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Diagnostic delay in progressive multifocal leukoencephalopathy.

Dhanashri P Miskin¹, Long H Ngo², Igor J Koralnik¹

¹Division of Neuro-Immunology Departments of Neurology and Medicine Center for Virology and Vaccine Research Beth Israel Deaconess Medical Center Harvard Medical School Boston Massachusetts.

Annals of Clinical and Translational Neurology

|May 28, 2016

Summary

This summary is machine-generated.

Diagnosing progressive multifocal leukoencephalopathy (PML) often takes over a month, with a median of 74 days. Delays are common, especially in HIV-positive patients with higher CD4 counts, impacting survival.

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Area of Science:

Neurology
Infectious Diseases
Immunology

Background:

Progressive multifocal leukoencephalopathy (PML) is a rare, severe demyelinating disease of the central nervous system.
PML is caused by the JC virus (JCV) and primarily affects immunocompromised individuals.
Diagnostic delays can significantly impact patient outcomes and treatment strategies.

Purpose of the Study:

To investigate the extent and factors associated with diagnostic delay in progressive multifocal leukoencephalopathy (PML).
To analyze the time from symptom onset to diagnosis in a cohort of PML patients.
To identify patient subgroups experiencing longer diagnostic delays.

Main Methods:

Retrospective analysis of 111 patients diagnosed with PML between 1993 and 2015.
Calculation of the median time from initial symptom to diagnosis.
Comparison of diagnostic timelines across different patient characteristics and underlying conditions.

Main Results:

The median diagnostic delay for PML was 74 days (range: 1-1643 days).
Nearly two-thirds of patients had an alternative diagnosis considered before PML.
Over three-quarters of patients experienced a diagnostic delay exceeding one month.
Extended delays were more frequent in patients with possible PML and in HIV-positive individuals with higher CD4+ T-cell counts at symptom onset.

Conclusions:

Significant diagnostic delays are common in progressive multifocal leukoencephalopathy, regardless of the underlying immunosuppressive condition.
Prompt diagnosis of PML is crucial for initiating appropriate management, potentially improving survival through immune reconstitution and avoiding unnecessary interventions.