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Encephalitis and CSF increased level of interferon-α in Kikuchi-Fujimoto disease

Antoine Guéguen1, Thomas Sené, Elisabeth Maillart

  • 1Neurology Department, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France. agueguen@fo-rothschild.fr

BMJ Case Reports
|August 29, 2012
PubMed

Insights

Kikuchi-Fujimoto disease (KFD) can cause encephalitis, characterized by specific brain lesions and elevated interferon-alpha (IFN-α) in cerebrospinal fluid. This finding aids in diagnosing neurological complications of KFD.

Area of Science:

  • Neurology
  • Immunology
  • Radiology

Background:

  • Kikuchi-Fujimoto disease (KFD) is a rare, benign lymphohistiocytic disorder.
  • Neurological manifestations in KFD are recognized but poorly characterized.
  • Diagnostic biomarkers for KFD-associated neurological conditions are lacking.

Observation:

  • A 39-year-old male presented with headaches, diplopia, cognitive, and behavioral changes.
  • Brain MRI revealed pontine and temporal lobe lesions with perivascular and ependymal enhancement.
  • Cervical and mediastinal lymphadenopathy was identified via PET scan.

Findings:

  • Cerebrospinal fluid (CSF) analysis showed elevated interferon-alpha (IFN-α) levels without evidence of viral infection.
  • Biopsy confirmed Kikuchi-Fujimoto disease.
  • The patient's encephalitis spontaneously resolved.

Implications:

  • This study characterizes specific brain lesion patterns in KFD-associated encephalitis.
  • Elevated CSF IFN-α levels may serve as a potential biomarker for neurological involvement in KFD.
  • Findings contribute to understanding and diagnosing KFD neurological complications.

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