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      A Perplexing Case of the Pituitary Apoplexy

      , ,
      Canadian Journal of General Internal Medicine
      Dougmar Publishing Group, Inc.

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          Abstract

          We report an unusual clinical presentation of adrenal insufficiency. A 54-year-old post-menopausal woman with well-controlled hypertension presented with 1 month of progressive anorexia and intermittent low-grade fever. She underwent extensive initial investigations that included computed tomography (CT) of the head, chest, and abdomen; gastroscopy; infectious and autoimmune workups; all of which were unrevealing. The head CT did not demonstrate any hyperintense or space-occupying lesion. When she represented to hospital, we noticed an empty sella on the initial CT. This prompted a magnetic resonance imaging study that revealed pituitary apoplexy, leading to eventual diagnosis of secondary adrenal insufficiency (morning cortisol 81 nmol/L and ACTH 1 pmol/L). Her symptoms resolved within 24 hours of glucocorticoid replacement. Diagnosis was elusive due to patient’s atypical presentation – she did not have visual symptoms or cranial nerve deficits to suggest pituitary apoplexy, and also did not have any objective findings such as hypotension or electrolyte abnormalities to suggest adrenal insufficiency. RÉSUMÉ Nous rapportons ici un tableau clinique inhabituel en matière d’insuffisance surrénale. Une femme de 54 ans, ménopausée, dont l’hypertension est bien contrôlée, souffre depuis un mois d’anorexie progressive et d’une fièvre légère intermittente. Elle a subi de nombreux examens, notamment de tomodensitométrie (TDM) de la tête, du thorax et de l’abdomen; une gastroscopie; ainsi que des investigations techniques sur les plans infectieux et auto-immuns. Tous ces examens ont été vains. La TDM de la tête n’a montré aucune lésion hyperintense ou étendue. Lorsque la patiente s’est présentée de nouveau à l’hôpital, une relecture de la TDM initiale indiqua une selle turcique vide. Cela nous a incité à effectuer un examen d’imagerie par résonnance magnétique, qui révéla une apoplexie hypophysaire et nous mena finalement à un diagnostic d’insuffisance surrénale secondaire (taux de cortisol matinal de 81 nmol/L et ACTH de 1 pmol/L). Les symptômes se sont résorbés dans les 24 heures qui suivirent l’administration de glucocorticoïdes. C’est en raison du tableau atypique des symptômes de la patiente que le diagnostic a été difficile à cerner – il y avait absence de symptômes au niveau des yeux et absence de déficits au niveau des nerfs crâniens, ce qui aurait pu indiquer une apoplexie hypophysaire. De plus, il y avait absence de signe objectif comme de l’hypotension ou des anomalies électrolytiques indiquant une insuffisance surrénale.  

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          Most cited references14

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          Classical pituitary apoplexy: clinical features, management and outcome.

