1/21. A case of hypothalamic adrenal insufficiency manifested normal ACTH response to insulin-induced hypoglycemia.A low plasma ACTH response to insulin-induced hypoglycemia and an exaggerated and delayed plasma ACTH response to CRH stimulation have been considered as an indicator of hypothalamic hypopituitarism. We report a case of hypothalamic adrenal insufficiency which manifested normal ACTH response to insulin-induced hypoglycemia. This case provides important information to categorize hypothalamic adrenal insufficiency caused by abnormal regulation of CRH release.- - - - - - - - - - ranking = 1keywords = adrenal insufficiency, adrenal, insufficiency (Clic here for more details about this article) |
2/21. Hypothalamic encephalitis with bradycardia.A 74-year-old man developed fever, somnolence, hyponatremia, and life-threatening sinus bradycardia for three weeks. He showed a slight elevation of lymphocyte count and protein level in the cerebrospinal fluid. A brain CT scan revealed a diffuse low density area around the hypothalamus which was identified as a high intensity signal by flair MR imaging. Marked sinus bradycardia developed with no abnormality in the echocardiograph or cardiac enzymes. Over the next 6 weeks he became alert and normal sinus rhythm resumed. The results of endocrine tests were compatible with hypothalamic insufficiency with partial hypopituitarism and the syndrome of inappropriate secretion of ADH.- - - - - - - - - - ranking = 0.0033116590341401keywords = insufficiency (Clic here for more details about this article) |
3/21. A case of lymphocytic infundibuloneurohypophysitis associated with systemic lupus erythematosus.A 27-year-old man was admitted to our hospital with facial erythema and general malaise. He had previously suffered from orbital myositis, central diabetes insipidus (DI), peripheral neuritis, and hypogonadotropic hypogonadism. Physical and immunological examinations revealed that he was suffering from systemic lupus erythematosus (SLE). magnetic resonance imaging of the hypothalamic-pituitary region demonstrated a significant enlargement of the pituitary stalk and posterior pituitary. Endocrinological examinations showed that he had not only DI and hypogonadotropic hypogonadism but also hypoadrenalism and hypothyroidism, which were ascribed to the pituitary stalk lesion. Lymphocytic infundibuloneurohypophysitis associated with SLE was diagnosed. Administration of 30 mg/day of prednisolone for one month resulted in a marked reduction of the pituitary stalk thickening and posterior pituitary. It is recommended that a pharmacological dose of glucocorticoid be used in the treatment of lymphocytic hypophysitis patients who show significant thickening of the pituitary stalk and/or a large pituitary mass.- - - - - - - - - - ranking = 0.010154221770226keywords = adrenal (Clic here for more details about this article) |
4/21. Hypothalamic-pituitary sarcoidosis with hypopituitarism. Long-term remission with methylprednisolone pulse therapy.A 29-year-old man presented with diplopia and decreased strength in the left arm. A magnetic resonance image (MRI) showed an extensive hypothalamic and pituitary gland mass, and hormonal studies showed partial hypopituitarism and mild hyperprolactinemia without diabetes insipidus. Biopsies of the hypothalamic lesion and of a mediastinal lymph node demonstrated noncaseating granulomas, and a Kveim-Siltzbach test was positive. He was successfully treated with a regimen of high-dose intravenous methylprednisolone pulse therapy for eight weeks along with a low dose of oral corticosteroids which was maintained indefinitely. An MRI obtained immediately after pulse therapy revealed a substantial reduction in the hypothalamic-pituitary mass, which was maintained in an MRI performed 3 years later. However, hormonal deficits persisted and indefinite hormonal substitutive therapy was required.- - - - - - - - - - ranking = 5.9554581197804E-5keywords = gland (Clic here for more details about this article) |
5/21. Giant intrasellar arachnoid cyst manifesting as adrenal insufficiency due to hypothalamic dysfunction--case report--.A 67-year-old man first noticed loss of pubic and axillary hair in 1992 and then a visual field defect in 2001. He experienced loss of consciousness attributed to hyponatremia in April 2002. magnetic resonance imaging showed a giant intrasellar cystic mass, 40 mm in diameter, that had compressed the optic chiasm. The patient complained of chronic headache, and neurological examination revealed bitemporal hemianopsia. Preoperative endocrinological examination indicated adrenal insufficiency, and hypothyroidism due to hypothalamic dysfunction. The patient underwent endonasal transsphenoidal surgery. The cyst membrane was opened and serous fluid was drained. Histological examination identified the excised cyst membrane as arachnoid membrane. The patient's headaches resolved postoperatively, but the bitemporal hemianopsia and endocrinological function were unchanged. This arachnoid cyst associated with hypothalamic dysfunction might have been caused by an inflammatory episode in the suprasellar region.- - - - - - - - - - ranking = 0.83333333333333keywords = adrenal insufficiency, adrenal, insufficiency (Clic here for more details about this article) |
