1/9. Langerhans cell histiocytosis limited to the pituitary-hypothalamic axis--two case reports.Langerhans cell histiocytosis rarely presents as a solitary lesion in the pituitary-hypothalamic region, and is indistinguishable from germinoma, which occurs much more frequently, especially in Japanese. A 14-year-old girl and a 9-year-old girl presented with polydipsia and polyuria as the initial symptoms. Magnetic resonance (MR) imaging demonstrated a round mass at the pituitary stalk appearing as isointense on T1-weighted imaging and hyperintense on T2-weighted imaging. Endocrinological examination revealed mild hypopituitarism with central diabetes insipidus. Both patients underwent open craniotomy. Histological examination revealed granulomatous tissue with eosinophil infiltration and frequent Langerhans histiocyte clustering, compatible with the diagnosis of Langerhans cell histiocytosis. Low-dose local irradiation of 20 Gy was administered. First patient was followed up for 8 years, and her hypopituitarism gradually improved to a minimal level with only amenorrhea as the residual symptom. Recent MR imaging showed no residual mass at the region. Second patient was followed up for 15 months, and her diabetes insipidus is stable. MR imaging performed 5 months after the treatment showed marked reduction of the mass. These cases reemphasize the importance of histological diagnosis for lesions with similar neuroimaging appearances. biopsy and low-dose irradiation are an effective treatment for this rare and essentially benign lesion, as opposed to attempting total removal of the mass.- - - - - - - - - - ranking = 1keywords = amenorrhea (Clic here for more details about this article) |
2/9. Sudden unexpected death resulting from hypothalamic sarcoidosis.The authors report a case of sudden death in a 23-year-old woman in whom autopsy by the medical examiner revealed hypothalamic sarcoidosis. The decedent had experienced 6 months of amenorrhea and a 50-pound weight gain. She sought medical attention when she experienced headache, and after imaging and laboratory studies, she was suspected of having neurosarcoidosis. She appeared to respond well to corticosteroid therapy. However, 6 weeks after the beginning of therapy, she was found dead on the floor of her residence. autopsy showed extensive granulomatous inflammation of the hypothalamus and adjacent structures. Previously reported cases of hypothalamic sarcoidosis are reviewed. A hypothesis for the mechanism of sudden death is presented.- - - - - - - - - - ranking = 1keywords = amenorrhea (Clic here for more details about this article) |
3/9. Hypothalamic amenorrhea in a case of mitochondrial encephalomyopathy.A 26-year-old female with myoclonus epilepsy associated with ragged-red fibers is reported. Clinically, she had myoclonus epilepsy, cerebellar ataxia, a bilateral neurosensory type hearing loss, retinitis pigmentosa and short stature. She also presented with primary amenorrhea and poor development of secondary sexual characteristics. Endocrinologic studies revealed that hypothalamic dysfunction was the most plausible cause of her primary amenorrhea. magnetic resonance imaging showed marked dilatation of the third ventricle indicating thalamic and hypothalamic degeneration. We conclude that hypothalamic dysfunction may be one of the characteristic features of mitochondrial encephalomyopathies.- - - - - - - - - - ranking = 6keywords = amenorrhea (Clic here for more details about this article) |
4/9. Cyclical edema in a patient with hypothalamic disorders and chronic glomerulonephritis: arginine vasopressin-dependent atrial natriuretic hormone release.A 28-year-old woman had hypothalamic disorders (amenorrhea, obesity, psychiatric abnormalities, polydipsia and fever) and chronic glomerulonephritis. She also suffered from general edema associated with cyclical oliguria and polyuria. Her body weight and plasma osmolality increased during the oliguria phase lasting 2 to 8 days and decreased after paroxysmal polyuria accompanied by the natriuresis. These episodes occurred repeatedly, regardless of the treatment with or without diuretics. The release of arginine vasopressin in response to increased plasma osmolality was exaggerated, but changes in plasma volume did not affect arginine vasopressin release. Plasma atrial natriuretic hormone increased in response to a rise in plasma arginine vasopressin and plasma volume during the oliguria phase, thereby resulting in the diuresis and natriuresis. The renin-angiotensin-aldosterone system was secondarily activated by body fluid depletion and diuretics, and this might play an additive role in general swelling. Plasma gonadal hormones did not change to explain the edema. The mechanism of this cyclical edema remains unknown, but it is likely that hypothalamic dysfunction related to psychiatric abnormalities may exaggerate arginine vasopressin release, and enhanced renal sympathetic activity may cause retention of Na and water, and the increase in atrial natriuretic hormone release responding to the plasma volume expansion may bring about the diuresis and natriuresis.- - - - - - - - - - ranking = 1keywords = amenorrhea (Clic here for more details about this article) |
