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An Update on Idiopathic Hypertrophic Cranial Pachymeningitis for the Headache Practitioner

  • Uncommon and/or Unusual Headaches and Syndromes (J Ailani, Section Editor)
  • Published:
Current Pain and Headache Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

We aim to review idiopathic hypertrophic cranial pachymeninigitis (IHCP), describe common head pain patterns and features associated with the disorder, suggest potential classification of head pain syndromes based on the recently published International Classification of Headache Disorders-3, explore pathophysiology found to be associated with cases of IHCP, and indicate common treatment for the disorder.

Recent Findings

It is suggested that a subset of IHCP is an IgG4-related autoimmune disorder. Patients with IHCP were found to have elevated cerebrospinal fluid (CSF) protein and lymphocytic pleocytosis. Corticosteroids are a mainstay of treatment. Other immunosuppressive agents and steroid sparing agents as add-on therapy may have utility in the treatment of cases refractory to corticosteroids alone.

Summary

Clinical manifestations of IHCP depend upon the location of the inflammatory lesions and compression of the adjacent nervous system structures. Headache and loss of cranial nerve function were the most common presenting features of hypertrophic cranial pachymeninigitis. Several headache diagnoses may result from IHCP. Gadolinium-enhanced MRI is the standard imaging modality for diagnosing. Although the pathophysiology is poorly understood, many cases of hypertrophic pachymeninigitis (HP) are thought to be closely related to inflammatory disorders. Cases of HP previously thought to be idiopathic may have IgG4 pathophysiology. CSF and serological studies are helpful. Treatment involves immunosuppressive agents. Advancement in neuroimaging, assays, tests, and further delineation of inflammatory disorders affecting the nervous system may provide further insight to the etiology of cases of HP previously considered and diagnosed as idiopathic.

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Authors and Affiliations

  1. Department of Neurology, University of Michigan, 1914 Taubman Center, 1500 E. Medical Center Dr. SPC 5316, Ann Arbor, MI, 48109-5316, USA

    Larry Charleston IV

  2. Department of Anesthesiology, University of Michigan, Ann Arbor, MI, USA

    Wade Cooper

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Correspondence to Larry Charleston IV.

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Conflict of Interest

Larry Charleston IV reports receiving personal fees for acting as a consultant/advisory board member for Allgeran/Abbvie, Alder, Biohaven, and Impax. Wade Cooper reports receiving personal fees for acting as a consultant/advisory board member for Teva, Lilly, Allergan/Abbvie, Biohaven, Amgen, Alder and Theranica.

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Charleston, L., Cooper, W. An Update on Idiopathic Hypertrophic Cranial Pachymeningitis for the Headache Practitioner. Curr Pain Headache Rep 24, 57 (2020). https://doi.org/10.1007/s11916-020-00893-5

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