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Neuro-Sweet's disease manifesting as limbic encephalitis and ventriculitis

Aoi Shiina1, Makoto Shibata1, Hiroo Kasahara1

  • 1Department of Neurology, Gunma University Graduate School of Medicine, Maebashi, Japan.

Clinical Neurology and Neurosurgery
|March 22, 2025
PubMed

Insights

Neuro-Sweet's disease (NSD) can present with rare brain MRI findings resembling limbic encephalitis. Early diagnosis and treatment with steroids and colchicine significantly improve neurological symptoms in these cases.

Area of Science:

  • Neurology
  • Immunology
  • Radiology

Background:

  • Neuro-Sweet's disease (NSD) is a rare systemic inflammatory disorder.
  • Typical NSD involves fever, neutrophilia, and painful cutaneous lesions.
  • Neurological involvement is uncommon and presents diagnostic challenges.

Observation:

  • A patient presented with altered consciousness and hemiparesis, lacking typical skin manifestations of NSD.
  • Brain MRI revealed findings mimicking limbic encephalitis and ventriculitis.
  • Cerebrospinal fluid analysis showed elevated interleukin-6 and increased neutrophils.

Findings:

  • The patient tested positive for HLA-B54 and HLA-Cw1, associated with NSD.
  • Brain biopsy confirmed neutrophilic inflammation.
  • These findings supported a diagnosis of neuro-Sweet's disease despite atypical presentation.

Implications:

  • NSD should be considered in the differential diagnosis for patients with neuroimaging suggestive of limbic encephalitis or ventriculitis.
  • Prompt recognition and immunosuppressive therapy, including corticosteroids and colchicine, can lead to significant neurological recovery.
  • This case highlights the importance of considering systemic inflammatory conditions in neurological presentations.
Keywords:
Brain biopsyHuman leukocyte antigenLimbic encephalitisNeuro-Sweet’s diseaseVentriculitis

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