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Published on: June 18, 2021
IgG4-related hypertrophic pachymeningitis with chronic subdural haematoma
Nicole Lichtblau1, Jahard Aliaga-Arias2,3, Dimitrios Kalaitzoglou2
1Department of Neurology, King's College Hospital NHS Foundation Trust, London, UK nicole.lichtblau@nhs.net.
Abstract:
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.
Insights
This study reports a rare case of hypertrophic pachymeningitis and chronic subdural hematoma caused by IgG4-related disease. It highlights diagnostic challenges and management strategies for this complex neurological condition.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Hypertrophic pachymeningitis is a rare dural disorder.
- Causes include inflammatory, infectious, neoplastic conditions, or idiopathic factors.
Purpose of the Study:
- To report a rare case of hypertrophic pachymeningitis with bilateral chronic subdural hematoma.
- To discuss diagnostic challenges and potential mechanisms of subdural hematoma in inflammatory conditions.
Main Methods:
- Case report of a male patient.
- Review of diagnostic challenges and underlying mechanisms.
Main Results:
- The patient presented with hypertrophic pachymeningitis and bilateral chronic subdural hematoma.
- The condition was attributed to IgG4-related disease.
Conclusions:
- Isolated IgG4-related hypertrophic pachymeningitis with chronic subdural hematoma is exceptionally rare.
- Multidisciplinary team management is recommended for this complex condition.

