Infundibulohypophysitis in a man presenting with diabetes insipidus and cavernous sinus involvement

Abstract

Infundibulohypophysitis is an unusual inflammatory condition that aVects the infundibulum, the pituitary stalk, and the neurohypophysis and may be part of a range that includes lymphocytic hypophysitis. Lymphocytic hypophysitis occurs mainly in women and most often presents in the later stages of pregnancy. Infundibulohypophysitis usually presents with diabetes insipidus and the cause remains unclear. The case of a 46 year old man with a 12 week history of polyuria and polydipsia is reported. Cranial diabetes insipidus was diagnosed on the basis of a water deprivation test. Initial cranial and pituitary imaging studies were normal. He subsequently developed symptoms of panhypopituitism over a period of 6-9 months and then, more acutely, developed diplopia secondary to a fourth nerve palsy. Further brain imaging studies disclosed an enhancing pituitary stalk and a left cavernous sinus lesion. An initial trial of immunosuppressive treatment did not help symptoms significantly. The diagnosis of infundibulohypophysitis was made on histological evidence. The patient was treated with prednisolone and methotrexate. At 9 months he is well, without symptoms, and the radiological abnormalities have resolved. (J Neurol Neurosurg Psychiatry 2001;71:798-801) Keywords: infundibulohypophysitis; cavernous sinus; diabetes insipidus Lymphocytic infundibulohypophysitis is an inflammatory condition aVecting the posterior pituitary gland and often presenting clinically with cranial diabetes insipidus.1 It is distinct from lymphocytic hypophysitis, which aVects the anterior pituitary and usually presents at the end of pregnancy with visual disturbances. 2DiVerentiating between the conditions may be diYcult and requires detailed pituitary function testing and MRI.3 A definitive diagnosis, however, requires histology. We describe the unusual case of a man with lymphocytic infundibulohypophysitis who presented with diabetes insipidus but, over the period of a year and despite immunosuppressive therapy, the condition progressed to involve most of the pituitary gland and the cavernous sinus. Treatment of both conditions remains controversial

Lymphocytic Hypophysitis with a Long Latent Period from Onset of Central Diabetes Insipidus to Development of Pituitary Enlargement

“…Typically, this disease occurs in young women in late pregnancy or in the postpartum period (2). However, there have been some recent reports of men presenting with lymphocytic hypophysitis (8)(9)(10). Previously, distinctions were made between lymphocytic hypophysitis and lymphocytic infundibulohypophysitis.…”

Parasellar T2 Dark Sign on MR Imaging in Patients with Lymphocytic Hypophysitis

“…41 Therefore, the parasellar T2 dark sign is more suitable for LYH that infiltrates around the pituitary. Some previous studies have reported cavernous involvement in patients with LYH, 5,6,10,[42][43][44][45][46] but no studies have mentioned the parasellar T2 dark sign.…”

Granulomatous hypophysitis: experience with eight surgical cases of a single center

“…Some authors have reported satisfactory responses to high dose steroid therapy and methotrexate injection [5,9,16,18,19]. The diagnosis of all cases was lymphocytic hypophysitis.…”