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The national incidence of PML in Sweden, 1988-2013

Ellen Iacobaeus1, Sarah Burkill2, Shahram Bahmanyar2

  • 1From the Department of Clinical Neuroscience, Division of Neurology (E.I., R.H., T.O., L.B.), Neuroimmunology Unit, Center for Molecular Medicine, Department of Clinical Neuroscience (T.O., L.B.), and Center for Pharmacoepidemiology (S. Burkill, S. Bahmanyar, C.B.) and Clinical Epidemiology Unit (M.F., S.M.), Department of Medicine, Solna, Karolinska Institutet, Stockholm; Clinical Epidemiology and Biostatistics (S.M.), School of Medical Sciences, Örebro University, Sweden; and Department of Epidemiology and Public Health (S.M.), University College London, UK. Ellen.Iacobaeus@karolinska.se.

Neurology
|January 12, 2018
PubMed
Abstract

Insights

The incidence of progressive multifocal leukoencephalopathy (PML) in Sweden increased significantly from 2011-2013, coinciding with monoclonal antibody use. Accurate PML diagnosis is crucial and more common in neurology departments.

Area of Science:

  • Neurology
  • Epidemiology
  • Immunology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare, opportunistic demyelinating disease of the central nervous system.
  • PML is primarily associated with the JC virus and occurs in immunocompromised individuals.

Purpose of the Study:

  • To determine the incidence and patient characteristics of PML in Sweden from 1988 to 2013.
  • To investigate potential associations between PML incidence and specific patient factors or treatments.

Main Methods:

  • A nationwide population-based study using the Swedish National Patient Register.
  • Inclusion of all PML diagnoses between 1988 and 2013, with validation through medical record review.
  • Analysis of patient demographics, underlying conditions, and prior treatments.

Main Results:

  • Of 250 identified cases, 108 were confirmed as definite, probable, or possible PML.
  • PML incidence rose from 0.026 per 100,000 individuals (1988-2010) to 0.11 per 100,000 (2011-2013).
  • Common underlying conditions included hematologic malignancies, HIV/AIDS, and autoimmune diseases; 26 patients had prior monoclonal antibody treatment.

Conclusions:

  • An increased incidence of PML in Sweden was observed, correlating with prior monoclonal antibody use.
  • Accurate PML diagnosis was significantly higher in neurology departments, highlighting the need for specialized care.
  • Prompt neurological consultation is vital for suspected PML cases due to diagnostic challenges.

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