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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

970
Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency...
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Related Experiment Video

Updated: Jul 15, 2025

Visualizing Impairment of the Endothelial and Glial Barriers of the Neurovascular Unit during Experimental Autoimmune Encephalomyelitis In Vivo
10:50

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Progressive multifocal leukoencephalopathy without overt immunosuppression.

Varun Jain1, Hannah Branstetter1, Srikar Savaram1

  • 1Department of Neurology, College of Medicine, University of Florida, Gainesville, FL.

Medicine
|September 29, 2023
PubMed
Summary
This summary is machine-generated.

Progressive multifocal leukoencephalopathy (PML) can occur without obvious immunosuppression, challenging its rarity. This study found PML in 11.8% of cases without overt immunosuppression, suggesting it may be more common than previously thought.

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Area of Science:

  • Neurology
  • Infectious Diseases
  • Immunology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare, severe central nervous system disease.
  • PML is typically associated with significant immunodeficiency.
  • The occurrence and characteristics of PML in individuals without overt immunosuppression are not well-defined.

Purpose of the Study:

  • To investigate the prevalence and characteristics of PML cases occurring in the absence of overt immunosuppression.
  • To determine if PML without overt immunosuppression is more common than previously reported.

Main Methods:

  • Retrospective review of patients diagnosed with PML (ICD10 code A81.2) at a single center.
  • Defined PML without overt immunosuppression by excluding HIV, hematological malignancy, immunosuppressive medications, and specific autoimmune conditions.
  • Included only cases with definite clinical or histological PML diagnosis.

Main Results:

  • Out of 52 patients with the PML diagnosis code, 17 met definite PML criteria.
  • Overt immunosuppression was present in 15 of 17 (88.2%) cases, primarily due to HIV or immunosuppressive medications.
  • Two of 17 (11.8%) cases presented with PML without overt immunosuppression, with potential contributing factors including a dog bite with hypogammaglobulinemia and significant alcohol use.

Conclusions:

  • PML without overt immunosuppression may occur more frequently than previously recognized.
  • The diagnosis of PML should be considered even in patients without apparent immunosuppression if clinical and radiographic findings are suggestive.
  • Both identified cases without overt immunosuppression were fatal, highlighting the severity of the condition.