patients with natalizumab-associated PML poses a diagnostic challenge for clinicians. Unique clinical features, sensitive laboratory analyses, and advanced MRI techniques have been identified that aid in the diagnosis of natalizumab-associated PML.
Summary:
There continues to be significant gaps in our understanding of PML pathogenesis and its relationship with therapeutic immunosuppression. There have been advances made in our ability to treat multiple sclerosis, although these have come with the unintended risk of PML. Fortunately, natalizumab-associated PML remains a rare entity compared to multiple sclerosis-associated disability, and the risk may be mitigated with appropriate patient selection, accurate and rapid diagnosis, and aggressive treatment strategies.
Insights
Progressive multifocal leukoencephalopathy (PML) is a rare infection linked to natalizumab treatment for multiple sclerosis. Risk stratification, early diagnosis, and treatment can mitigate this risk.
Area of Science:
- Neuroimmunology
- Infectious Diseases
- Neurology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic central nervous system infection.
- PML is associated with selective immunosuppression, particularly in multiple sclerosis (MS) patients.
- Natalizumab, a treatment for MS, has been linked to an increased risk of PML.
Purpose of the Study:
- To review the pathogenesis, clinical presentation, diagnosis, and treatment of natalizumab-associated PML.
- To highlight advances in understanding and managing this rare complication.
- To inform clinicians on risk stratification and diagnostic strategies.
Main Methods:
- Literature review focusing on natalizumab-associated PML.
- Analysis of diagnostic challenges and advancements.
- Discussion of treatment strategies and risk mitigation.
Main Results:
- Risk stratification tools, including JC-virus antibody status, prior immunosuppression, and treatment duration, can help minimize PML risk.
- Diagnosis of natalizumab-associated PML presents challenges but is aided by unique clinical features, sensitive lab tests, and advanced MRI.
- Despite advances in MS treatment, PML remains an unintended risk.
Conclusions:
- Significant gaps remain in understanding PML pathogenesis and its link to therapeutic immunosuppression.
- Natalizumab-associated PML is rare compared to MS-related disability.
- Risk mitigation is possible through appropriate patient selection, accurate diagnosis, and aggressive treatment.
Related Concept Videos
Multiple Sclerosis l: Introduction
Encephalitis l: Introduction
Encephalitis ll: Pathophysiology
Parkinson's Disease: Overview
Hepatic Encephalopathy
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