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IgG4-related hypertrophic pachymeningitis with chronic subdural haematoma
  1. Nicole Lichtblau1,
  2. Jahard Aliaga-Arias2,3,
  3. Dimitrios Kalaitzoglou2,
  4. Istvan Bodi4,
  5. Keyoumars Ashkan2,
  6. Ranj Bhangoo2,
  7. Francesco Vergani2,
  8. Dorothy Joe1,
  9. Biba Stanton1,5,
  10. James Galloway6,
  11. Lalani Carlton-Jones7,
  12. Jose Pedro Lavrador2
  1. 1Department of Neurology, King's College Hospital NHS Foundation Trust, London, UK
  2. 2Department of Neurosurgery, King's College Hospital NHS Foundation Trust, London, UK
  3. 3Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, Universita degli Studi di Brescia, Brescia, Italy
  4. 4Clinical Neuropathology, King's College Hospital NHS Foundation Trust, London, UK
  5. 5Neuropsychiatry Service, South London and Maudsley NHS Trust, London, UK
  6. 6Department of Rheumatology, King's College Hospital NHS Foundation Trust, London, UK
  7. 7Department of Neuroradiology, King's College Hospital NHS Foundation Trust, London, UK
  1. Correspondence to Dr Nicole Lichtblau, Neurology, King's College Hospital NHS Foundation Trust, London, SE5 9RS, UK; nicole.lichtblau{at}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.

  • NEUROIMMUNOLOGY
  • NEUROPATHOLOGY
  • NEURORADIOLOGY

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Data availability statement

Data are available on reasonable request.

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Footnotes

  • 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.