National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Idiopathic hypertrophic pachymeningitis


Other Names:
Idiopathic hypertrophic cranial pachymeningitis; Idiopathic hypertrophic spinal pachymeningitis; Idiopathic hypertrophic craniospinal pachymeningitis
Idiopathic hypertrophic pachymeningitis (IHP) is a rare disorder causing inflammation and thickening of the outer layer (dura) of the brain and/or spinal cord.[1][2] IHP can be widespread or cause tumor-like lesions.[1] Before IHP can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections (syphilis, tuberculosis, and Lyme disease), and autoimmune diseases ( rheumatoid arthritis, Sjogren’s syndrome, Wegener’s granulomatosis, and IgG4-related disease) must be ruled out. IHP often presents with headache and cranial nerve impairment.

Treatment may involve prednisone and/or an immune suppressing drug. This treatment often improves symptoms, however complete recovery is rare.[1] Surgery may be recommended for people with advanced or severe IHP.[2] Some people with IHP have no symptoms and may not need treatment.[1]
Last updated: 3/30/2017

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In-Depth Information

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  1. Hahn LD1, Fulbright R2, Baehring JM3. Hypertrophic pachymeningitis. J Neurol Sci. Aug 15, 2016; 367:278-83. Accessed 3/30/2017.
  2. Lee YC, Chueng YC, Hsu SW, Lui CC. Idiopathic Hypertrophic Cranial Pachymeningitis: Case Report with 7 Years of Imaging Follow-up. American Journal of Neuroradiology. January 2003; 24(1):119-123. http://www.ajnr.org/content/24/1/119.full. Accessed 3/30/2017.