SynthesisNeurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2025
A case of IgG4-related spinal pachymeningitis with a large spinal cord cavity: case report and updated systematic review.
Synthesis in Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- IgG4-related spinal hypertrophic pachymeningitis: a systematic review.European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society · 2026Review
- New Developments in the Treatment of IgG4-Related Disease: A Comprehensive Clinical Approach.Journal of clinical medicine · 2025Review
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Authors and funding
4 authors.
Funding
Abstract
backgroundIgG4-related spinal pachymeningitis (IgG4-RSP) is rare, particularly in cases where spinal cord cavities develop after the removal of thickened dura mater, making diagnosis challenging. A 31-year-old male presented with IgG4-RSP, and the clinical presentation, diagnostic imaging, pathology, treatment, and follow-up outcomes were documented. We also conducted a thorough review of the relevant literature. CASE REPORT: The patient presented with two-year progressive lower limb weakness. He experienced lower back pain, numbness in both legs, and urinary and defecatory difficulties without a notable medical history. Initial magnetic resonance imaging (MRI) revealed dura mater lesions and thoracic spinal cord compression, leading to surgery that did not improve the symptoms. Pathological examination confirmed IgG4-related hypertrophic pachymeningitis. Physical examination revealed a reduced pain sensation below both rib margins and a muscle strength score of 3/5 in the lower limbs. Thoracic MRI revealed significantly enlarged spinal cord cavities compared to previous scans. Methylprednisolone treatment partially improved symptoms. Subsequent efgartigimod alfa treatment did not alleviate the symptoms further. The patient exhibited significant neurological deficits. LITERATURE REVIEW: Studies were identified through PubMed, resulting in 20 case reports that met the criteria and were pathologically confirmed. More than half of all patients with IgG4-RSP experience neck or back pain, with lesions typically found in the cervical spine and extending to the thoracic level.
conclusionsCaution should be exercised in the post-surgical spinal cord cavity. One-quarter of patients have a poor prognosis. Further research is needed to understand the pathophysiology of IgG4-RSP and develop specific treatments.
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