Abstract
Aims: To investigate the causes, clinical characteristics, imaging features, and therapeutic implications of hypertrophic pachymeningitis (HP) in a southern Chinese population.Methods: We retrospectively analyzed 48 patients with HP with different causes from 1 January 2006 to 31 December 2018. Clinical manifestation, laboratory findings, and neuroimaging results were evaluated in all HP patients.Results: The mean age at onset was 50 ± 12 years. The most common diagnosis was idiopathic HP (67%), followed by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (15%), tuberculous meningitis (8%), viral meningitis (6%), and bacterial meningitis (4%). Headache was the most common symptom. The most frequently changed laboratory finding was elevated erythrocyte sedimentation rate (ESR). Imaging was characterized by cerebral or spinal dura mater enhancement in MRI scan with contrast. Enhancements were mainly located in the posterior fossa for idiopathic HP; frontal, parietal, and occipital lobes for ANCA-related HP; and posterior fossa for tuberculous-associated HP. Diffuse enhancement was found in most cases, except for tuberculous-associated HP. Glucocorticoid or immunosuppressive treatment was applied in most cases.Conclusions: The etiology of HP varied among patients, with idiopathic HP being the most common. MRI showed enhancement of the dura mater, which differed according to different etiologies. Glucocorticoid or immunosuppressive agents were the primary drugs for treatment.
Highlights
Hypertrophic pachymeningitis (HP) is a fibrosing inflammatory disorder featuring localized or diffused thickening of the cranial or spinal dura mater
The disorder can be divided into cranial or spinal pachymeningitis by lesion location and idiopathic or secondary HP by etiology
The entry criteria were defined as follows: (i) diagnosis was based on dura mater biopsy or dura mater enhancement in gadolinium MRI T1 sequences, and (ii) dural thickening could not be explained by intracranial hypotension, neoplastic pachymeningitis, or other conditions
Summary
Hypertrophic pachymeningitis (HP) is a fibrosing inflammatory disorder featuring localized or diffused thickening of the cranial or spinal dura mater. Enhanced MRI is currently the most powerful tool to diagnose HP. The disorder can be divided into cranial or spinal pachymeningitis by lesion location and idiopathic or secondary HP by etiology. Infections and autoimmune diseases are among the most identified causes of secondary HP [1]. The thickening of the dura mater is present in other conditions, such as intracranial hypotension syndrome or neoplastic pachymeningitis, which should be carefully differentiated to avoid misdiagnosis.
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