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A case of IgG4-related spinal pachymeningitis with a large spinal cord cavity: case report and updated systematic

Jiaqian Zhu1,2, Yanni Yu1,2, Peng Sun3,4

  • 1School of Medicine, Shenzhen University, Shenzhen, China.

Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
|July 27, 2025
PubMed
Abstract

Insights

IgG4-related spinal pachymeningitis (IgG4-RSP) can cause challenging spinal cord cavities after surgery. Early diagnosis and treatment are crucial, as a significant portion of patients face a poor prognosis.

Area of Science:

  • Neurology
  • Immunology
  • Pathology

Background:

  • IgG4-related spinal pachymeningitis (IgG4-RSP) is a rare condition.
  • Spinal cord cavities developing after dural thickening removal present diagnostic challenges.
  • This report details a case of IgG4-RSP in a 31-year-old male.

Purpose of the Study:

  • To document the clinical presentation, imaging, pathology, treatment, and outcomes of a challenging IgG4-RSP case.
  • To review existing literature on IgG4-RSP, focusing on diagnostic and prognostic factors.

Main Methods:

  • Case report of a patient with progressive neurological deficits.
  • Review of 20 pathologically confirmed IgG4-RSP case reports from PubMed.
  • Analysis of diagnostic imaging (MRI) and pathological findings.

Main Results:

  • The patient presented with lower limb weakness, back pain, and sensory deficits.
  • Initial surgery did not improve symptoms; MRI revealed worsening spinal cord cavities.
  • Methylprednisolone provided partial symptom improvement, but further treatment was ineffective.
  • Literature review indicated neck/back pain and cervical/thoracic lesions are common in IgG4-RSP.

Conclusions:

  • Post-surgical spinal cord cavities require careful monitoring in IgG4-RSP.
  • Approximately 25% of patients with IgG4-RSP have a poor prognosis.
  • Further research is needed to elucidate IgG4-RSP pathophysiology and develop targeted therapies.
Keywords:
Clinical featuresIgG4-related diseaseSpinal cord cavitySpinal pachymeningitisSurgery

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