Idiopathic hypertrophic pachymeningitis

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Disease Overview

Idiopathic hypertrophic pachymeningitis (IHP) is a rare disorder causing inflammation and thickening of the outer layer (dura) of the brain and/or spinal cord.[13281][13282] IHP can be widespread or cause tumor-like lesions.[13281] 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.[13281] Surgery may be recommended for people with advanced or severe IHP.[13282] Some people with IHP have no symptoms and may not need treatment.[13281]


Synonyms

  • Idiopathic hypertrophic cranial pachymeningitis
  • Idiopathic hypertrophic spinal pachymeningitis
  • Idiopathic hypertrophic craniospinal pachymeningitis

For more information, visit GARD.

National Organization for Rare Disorders