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Pituitary apoplexy can occur spontaneously or following anterior pituitary stimulation tests. Apoplexy is a rare complication of Cushing's disease. We report a 19-year-old woman who was admitted to the National Institutes of Health for evaluation of possible Cushing's syndrome. Her symptoms and initial laboratory work were suggestive of Cushing's disease. Magnetic resonance imaging (MRI) revealed a macroadenoma of the pituitary gland. As part of her evaluation she received corticotrophin-releasing hormone (CRH). Two days later she developed severe headache, accompanied by nausea and vomiting, followed by meningismus, ptosis and diplopia. A diagnosis of pituitary apoplexy was made and she was treated conservatively with dexamethasone. Her neurological symptoms resolved shortly afterwards. By the time of discharge her anterior pituitary function was suppressed. All symptoms and signs of Cushing's syndrome resolved thereafter. This is the first case to demonstrate that CRH administration can induce pituitary apoplexy in a patient with Cushing's disease. Therapy with glucocorticoids was effective in our case, suggesting that conservative treatment can be successfully and safely applied in certain cases with pituitary apoplexy.
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PMID:Pituitary apoplexy induced by corticotrophin-releasing hormone in a patient with Cushing's disease. 1269 33

A 73-year-old woman presented with disturbance of right eyelid opening with ptosis and impairment of right ocular movement, and later was found to have Cushingoid features. Endocrine examinations revealed that plasma ACTH and cortisol levels were elevated, lack of circadian rhythm, resistant to low-doses (0.5, 1, 2 mg) and high-dose (8 mg) dexamethasone, and responsive to CRH and DDAVP. Magnetic resonance imaging (MRI) revealed a large pituitary tumor invading the right cavernous sinus. After two months treated with bromocriptine (5 mg/day), she showed clinical improvement with normalization of plasma ACTH and cortisol levels, and improvement of right eyelid opening and ocular movement. MRI, however, revealed no apparent reduction in the size of pituitary tumor. This is a rare case of bromocriptine-responsive Cushing's disease.
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PMID:Bromocriptine-responsive Cushing's disease; clinical and biochemical remission accompanied by amelioration of impaired ocular movement. 1763 11

The human eye, as an organ, can offer critical clues to the diagnosis of various systemic illnesses. Ocular changes are common in various endocrine disorders such as diabetes mellitus and Graves' disease. However there exist a large number of lesser known endocrine disorders where ocular involvement is significant. Awareness of these associations is the first step in the diagnosis and management of these complex patients. The rare syndromes involving the pituitary hypothalamic axis with significant ocular involvement include Septo-optic dysplasia, Kallman's syndrome, and Empty Sella syndrome all affecting the optic nerve at the optic chiasa. The syndromes involving the thyroid and parathyroid glands that have ocular manifestations and are rare include Mc Cune Albright syndrome wherein optic nerve decompression may occur due to fibrous dysplasia, primary hyperparathyroidism that may present as red eye due to scleritis and Ascher syndrome wherein ptosis occurs. Allgrove's syndrome, Cushing's disease, and Addison's disease are the rare endocrine syndromes discussed involving the adrenals and eye. Ocular involvement is also seen in gonadal syndromes such as Bardet Biedl, Turner's, Rothmund's, and Klinefelter's syndrome. This review also highlights the ocular manifestation of miscellaneous syndromes such as Werner's, Cockayne's, Wolfram's, Kearns Sayre's, and Autoimmune polyendocrine syndrome. The knowledge of these relatively uncommon endocrine disorders and their ocular manifestations will help an endocrinologist reach a diagnosis and will alert an ophthalmologist to seek specialty consultation of an endocrinologist when encountered with such cases.
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PMID:The eye as a window to rare endocrine disorders. 2262 95