To classify the causes of hypogonadotropic hypogonadism in Korean patients, and to improve the endocrinologic evaluation for the disease, we retrospectively studied the clinical findings and result of combined pituitary stimulation test in 35 patients with hypogonadotropic hypogonadism. The following results were obtained.1) The ratio of male to female was 1.3:1, and the 50% of male patients was under 20 years of age and the 20% of female patients in 30th decades. 2) The chief complaints of male patients on the admission were the failure of secondary sexual characteristics(95.0%) and loss of hair(5.0%), those of female patients were amenorrhea(46.7%), infertility(26.7%), failure of secondary characteristics(13.3%) and loss of hair(13.3%). 3) The causes of male hypogonadotropic hypogonadism were craniopharyngioma(35.0%), idiopathic(30.0%), Kallmann's syndrome(15.0%), pituitary adenoma(10.0%) and germinoma(5.0%), and those of female hypogonadotropic hypogonadism were prolactinoma(13.3%), Sheehan's syndrome(26.6%), pituitary adenoma(6.7%), tuberculous granuloma(6.7%), germinoma(6.7%), idiopathic hypogonadotropic hypogonadism(40.0%).4) The responses of LH and FSH to GnRH test were absent or markedly blunted in diffuse pituitary diseases such as pituitary tuberculous granuloma, pituitary macroadenomas, Sheehan's syndrome. However those were also absent or blunted in Cushing's disease and hypothalamic disease such as Kallmann's syndrome, germinoma, craniopharyngioma, idiopathic hypogonadotropic hypogonadism. 5) The responses of LH, FSH increased after repeated injection of GnRH in a patient with germinoma. 6) In diffuse destructive pituitary diseases such as Sheehan's syndrome, nonfunctioning macroadenomas, tuberculous granuloma, large prolactinoma, the combined deficiency of pituitary hormones other than gonadotropins was observed. 7) In many cases with hypothalamic diseases, the combined defects of pituitary hormone response were also seen.These data suggest that GnRH test is not always useful to localize the lesion between pituitary and hypothalamus, and combined pituitary stimulation test revealed defects of pituitary hormones other than gonadotropin in various hypothalamic diseases.Therefore repeated GnRH test would be useful for the differential diagnosis, and CRH test and GRH test would be necessary to demonstrate whether pituitary abnormality is present.
Craniopharyngioma ; Diagnosis, Differential ; Female ; Germinoma ; Gonadotropin-Releasing Hormone ; Gonadotropins ; Granuloma ; Humans ; Hypogonadism ; Hypopituitarism ; Hypothalamic Diseases ; Hypothalamus ; Kallmann Syndrome ; Male ; Pituitary Diseases ; Pituitary Hormones ; Prolactinoma ; Retrospective Studies

OBJECTIVE: Pituitary apoplexy is a rare clinical syndrome caused by pituitary hemorrhage, hemorrhagic infarction, or ischemic infarction within a pituitary tumor or surrounding structure. We analyzed surgical outcomes of pituitary apoplexy. METHODS: From 1995 to 2004, we reviewed our experience of 29 cases with pituitary apoplexy. In all patients, pre- and postoperative clinical presentation were checked and endocrine study were performed. RESULTS: The most frequent symptoms were visual disturbance (24 cases, 82.8%) and headache (22 cases, 75.9%). After surgery, headache improved in 86.4%, 88.9% among 18cases who had preoperative reduction in visual acuity and 75.0% among 12 cases who had preoperative reduction in visual field improved. In endocrine study, long-term steroid and thyroid hormone replacement therapy was necessary in 42.9% of 14 cases presenting preoperative hypopituitarism. Postoperative transient hypopituitarism developed in 5 cases (33.3%) and they all recovered in follow up study. Postoperative endocrinological recovery were in 77.9% of 9 cases with preoperative prolactinoma, 1 case in 2 cases with acromegaly and one case with Cushing disease. Postoperative complications were diabetes inspidus(DI) in 1 case (3.4%), cerebrospinal fluid (CSF) leakage in 2 cases (6.8%) and death in 1 case (3.4%) due to sepsis. CONCLUSION: We report good results through surgery of pituitary apoplexy in a clinical and endocrine outcomes. The surgery should be performed as soon as possible to be a suitable method for treating pituitary apoplexy.
Acromegaly ; Cerebrospinal Fluid ; Follow-Up Studies ; Headache ; Hemorrhage ; Hormone Replacement Therapy ; Humans ; Hypopituitarism ; Infarction ; Pituitary ACTH Hypersecretion ; Pituitary Apoplexy* ; Pituitary Neoplasms ; Postoperative Complications ; Prolactinoma ; Sepsis ; Thyroid Gland ; Visual Acuity ; Visual Fields

