Hypertrophic pachymeningitis is a rare disorder of the dura mater of the spine or brain. It can be caused by inflammatory, infective or neoplastic conditions or can be idiopathic. We report a man with hypertrophic pachymeningitis and bilateral chronic subdural haematoma caused by IgG4-related disease. We highlight the diagnostic challenges and discuss possible underlying mechanisms of subdural haematoma formation in inflammatory conditions. Isolated IgG4-related hypertrophic pachymeningitis with chronic subdural haematoma is very rare; previously reported cases have suggested a possible predilection for men in their sixth decade, presenting with headache as the dominant symptom. Given the rarity and complexity of the condition, it should be managed in a multidisciplinary team setting.
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Contributors NL, JA-A and DK have written the manuscript. IB has provided content and pictures in regard to the neuropathological aspects of the case. LC-J has provided content and pictures in regards to the neuroradiological aspects of the case. JG has provided content in regards to the rheumatological aspects of the case. All authors have been involved in the concipation of the manuscript and the proof reading process.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed by Claire Rice, Bristol, UK, and Katharine Harding, Newport, UK.