Primary central nervous system vasculitis preceded by granulomatous hypophysitis: Case report with a review of the literature

BACKGROUND: Primary central nervous system (CNS) vasculitis is an idiopathic inflammatory process that selectively affects CNS vasculature without a systemic inflammatory response, and causes luminal obstruction with resultant ischemia of recipient tissue. Its varying clinical symptoms and signs depend on the caliber of vessels involved and distribution and location of the affected structures. Granulomatous hypophysitis (GH) is an autoimmune inflammatory process typically affecting women, and usually presents with hypopituitarism, and at times, diabetes insipidus, and/or visual loss. Both entities are rare CNS diseases, which, to our knowledge, have never been previously reported in the same patient. CASE DESCRIPTION: We present a unique case of chronic progressive primary CNS vasculitis causing limbic encephalopathy in a 30-year-old male with only a history of medication-controlled hypertension. He initially presented 4 months prior with nonspecific neurological complaints and was found to have a homogenously enhancing and enlarged pituitary, which was biopsy proven to be GH. CONCLUSION: This rather unique presentation highlights the need to maintain a high index of suspicion for underlying PCNS vasculitis in a patient who does not fit the typical demographic for isolated GH.