Brain Neoplasms ; complications ; Humans ; Hypothalamic Diseases ; etiology ; Syndrome

Postpartum ischemic necrosis of the pituitary gland, known as Sheehan's syndrome, is well-established clinical entity. The recent progress in radiological imaging allows an easy and noninvasive study of the pituitary area in the patients while still alive. An empty or partially empty sella is a constant feature of Sheehan's syndrome in the later phase. We report a case of nonhemorrhagic postpartum pituitary infarction documented in the acute phase with clinical, endocrine, and sequential magnetic resonance (MR) imaging studies.
Humans ; Hypopituitarism* ; Infarction ; Necrosis ; Pituitary Apoplexy* ; Pituitary Gland ; Pituitary Neoplasms* ; Postpartum Period

The occurrence of symptomatic pituitary hemorrhage into a Rathke's cleft cyst (RCC) is extremely rare. The author reports an interesting case of intra- and suprasellar RCC presented with features of pituitary apoplexy. This 62-year-old woman suffered acute headache, mental confusion, and partial hypopituitarism. The characteristics of the magnetic resonance imaging seemed most compatible with a hemorrhagic pituitary adenoma. Transsphenoidal drainage of the cyst contents confirmed the diagnosis of hemorrhagic RCC and resolved the symptoms. All published data on this rare clinical entity are extracted and reviewed.
Drainage ; Female ; Headache ; Hemorrhage ; Humans ; Hypopituitarism ; Magnetic Resonance Imaging ; Middle Aged ; Pituitary Apoplexy ; Pituitary Neoplasms ; Stroke

Empty sella is defined as a sella which, regardless of its size, is completely or partly filled with cerebrospinal fluid (CSF), and is divided into two groups, primary or secondary. Secondary empty sella syndrome is associated with a prior surgery, radiation therapy or medical treatment of pituitary gland, and otherwise primary. The endocrine function of primary empty sella syndrome is usually normal, but sometimes, this syndrome is associated with complete or partial pituitary insufficiency and rarely pituitary hypersecretion. In the cases of hypersecretion of pituitary hormones, hyperprolactinemia, acromegaly and hypersecretion of adrenocorticotrophin (ACTH) has been reported. but, hypersecretion of ACTH is rarely reported. We describe a patient of primary empty sella syndrome combined with Cushing's disease. So we present this case with a review of the literature.
Acromegaly ; Adrenocorticotropic Hormone ; Cerebrospinal Fluid ; Cushing Syndrome ; Empty Sella Syndrome* ; Humans ; Hyperprolactinemia ; Hypopituitarism ; Pituitary Gland ; Pituitary Hormones

The central nervous system (CNS) has important roles in the regulation of sodium and water homeostasis, so the risk of hyponatremia is increased for patients with neurological disorders. Cerebral salt wasting (CSW) is a syndrome that is characterized by hyponatremia and extracellular volume contraction, and these problems are caused by natriuresis and the concomitant polyuria in patient with intracranial disease. It is critical to differentiate CSW from syndrome of inappropriate secretion of antidiuretic hormone (SIADH) for patients with hyponatremia and after they experience cerebral insults. This is because fluid restriction is the treatment of choice for SIADH and administration of isotonic or hypertonic saline is important for treating CSW. We report here on a case of CSW following trassphenoidal surgery for pituitary tumor in a 67-year-old man with nonfunctioning pituitary macroadenoma. Hyposmotic hyponatremia, increased urine output and signs of dehydration developed postoperatively, and the patient was managed by water and salt supplementation.
Aged ; Central Nervous System ; Dehydration ; Homeostasis ; Humans ; Hyponatremia ; Inappropriate ADH Syndrome ; Natriuresis ; Nervous System Diseases ; Pituitary Neoplasms ; Polyuria ; Sodium