5/9. An X;9 translocation, primary amenorrhea, and hypothalamic dysfunction.A white girl presented at age 16 yr with delayed puberty and primary amenorrhea. She had 46 chromosomes with a de novo reciprocal X;9 translocation. The normal X chromosome was found to be heterochromatic, thus preserving the function of the translocation portion of the 9. Her total estrogen and serum estradiol levels were low and her serum follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels were at the lower end of the normal adult range. She had a reasonably good FSH and LH response to GnRH, but an inadequate response to 100 mg of clomiphene daily for 1 wk. This would suggest that the abnormality of function is probably hypothalamic, a hitherto unreported association. De novo translocations between X chromosomes and autosomes are rare and none identical to this case has been described. The breakpoint of the x chromosome was at p22, well outside the "critical region" for female reproductive function. It seems probable that her chromosome abnormality is responsible for her clinical state.- - - - - - - - - - ranking = 5keywords = amenorrhea (Clic here for more details about this article) |
6/9. Neuroendocrinological aspects of histiocytosis X of the central nervous system.Detailed neuroendocrine studies were carried out in a patient with histiocytosis X involving the hypothalamus. She presented with amenorrhea, galactorrhea, and diabetes insipidus. The diagnosis was established through needle biopsy with the aid of a computerized tomography (CT) scanner. The basic pathology and clinical features of the disease are outlined, with particular emphasis on the endocrine abnormalities seen in isolated hypothalamic histiocytosis X.- - - - - - - - - - ranking = 1keywords = amenorrhea (Clic here for more details about this article) |
7/9. A case of idiopathic hypothalamic hypothyroidism.The availability of thyrotropin hormone (TRH) has made it possible to determine whether tropic hormone deficiency is caused by pituitary or hypothalamic dysfunction. A case of hypothalamic hypothyroidism was described ina a 17 year old woman. This patient was admitted for the evaluation of hypothyroidism and secondary amenorrhea. Her T3 and T4 were decreased, with an undetectable level of base line thyrotropin. the TRH test revealed normal but delayed response of TSH. Her base line prolactin and its response to TRH were normal. Adenocorticotropic hormone (ACTH), cortisol, growth hormone (GH), and urinary 17-hydroxysteroids were also normal. ACTH response to metyrapone was normal. Evaluation of the pituitary-gonadal axis revealed a normal increase in both lutenizing hormone (LH) and follicle stimulating hormone (FSH) following the intravenous administration of lutenizing hormone releasing hormone (LHRH). These results suggest that she had hypothalamic hypothyroidism as an isolated disturbance in the hypothalamic-pituitary axis. A deficiency of TRH is probably caused by a disorder of hypothalamic function of unknown etiology since the extensive studies did not reveal any secondary causes. It is recommended that patients with amenorrhea and hypothyroidism be evaluated for possible hypothalamic hypothyroidism.- - - - - - - - - - ranking = 2keywords = amenorrhea (Clic here for more details about this article) |
8/9. Hypothalamic hypogonadism in myotonic dystrophy.Hypothalamic-pituitary-ovarian axis function was assessed in a postpubertal female patient with myotonic dystrophy and secondary amenorrhea. The results suggested a hypothalamic basis for the amenorrhea, confirming previous reports regarding the nature of gonadal failure in women with this multisystemic disorder.- - - - - - - - - - ranking = 2keywords = amenorrhea (Clic here for more details about this article) |
9/9. Neurosarcoidosis presenting as secondary amenorrhea in a teenager.sarcoidosis is a systemic inflammatory disease of unknown etiology characterized by non-caseating epithelioid cell granulomata. The lungs and reticulo-endothelial system are typically involved, and virtually any organ system may be affected. sarcoidosis involving the central nervous system is relatively uncommon, estimated to occur in approximately 5% of patients with sarcoidosis in the united states, while the incidence throughout the world may be as high as 15%. Hypothalamic dysfunction is the most common manifestation of central nervous system parenchymatous disease in neurosarcoidosis. polyuria and polydipsia are the most frequently occurring symptoms in patients with sarcoidosis who have dysfunction of the pituitary and hypothalamus. We describe a patient with secondary amenorrhea resulting from neurosarcoidosis involving the pituitary and hypothalamus.- - - - - - - - - - ranking = 5keywords = amenorrhea (Clic here for more details about this article) |