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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
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Published on: July 4, 2007

Progressive multifocal leukoencephalopathy - the importance of early diagnosis illustrated in four cases

O Nived1, A A Bengtsson, A Jönsen

  • 1Department of Rheumatology, Clinical Sciences, Lund University Hospital, SE-221 85 Lund, Sweden. ola.nived@med.lu.se

Lupus
|October 15, 2008
PubMed

Insights

Progressive multifocal leukoencephalopathy (PML) is a rare, deadly brain disease. Early diagnosis and reduced immunosuppression improve survival in patients with SLE and RA.

Area of Science:

  • Neuroscience
  • Immunology
  • Infectious Diseases

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare, fatal demyelinating disease of the central nervous system.
  • PML is caused by the JC polyomavirus, typically reactivating in immunosuppressed individuals.
  • Cases are rare in systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) patients.

Observation:

  • Four new PML cases are reported: three in SLE patients and one in an RA patient.
  • One SLE patient and one RA patient survived PML.
  • Stereotactic brain biopsy was crucial for diagnosing three of the four cases, including the survivors.

Findings:

  • Survival was achieved through reduced immunosuppressive therapy and, in one case, without antiviral treatment.
  • PML diagnosis can be challenging, often necessitating brain biopsy.
  • PML may be more prevalent in SLE patients than previously estimated (1 in 800 patient-years).

Implications:

  • Early diagnosis and prompt reduction of immunosuppression are critical for improving PML outcomes.
  • Brain biopsy remains an important diagnostic tool for suspected PML.
  • The findings suggest a higher incidence of PML in SLE patients, warranting increased vigilance.

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