6/21. Hypothalamic failure as a sequela of heterozygous protein c deficiency?protein c deficiency can lead to cerebrovascular occlusive disease. We describe a patient in whom heterozygous protein c deficiency (type 1) is suspected on the grounds of reduced protein C activity and who suffered from multiple thrombo-embolic events involving the brain and peripheral organs. The patient developed hypothalamic failure with hypernatraemia, hypodipsia, hypersomnolence and hyperkapnia, obesity, hyperprolactinaemia, hypogonadotropic hypogonadism and growth hormone deficiency. We hypothesize that protein c deficiency caused cerebrovascular occlusions which eventually led to hypothalamic insufficiency in this patient. Disorders of the anticoagulant system should be looked for in patients with unexplained hypothalamic disease.- - - - - - - - - - ranking = 0.0033116590341401keywords = insufficiency (Clic here for more details about this article) |
7/21. Hypothalamic-pituitary dysfunction with adrenal insufficiency and hyperprolactinaemia in sarcoidosis. A case report.In sarcoidosis neuro-endocrine disorders are rare. A case of systemic sarcoidosis is presented in which hypothalamic-pituitary adrenal insufficiency and hyperprolactinaemia occurred simultaneously.- - - - - - - - - - ranking = 0.83333333333333keywords = adrenal insufficiency, adrenal, insufficiency (Clic here for more details about this article) |
8/21. Pituitary hyposecretion and hypersecretion produced by a Rathke's cleft cyst presenting as a noncystic hypothalamic mass.A 53-year-old man presented with polydipsia, polyuria, lightheadedness on standing, and syncope. Visual field deficits suggesting left optic tract compression and pituitary dysfunction consisting of diabetes insipidus, hyperprolactinemia, and anterior pituitary insufficiency were diagnosed. On the computed tomography scan, an isodense, ring-enhancing, parasellar mass was localized primarily in the suprasellar region and also extended into the sella. It was believed that the pituitary hormone hypersecretion and hyposecretion were due to hypothalamic dysfunction from the mass which was initially believed to be a craniopharyngioma. At surgery a Rathke's cleft cyst was resected. The unusual presentation of this Rathke's cleft cyst presenting as a hypothalamic lesion is discussed.- - - - - - - - - - ranking = 0.0033116590341401keywords = insufficiency (Clic here for more details about this article) |
9/21. Hypothalamic hypogonadism in congenital adrenal hypoplasia.Congenital adrenal hypoplasia (CAHP) in its X-linked form is associated with hypogonadotropic hypogonadism (HH). A 23 year old man with this disorder received substitution therapy with gluco- and mineralocorticoids starting one week after birth and, recently, pulsatile subcutaneous GnRH treatment via a miniature infusion pump with stepwise increasing doses from 50 to 200 ng/kg body weight/2 hours for a total of 394 days. testosterone levels increased from prepubertal levels to 409 ng/dl after 2 weeks and to 626 ng/dl after 3 months of treatment. The results of pulsatile GnRH therapy in our patient prove the hypogonadotropic hypogonadism to be of hypothalamic origin. Pulsatile GnRH substitution is a successful therapeutic regimen in patients with CAHP leading to pituitary and gonadal maturation.- - - - - - - - - - ranking = 0.050771108851129keywords = adrenal (Clic here for more details about this article) |
10/21. Suprasellar germinoma--occult presentation with hypothalamic failure 18 months before diagnosis.Hormonal evidence of widespread hypothalamic failure, including diabetes insipidus, hyperprolactinaemia, growth failure, hypothyroidism, and adrenal failure, was present in a boy of 14 years. Investigation with computerised axial tomography (CT) and metrizamide encephalography failed to identify any anatomical abnormality at the time of presentation. However, 18 months later papilloedema was noted at which time a suprasellar mass and gross dilatation of the ventricular system were readily identified using CT and a germinoma was subsequently diagnosed and treated. In the light of this experience we recommend that when "idiopathic" hypothalamic failure is diagnosed, imaging procedures, including CT or nuclear magnetic resonance examinations, should be repeated on at least one occasion, after an interval of approximately 6 months.- - - - - - - - - - ranking = 0.010154221770226keywords = adrenal (Clic here for more details about this article) |
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