ArticleActa neurochirurgica2022
IgG4-related hypertrophic pachymeningitis with tumor-like intracranial and intracerebral lesions.
Article in Acta neurochirurgica, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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10 citing papers in PubMed, 9 citations in OpenAlex.
- Estimated prevalence and distribution of causes of immune-mediated hypertrophic pachymeningitis in São Paulo, Brazil.Clinical rheumatology · 2026Article
- IgG4-Related Disease Manifested as Hypertrophic Pachymeningitis: A Case Report and Literature Review.Diagnostics (Basel, Switzerland) · 2026Article
- Recurrent spinal IgG4-related hypertrophic pachymeningitis and its management: a case report.Frontiers in immunology · 2026Article
- Histologically confirmed immunoglobulin G4-related hypophysitis in an adolescent girl: a case report with review of literature.Endocrine journal · 2025Review
- Immunoglobulin G4 biomarkers and pathogenesis in immunoglobulin G4-related spinal pachymeningitis.World journal of clinical cases · 2025Article
- IgG4-Related Disease in Childhood: Clinical Presentation, Management, and Diagnostic Challenges.Children (Basel, Switzerland) · 2025Review
- A case report of Immunoglobulin-G4-related hypertrophic sclerosing pachymeningitis.Radiology case reports · 2024Article
- Article
- Seronegative Immunoglobulin G4-related hypertrophic pachymeningitis misdiagnosed as meningitis and meningioma.The Kaohsiung journal of medical sciences · 2024Article
- Granulomatosis with Polyangiitis Presenting with Thrombovasculitic and Necrotizing Pachy- and Leptomeningitis Accompanied by a Brain Tumor-like Lesion.Internal medicine (Tokyo, Japan) · 2023Article
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8 authors at 1 institution in 1 country.
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Abstract
objectiveIgG4-related hypertrophic pachymeningitis is a rare fibroinflammatory disorder that may cause localized or diffused thickening of the dura mater. Misinterpretations of the clinical and imaging findings are common. Clinical manifestations depend on the location of the inflammatory lesion and on compression of neural structures leading to functional deficits. A dural biopsy is commonly needed for a definitive diagnosis. Immunomodulatory therapy is considered the therapy of choice.
methodsFour patients with IgG4-related hypertrophic pachymeningitis were identified over a 5-year period. Patient-related characteristics including age, preoperative workup, signs and symptoms of patients, and diagnostic procedures were evaluated. Furthermore, the surgical treatment and 5-year follow-up outcomes were analyzed.
resultsThere were two adults and two adolescents (mean age 32 years; range 15 to 67 years). Two patients were male, and two were female. No history of disease was known in any of the patients. Clinical symptoms were epilepsy (n = 2), ataxia and nausea (n = 1), and facial nerve palsy (n = 1). MR imaging studies showed contrast enhancing lesions in the temporal region in two patients, and in the cerebellar region in the other two patients. Subtotal resection was performed in two instances and a biopsy via a suboccipital retrosigmoid approach was obtained in the other two patients. Histochemical and immunohistochemical investigations revealed an IgG 4 disease in all of these patients. Immunomodulatorry therapy led to clinical stability during follow-up of 5 years in all four cases.
conclusionThe diagnosis of IgG4-related hypertrophic pachymeningitis is challenging, but is of great relevance as treatment differs significantly from other forms of pachymeningitis and a specific therapeutic approach may avoid long-term neurological complications. Our series contributes to a better clinical characterization of this rare disease.
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