Epidemiological, clinical, and radiological assessment of hypertrophic pachymeningitis in a tertiary neurology center in Brazil

Background: Hypertrophic pachymeningitis (HP) is a rare inflammatory disease characterized by localized or diffuse thickening of the dura mater. It manifests with varied neurological symptoms, especially persistent headache and cranial nerve palsies. Etiologies include infections, autoimmune diseases, neoplasms, and idiopathic forms. MRI with gadolinium enhancement is the imaging method of choice, while dural biopsy remains the diagnostic gold standard. Early identification is crucial to prevent permanent deficits, but diagnosis is often delayed due to its nonspecific presentation. Objective: This study aims to describe the clinical, epidemiological, and radiological profile of HP in a tertiary neurology center in Brazil. Methods: Data from 51 patients with HP were retrospectively analyzed. All underwent blood tests, HIV serology, autoantibody panels, chest CT, and gadolinium-enhanced brain MRI. Cerebrospinal fluid (CSF) analysis included routine parameters and specific microbiological stains. Results: Among the 51 patients, 14 were men and 37 women, with a mean age of 47.85 years (range 19–74); 49% were aged 40–60. Headache was the initial symptom in 80.39%, followed by cranial nerve involvement in 84.31%—notably nerves II, VI, and V. ESR was elevated in 15.7%, CRP in 19.6%, and both in 39.2%. ANA was positive in 16% of patients. MRI showed localized dural thickening in 74.5%, predominantly in the tentorial, parasellar, and falx regions. Hyperproteinorrachia was present in 51%. Etiologic classification identified 9 cases of granulomatosis with polyangiitis, 7 of neurosarcoidosis, and 7 idiopathic; 14 remained undefined. Conclusion: This study highlights the clinical heterogeneity of HP and the diagnostic value of combining imaging, laboratory, and CSF data. The findings support the creation of a structured diagnostic approach to improve early recognition and management of this rare condition.

• Publication year

  2025

• Venue

  Arquivos de Neuro-Psiquiatria

• Publication date

  Unknown publication date

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  Not labeled

• Identifiers
  DOI 10.5327/eani25043
• External record

  Open on Semantic Scholar

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  Semantic Scholar

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