1/71. Aplasia of right internal carotid artery and hypopituitarism.BACKGROUND: The pathogenesis of congenital hypopituitarism is unknown in many cases. OBJECTIVE: We report a case of congenital pan-anterior hypopituitarism in association with a complex vascular abnormality involving the central nervous system, nasal pyriform aperture stenosis, and a single central maxillary incisor. MATERIALS AND methods: MRI and MRA were used to define this patient's complex vascular anomaly. RESULTS: The vascular abnormality consists of absence of the right common carotid artery, the right internal carotid artery, the A1 segment of the right anterior cerebral artery, the anterior communicating artery, and partial absence of the M1 segment of the right middle cerebral artery. CONCLUSION: This unusual vascular anomaly may contribute to the pathogenesis of some cases of congenital hypopituitarism and related midline abnormalities, or may result from a common defect that causes pituitary insufficiency.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/71. Relapsing Whipple's disease presenting with hypopituitarism.A 44-year-old man with a history of Whipple's disease 8 years ago presented with recurrent grand mal seizures and signs of hypopituitarism on physical examination. magnetic resonance imaging of the brain revealed a hypothalamic lesion of 1 cm diameter in the region of the rostral infundibulum. hypopituitarism was confirmed by low levels of serum cortisol, free testosterone and free thyroxine without an elevated TSH. Whipple encephalitis with hypothalamic involvement was suggested and verified by positive polymerase chain reaction (PCR) for tropheryma whippelii in the cerebrospinal fluid. PCR for T. whippelii has become an important diagnostic tool for establishing the diagnosis of Whipple's disease especially in patients with unusual presentations and if the diagnosis cannot be confirmed histologically. Whipple's disease should be included in the differential diagnosis in hypopituitarism caused by infectious disease.- - - - - - - - - - ranking = 0.41394298988693keywords = brain (Clic here for more details about this article) |
3/71. Dysgenesis of the internal carotid artery associated with transsphenoidal encephalocele: a neural crest syndrome?We describe two original cases of internal carotid artery dysgenesis associated with a malformative spectrum, which includes transsphenoidal encephalocele, optic nerve coloboma, hypopituitarism, and hypertelorism. Cephalic neural crest cells migrate to various regions in the head and neck where they contribute to the development of structures as diverse as the anterior skull base, the walls of the craniofacial arteries, the forebrain, and the face. Data suggest that the link between these rare malformations is abnormal neural crest development.- - - - - - - - - - ranking = 0.41394298988693keywords = brain (Clic here for more details about this article) |
4/71. Lymphocytic hypophysitis in a patient with systemic lupus erythematosus.A case of lymphocytic hypophysitis in a patient with systemic lupus erythematosus is described. A 20-year-old woman was admitted to our hospital with generalized myalgia and facial rash in May 1998. The patient had a medical history, physical examination, and laboratory findings compatible with systemic lupus erythematosus (SLE). headache and nausea had developed 3 months previously and worsened over the following months. Hormonal investigation showed hypopituitarism except for prolactin. A magnetic resonance image of the brain showed a mass lesion in the pituitary fossa. A trans-sphenoidal surgical procedure was performed which revealed a dark-yellowish hematoma. Microscopic examination showed diffuse infiltration of lymphocytes and plasma cells with fibrosis in the anterior pituitary. Post-operatively the patient's headaches and nausea resolved. This indicates that lymphocytic hypophysitis may be associated with SLE.- - - - - - - - - - ranking = 0.41394298988693keywords = brain (Clic here for more details about this article) |
5/71. A patient of hypogonadotropic hypogonadism accompanied by growth hormone deficiency and decreased bone mineral density who attained normal growth.We present here a rare case of hypopituitarism accompanied by growth hormone (GH) deficiency and hypogonadotropic hypogonadism, in which the patient attained normal height but was of eunuchoid appearance. A 23-year-old man who had not reached puberty was referred to Saitama Medical School for hormonal evaluation. Basal hormonal data and hormone-stimulating tests revealed impaired secretion of GH, gonadotropins and adrenocorticotropic hormone (ACTH). serum levels of testosterone, estrone, estradiol and estriol were all below the detectable ranges. The patient's plasma ACTH responded to corticotropin releasing hormone, but not to insulin-induced hypoglycemia. serum GH showed a minimal response to GH-releasing hormone, but was unresponsive to insulin-induced hypoglycemia. serum luteinizing hormone and follicle stimulating hormone did not respond to luteinizing hormone-releasing hormone. The results were compatible with a diagnosis of hypothalamic hypopituitarism. Magnetic resonance images of the brain showed a small anterior pituitary, an ectopic posterior lobe and transection of the pituitary stalk. Although the patient showed signs of hypopituitarism, he finally attained normal height, possibly because of failed epiphyseal maturation. His bone mineral density was markedly reduced to 0.647 g/cm2 in the lumbar spine; this level was 61.7% of the average level of healthy young males. Our findings were compatible with a recently advocated view that estrogen is important in promoting epiphyseal fusion and in determining bone density in males as well as females.- - - - - - - - - - ranking = 0.41394298988693keywords = brain (Clic here for more details about this article) |