Ovarian hyperstimulation syndrome (OHSS) is one of the most serious complication of controlled ovarian hyperstimulation (COH) and ovulation induction. High serum estradiol concentration can induce the increase of capillary permeability and fluid shift, and it can results in hypovolemia, hemoconcetration, thromboembolism, oliguria, ascites and even death. This physiologic changes of OHSS and/or pregnancy might induce the deterioration of pituitary function in patients with abnormal pituitary conditions such as low pituitary reserve or tumor. However, all this while, panhypopituitarism or pituitary apoplexy in a pregnant woman with OHSS and/or pituitary adenoma has not been reported. We have experienced a case of panhypopituitarism developed in a pregnant woman with severe OHSS and residual pituitary adenoma. She had residual prolactinoma, despite a transsphenoidal adenoidectomy, and had suffered from uncontrolled hyperprolactinemia (HPRL) and chronic anovulation. Therefore, she had taken ovulation induction for getting a pregnancy and conceived, but, in the conception cycle, she was hospitalized for management of severe OHSS and hyperemesis gravidarum. During the period of hospitalization, abrupt panhypopituitarism was developed unexpectedly. Therefore, we present this case with the brief review of literature.
Adenoidectomy ; Anovulation ; Ascites ; Capillary Permeability ; Estradiol ; Female ; Fertilization ; Hospitalization ; Humans ; Hyperemesis Gravidarum ; Hyperprolactinemia ; Hypovolemia ; Oliguria ; Ovarian Hyperstimulation Syndrome* ; Ovulation Induction ; Pituitary Apoplexy ; Pituitary Neoplasms* ; Pregnancy ; Pregnant Women* ; Prolactinoma ; Thromboembolism

OBJECTIVE: Pituitary apoplexy, resulting from an acute infarction or hemorrhage mainly in pituitary adenomas, is a rare yet major clinical event with neurological, ophthalmological and hormonal emergent consequences. The authors review our surgical experience with a series of 16 cases of pituitary apoplexy. METHODS: The cases of pituitary apoplexy, operated via trans-sphenoidal approach in our hospital between 1998-2005, were retrospectively analyzed in terms of their clinicoradiological features, pathological findings and surgical outcomes. RESULTS: The mean age of patients (9 male and 7 female) was 47.1 years. The average time of presentation after onset of symptom was 8.9 days. Pituitary apoplexy occurred as an initial manifestation of pituitary adenoma in all patients. Headache was the most common presenting symptom (94%). Visual disturbance was found in 56% of patients. Thirty-one percent of the patients had hypopituitarism. On magnetic resonance imaging, this entire catastrophic event accompanied with macroadenoma in a mean size of 22.5 mm. Only four patients needed postoperative hormone replacement therapy. Three of them showed preoperative hypopituitary function, and one patient in normal pituitary function. There was no specific complication in any of these patients. CONCLUSION: Early trans-sphenoidal decompression with high-dose corticosteroid replacement showed good outcomes of pituitary apoplexy.
Decompression ; Headache ; Hemorrhage ; Hormone Replacement Therapy ; Humans ; Hypopituitarism ; Infarction ; Magnetic Resonance Imaging ; Male ; Pituitary Apoplexy* ; Pituitary Neoplasms ; Retrospective Studies

Rathke cleft cysts (RCCs) are benign epithelial lesions of the sellar and suprasellar region. Most RCCs remain clinically silent throughout an individual's life. Symptomatic patients with RCCs manifest headaches, endocrinopathies, and visual disturbances secondary to parasellar extension. Rarely, RCCs can present in a manner similar to pituitary tumor apoplexy. One such case is reported herein. This 53-year-old man presented with acute headache, vomiting, and partial hypopituitarism. Surgical drainage of the cyst contents via a transnasal route confirmed the diagnosis of RCC and pituitary infarction.
Central Nervous System Cysts ; Diagnosis ; Drainage ; Headache ; Humans ; Hypopituitarism ; Infarction ; Middle Aged ; Pituitary Apoplexy ; Pituitary Neoplasms ; Stroke* ; Vomiting