General description

Hypertrophic pachymeningitis is an inflammatory condition characterized by chronic headache and cranial neuropathy, commonly affecting middle-aged individuals. Symptoms related to cranial nerve involvement may include visual deficits, auditory disturbances, facial nerve palsy, oculomotor disturbances, and diplopia.

The condition can be caused by various systemic inflammatory diseases such as granulomatosis with polyangiitis (GPA), Rheumatoid arthritis, and IgG4-related disease, as well as infectious diseases, malignancies, SAPHO syndrome, POEMS syndrome, and cerebrospinal fluid hypovolemia. When no underlying primary disease is identified, it is termed idiopathic hypertrophic pachymeningitis.

Radiographic features

Imaging studies typically reveal focal or nodular thickening of the dura mater with contrast enhancement. A specific finding of hypertrophic pachymeningitis is the T2WI hypointensity of the thickened dura.