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
The case of a young woman who developed lymphocytic hypophysitis 2 weeks after delivery of a healthy baby is reported. The patient presented with clinical features suggestive of a pituitary mass lesion, but surgery was avoided when other clinical and radiologic features were considered. The patient recovered with steroid treatment only. We review the literature on this increasingly recognized condition and argue that medical management may be more suitable than previously thought.
A 19-year-old man presented with a 5-day history of vomiting and passing dark urine. He gave a 6-month history of arthralgias and joint swelling, particularly affecting larger joints. He had recurrent epistaxis and recent episcleritis. He was noted to be thin and pale, with conjunctival suffusion and deformity of his nasal bridge. He was normotensive (115/60 mm Hg) and apyrexic (35.5°C). Blood parameters revealed normocytic normochromic anemia and acute renal failure. Renal biopsy showed extensive crescent formation with segmental necrotizing inflammation and fibrin deposition of the glomeruli. cANCA was strongly positive (titer 1280; ELISA PR3ϩ) and he was diagnosed with Wegener granulomatosis. He commenced hemodialysis, steroids, and oral cyclophosphamide, and he was discharged home.One month later, he presented with acute onset of throbbing headaches associated with nausea and photophobia, as well as recurrent generalized seizures necessitating sedation and intensive care unit admission. He was pyrexic (38°C) and hypertensive (165/95 mm Hg). Renal indices were abnormal (urea 29.1mmol/L; creatinine 755 mol/L; potassium 5.4 mmol/L). Clinical examination revealed no focal neurologic deficits.Initial noncontrast brain CT was normal. Brain MRI with gadolinium showed small, ill-defined, nonenhancing high signal areas measuring 5-20 mm in several locations (figure, A).
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