Lymphocytic panhypophysitis in a young man with involvement of the cavernous sinus and clivus

Abstract

Lymphocytic hypophysitis is an unusual inflammatory lesion that is caused by autoimmune destruction of the pituitary gland. We report a case of 42-year-old man who presented with a 6-month history of severe headache, blurred vision in the right eye, hearing loss, polyuria, polydipsia, and impotence. Medical history showed that he and his mother had osteopetrosis. The results of the physical examination and laboratory tests showed that secondary hypothyroidism, hypogonadism, and hypocortisolism had developed. Central diabetes insipidus was diagnosed by water deprivation test. MRI of the sella showed pituitary enlargement with symmetrical suprasellar expansion, compression of the chiasma, thickened infundibulum, and involvement of both bilateral cavernous sinuses and clivus. Hormonal substitution with hydrocortisone, levothyroxine, and DDAVP resulted in rapid improvement of all symptoms and signs. Transsphenoidal biopsy was diagnostic of lymphocytic hypophysitis. In spite of extensive literature reviewing, we have not been aware of any case of lymphocytic hypophysitis with clivus involvement. The present case represents a variant of lymphocytic hypophysitis which has progressed to involve bilateral cavernous sinuses and the clivus.

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.…”

A Radiologic Score to Distinguish Autoimmune Hypophysitis from Nonsecreting Pituitary Adenoma Preoperatively

“…17 T2-weighted and fat-suppressed sequences are also useful, specifically in the search of clival bone marrow edema, which has been reported in some cases of hypophysitis, 31 but these sequences are not routinely performed and thus could not be included in our study.…”

Lymphocytic hypophysitis in a patient presenting with sequential episodes of optic neuritis

“…Very few cases of LYH have been reported previously in association with ON, and each of those patients presented with CDI initially, was diagnosed with a subtype of LYH, and later developed ON [4][5][6]. There is one case report of a middle-aged man with LIPH presenting with multiple progressive symptoms who initially had unilateral blurry vision four months prior that spontaneously resolved in a few weeks, and while the publication appears to attribute the patient's visual symptoms to optic chiasmal involvement as seen on MRI, these images were not obtained until months after his vision returned [7]. In the case of our patient, the imaging and clinical findings at the time of initial presentation confirm that the initial diagnosis was ON rather than visual loss from compression of the optic apparatus.…”

“…The mainstay of treatment for LYH is medical, including hormone replacement and immunosuppression with corticosteroids [1,2,[7][8][9], although steroid-unresponsive disease has been described and may be treated with other drugs or radiation therapy [1,7,8,10]. Surgical resection (typically via a transsphenoidal approach) may be required in cases of substantial mass effect on the optic apparatus.…”

“…Surgical resection (typically via a transsphenoidal approach) may be required in cases of substantial mass effect on the optic apparatus. Hypopituitarism is almost certainly related to dysfunction of inflamed pituitary gland rather than to compression of functional parenchyma by the affected tissue, and surgery should not be performed with the goal of improving endocrinological function [7].…”