6/71. Basal meningoencephalocele, anomaly of optic disc and panhypopituitarism in association with moyamoya disease.Basal meningoencephalocele is frequently associated with midfacial anomaly, optic disc anomaly, brain anomaly, cerebrospinal fluid rhinorrhea, chiasma syndrome, and endocrinologic disturbance. The combination of basal meningoencephalocele and moyamoya disease is extremely rare. A 29-year-old man had basal meningoencephalocele (transsphenoidal type), anomaly of the optic disc (morning glory syndrome), panhypopituitarism and moyamoya disease. The patient was treated by hormone replacement, but surgical intervention was not required. Basal meningoencephalocele and moyamoya disease are a possible combination of the diseases.- - - - - - - - - - ranking = 0.41394298988693keywords = brain (Clic here for more details about this article) |
7/71. Hyperinsulinemic hypoglycemia associated with possible hypopituitarism in a patient with acquired immunodeficiency syndrome.OBJECTIVE: To describe endocrine dysfunctions associated with human immunodeficiency virus (hiv) infection. methods: We present a case report, discuss the laboratory findings, and suggest potential contributing factors in this complex milieu. RESULTS: A 44-year-old man infected with hiv was admitted with loss of consciousness after a grand mal seizure and was found to have a prolonged course of hypoglycemia in conjunction with increased serum levels of insulin and C peptide. Iatrogenic hypoglycemia was excluded by laboratory evaluations. Hypothalamic-pituitary dysfunction was suspected because the serum growth hormone and cortisol concentrations during hypoglycemia were low. Radiologic evaluation of the brain revealed no structural abnormalities involving the hypothalamic-pituitary area. A normal cortisol response to adrenocorticotropic hormone stimulation (cosyntropin, 250 mg) was documented on two occasions. The cause of the severe hypoglycemic episode and the possible hypothalamic-pituitary-adrenal axis dysfunction in this patient is unknown. CONCLUSION: Despite the complexity of endocrine disorders in hiv-infected patients, the course of the disease in this patient underscores the possibility that some of the endocrinologic changes may be reversible.- - - - - - - - - - ranking = 0.41394298988693keywords = brain (Clic here for more details about this article) |
8/71. Primary central nervous system lymphoma in childhood presenting as progressive panhypopituitarism.We report a 15-year-old boy who had isolated central diabetes insipidus initially diagnosed at age 11 years. A brain magnetic resonance imaging (MRI) was normal at the time. At age 12 years, growth hormone (GH) testing was performed because of a decline in linear growth rate and demonstrated GH deficiency. After a repeat normal brain MRI, GH therapy was begun. Three years later, hormonal testing revealed prepubertal gonadotropins and low testosterone levels, free thyroxine index, and morning cortisol levels. Repeat brain MRI demonstrated a 9-mm enhancing lesion in the region of the pituitary stalk. The pathologic diagnosis was that of a high-grade malignant B-cell lymphoma, suggestive of burkitt lymphoma. growth hormone therapy has not been associated with an increased incidence of lymphoma. This report underscores the need for vigilance in follow-up brain imaging and hormonal evaluation in children with diabetes insipidus, especially those with evolving anterior hormone deficiencies.- - - - - - - - - - ranking = 5.6557719595477keywords = central nervous system, brain, nervous system (Clic here for more details about this article) |
9/71. hypopituitarism in langerhans cell histiocytosis: seven cases and literature review.central nervous system (CNS) involvement and, in particular, hypothalamic-pituitary involvement are well described features of Langerhans cell histiocytosis (LCH). The actual incidence of CNS-LCH disease is unknown and the natural history is poorly understood. diabetes insipidus (DI) is reported to be the most common and well described manifestation of hypothalamic-pituitary involvement (up to 50%). Anterior pituitary dysfunction has been reported in up to 20% of patients with LCH, and occurs almost exclusively concurrently with DI. In the current paper we describe our experience with 7 patients (6 females and 1 male) in whom hypothalamicpituitary involvement was a major feature of LCH. diagnosis was made in 4 patients during childhood or adolescence, and 3 patients were over 18 years old at the time of diagnosis. Our series exemplifies the wide spectrum of LCH-induced hypopituitarism, and demonstrates some unique features, including a higher incidence of CRH/ACTH deficiency compared to other reports (4/7 patients), and massive obesity in 2 of our patients. Endocrine function was not improved in any of our patients following medical treatment of LCH with chemotherapy and glucocorticoids. We conclude that pituitary-hypothalamic dysfunction is a common feature of LCH, and therefore all LCH patients should undergo a thorough endocrine evaluation periodically.- - - - - - - - - - ranking = 0.23848036002645keywords = nervous system (Clic here for more details about this article) |
10/71. A case of hypopituitarism with diabetes insipidus and loss of thirst. role of antidiuretic hormone and angiotensin ii in the control of urine flow and osmolality.A 20-yr-old male was found to have diabetes insipidus is association with panhypopituitarism but without any focal neurological lesion being identified. He was initially treated with steroid supplements, the features of diabetes insipidus being controlled with a thiazide diuretic. Eighteen months later the patient lost thirst sensation and stopped treatment, subsequently being re-admitted with severe dehydration, oliguria and focal neurological signs. Further investigation, including brain biopsy, confirmed the presence of an atypical pinealoma which was considered inoperable. Measurements of plasma antidiuretic hormone (ADH) and angiotensin ii (AII) concentrations during the severe dehydration showed very high levels of AII, but inappropriately low plasma ADH levels for the severity of dehydration. We consider that the evidence obtained from this case supports the view that the oliguria with hypertonic urine present during severe dehydration was due to a direct renal action of the very high AII levels, possibly supplemented by the residual ADH secretion.- - - - - - - - - - ranking = 0.41394298988693keywords = brain (Clic here for more details about this article) |
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