          The term classical pituitary apoplexy describes a clinical syndrome characterized by sudden headache, vomiting, visual impairment and meningismus caused by the rapid enlargement of a pituitary adenoma usually due to haemorrhagic infarction of the tumour. Most published reports looking at the clinical features and management of pituitary apoplexy have not differentiated between patients with clinical and subclinical apoplexy, the latter diagnosed at surgery. Furthermore, little is reported on the clinical outcome, in particular visual and endocrinological, and the role of radiotherapy. The purpose of this study was to observe not only the clinical presentation but also the possible predisposing events, investigations, management, clinical outcome as well as the role of radiotherapy in patients presenting with classical pituitary apoplexy. In a retrospective analysis 1985-96, the medical records of 21 male and 14 female patients (mean age 49.8 years, range 30-74) with classical pituitary apoplexy were reviewed. This represents all patients seen with this condition over the stated period. In all patients, pre- and post- operative measurements were made of FT4, FT3, TSH, PRL, LH, FSH, cortisol (0900 h), GH, oestradiol (females) and testosterone (males). Pituitary imaging was by computerized tomography (CT) scan, magnetic resonance imaging (MRI) or both. Patients were followed for up to 11 years (mean 6.3 years: range 0.5-11). Headache (97%) was the commonest presenting symptom, followed by nausea (80%) and a reduction of visual fields (71%). Hypertension, defined as a systolic > 160 mmHg and/or a diastolic > 90 mmHg, was seen in 26% of patients. MRI correctly identified pituitary haemorrhage in 88% (n = 7), but CT scanning identified haemorrhage in only 21% (n = 6). By immunostaining criteria, null-cell adenomas were the most common tumour type (61%). Transsphenoidal surgery resulted in improvement in visual acuity in 86%. Complete restoration of visual acuity occurred in all patients operated on within 8 days but only in 46% of patients operated on after this time (9-34 days). Long-term steroid or thyroid hormone replacement was necessary in 58% and 45% of patients, respectively. Of the male patients, 43% required testosterone replacement, and long-term desmopressin therapy was required in 6%. Only two patients (6%) with tumour recurrence after transsphenoidal surgery for the initial apoplectic event, subsequently required radiotherapy. In classical pituitary apoplexy, headache is the commonest presenting symptom and hypertension may be an important predisposing factor. MRI is the imaging method of choice. Transsphenoidal surgery is safe and effective. It is indicated if there are associated abnormalities of visual acuity or visual fields because, when performed within 8 days, it resulted in significantly greater improvement in visual acuity and fields than if surgery was performed after this time. Radiotherapy is not indicated immediately as the risk of tumour recurrence is small, but careful follow-up initially with annual imaging is indicated in this group.
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            Mortality in patients with pituitary disease.

            Pituitary disease is associated with increased mortality predominantly due to vascular disease. Control of cortisol secretion and GH hypersecretion (and cardiovascular risk factor reduction) is key in the reduction of mortality in patients with Cushing's disease and acromegaly, retrospectively. For patients with acromegaly, the role of IGF-I is less clear-cut. Confounding pituitary hormone deficiencies such as gonadotropins and particularly ACTH deficiency (with higher doses of hydrocortisone replacement) may have a detrimental effect on outcome in patients with pituitary disease. Pituitary radiotherapy is a further factor that has been associated with increased mortality (particularly cerebrovascular). Although standardized mortality ratios in pituitary disease are falling due to improved treatment, mortality for many conditions are still elevated above that of the general population, and therefore further measures are needed. Craniopharyngioma patients have a particularly increased risk of mortality as a result of the tumor itself and treatment to control tumor growth; this is a key area for future research in order to optimize the outcome for these patients.
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              UK guidelines for the management of pituitary apoplexy.

              Classical pituitary apoplexy is a medical emergency and rapid replacement with hydrocortisone maybe life saving. It is a clinical syndrome characterized by the sudden onset of headache, vomiting, visual impairment and decreased consciousness caused by haemorrhage and/or infarction of the pituitary gland. It is associated with the sudden onset of headache accompanied or not by neurological symptoms involving the second, third, fourth and sixth cranial nerves. If diagnosed patients should be referred to a multidisciplinary team comprising, amongst others, a neurosurgeon and an endocrinologist. Apart from patients with worsening neurological symptoms in whom surgery is indicated, it is unclear currently for the majority of patients whether conservative or surgical management carries the best outcome. Post apoplexy, there needs to be careful monitoring for recurrence of tumour growth. It is suggested that further trials be carried out into the management of pituitary apoplexy to optimize treatment. © 2010 Blackwell Publishing Ltd.
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                Author and article information

                Journal
                Canadian Journal of General Internal Medicine
                Can Journ Gen Int Med
                Dougmar Publishing Group, Inc.
                2369-1778
                1911-1606
                January 02 2018
                January 02 2018
                : 12
                : 4
                Article
                10.22374/cjgim.v12i4.174
                73a6a562-2b6d-4382-8697-8bcf2e5bfffc
                © 2018

                Copyright of articles published in all DPG titles is retained by the author. The author grants DPG the rights to publish the article and identify itself as the original publisher. The author grants DPG exclusive commercial rights to the article. The author grants any non-commercial third party the rights to use the article freely provided original author(s) and citation details are cited. To view a copy of this license, visit https://creativecommons.org/licenses/by-nc/4.0/


                General medicine,Geriatric medicine,Neurology,Internal medicine
                General medicine, Geriatric medicine, Neurology, Internal medicine

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