Cases reported "Hyperphagia"

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1/88. pseudohypoparathyroidism--another monogenic obesity syndrome.

    obesity is a common feature of pseudohypoparathyroidism (PHP) type 1a, but is usually associated with short stature. We describe two children referred because of hyperphagia and excessive weight gain from early infancy. Tall stature in both children initially confounded the diagnosis of PHP, but on follow-up both children developed the typical hormonal abnormalities and Case 2 developed typical skeletal features of Albright hereditary osteodystrophy. PHP type 1a is caused by germline loss of function mutations in the alpha subunit of GS, the ubiquitously expressed G protein that couples many hormone receptors to the adenylate cyclase second messenger system. Recent evidence suggest that the hypothalamic GS protein coupled melanocortin-4 receptor (MC4R) may mediate the central effects of leptin on inhibition of satiety. Similar patterns of infancy onset hyperphagia, excessive weight gain and tall stature are seen in subjects with congenital leptin deficiency and in subjects with MC4R mutations. We suggest that the genetic mutations in GSalpha which underlie PHP type 1a may also directly result in severe obesity. This diagnosis should be considered in any child with a history of hyperphagia and early onset morbid obesity. ( info)

2/88. hyperphagia and self-mutilation in prader-willi syndrome: psychopharmacological issues.

    This study focused in the treatment of two major Prader-Willi symptoms: hyperphagia and self-injurious behavior (SIB). Four patients participated in a four-year study with monthly follow-ups. patients lived in a behaviorally structured environment, and were treated with selective serotonin reuptake blockers and phenothiazines. Psychopharmacological intervention improved SIB symptoms, but was ineffective to control appetite satiation. ( info)

3/88. A boy with normal growth in spite of growth hormone deficiency after resection of a suprasellar teratoma.

    We reported a boy with panhypopituitarism after removal of a suprasellar teratoma and pituitary stalk transection at the age of 3 months. His growth was accelerated after 5 years of age without growth hormone (GH) therapy, although he had poor height growth until age 4 under treatment with hydrocortisone, levothyroxine sodium, and desamino-D-arginine vasopressin (DDAVP). hyperphagia and obesity developed after surgery. Endocrinological examination revealed no GH response to glucagon, low serum levels of insulin-like growth factor (IGF)-1 and IGF binding protein-3 (IGFBP-3). serum prolactin was normal, but serum insulin was high. Some patients who received an operation for craniopharyngioma were reported to achieve normal growth without GH secretion, but the mechanism is still unknown. High serum levels of prolactin or insulin can be associated with normal IGF in GH deficient patients. This patient had obesity and high serum insulin, which may be related to growth without GH secretion. ( info)

4/88. mazindol administration improved hyperphagia after surgery for craniopharyngioma--case report.

    A 54-year-old man presented with visual disturbance and polydipsia. magnetic resonance imaging disclosed a cystic mass which extended from the intrasellar to the suprasellar region. Bifrontal craniotomy was performed and the tumor was totally removed. Histological findings confirmed the diagnosis of craniopharyngioma. Postoperatively, the patient suffered from transient disorientation. About one month after the operation the patient manifested hyperphagia and he gained 15 kg in one month. mazindol, a non-amphetaminergic anorectic agent, was administered for 3 weeks. His appetite normalized and his weight fell and stabilized even after mazindol administration was ceased. ( info)

5/88. Morbid obesity and hyperphagia in the wagr syndrome.

    A 33-year-old man with wagr syndrome is described with morbid obesity associated with hyperphagia and an apparent lack of satiety. It is possible that a gene associated with satiety is present at 11p13 although it is premature to conclude that obesity is a specific feature of wagr syndrome. ( info)

6/88. A case of kleine-levin syndrome examined with SPECT and neuropsychological testing.

    A case of kleine-levin syndrome with typical periodic hypersomnia and bulimia was diagnosed. On examination with single photo emission tomography (SPECT) (CERETEC) during a relapse period and 2 weeks later there was marked cortical hypoperfusion of the frontal and temporal lobes, especially on the left side as well as in the right parietal lobe. Neuropsychological testing performed 1 week after a relapse showed a reduction in encoding to memory function of verbal learning indicating neocortical damage of the left fronto-temporal region. A follow-up 2 months later after the patient had spontaneously recovered showed only a slight left fronto-temporal disturbance. CT and MRI of the brain were normal although the MRI showed a large and asymmetric mamillary body. Neuropsychological testing 6 years after recovery showed pronounced reduction in short-time verbal and visual memory. Seven years after recovery SPECT demonstrated a normalized frontal perfusion but still a slight hypoperfusion in the left temporal lobe. Our results correlate to autopsy findings in two cases described previously. ( info)

7/88. Efficacy of lithium treatment in kleine-levin syndrome.

    The kleine-levin syndrome (KLS) is characterized by periodic, sudden-onset episodes of hypersomnia, compulsive hyperphagia, and behavioral-emotional symptoms, lasting from a few days to a few weeks, with complete remission in the intercritical periods. We report on efficacy of lithium treatment in a highly recurring form of the disorder in a 17-year-old male adolescent. The decreasing severity of the disorder paralleled the progressive increase of lithium dosage, up to 0.9 mEq l(-1). Implications regarding the pharmacological treatment of this neglected disorder are discussed. ( info)

8/88. bulimia nervosa in hong kong Chinese patients.

    In contrast to the West, bulimic disorders are rarer than anorexia nervosa in hong kong. Four female normal-weight bulimic patients with mostly typical clinical features and conspicuous morbidity are reported. The case histories support the hypothesis that binge-eating is used to regulate unpleasant effect. ( info)

9/88. subthalamic nucleus tumor causing hyperphagia--case report.

    A 42-year-old woman with locally advanced breast cancer developed headache just after completing adjuvant chemotherapy. magnetic resonance imaging revealed a mass located in the left subthalamic nucleus (STN) and involving the posterior part of the thalamus and the hypothalamus. The patient refused a radiologically guided biopsy and gamma knife treatment was not financially possible. Palliative whole brain radiotherapy with hormonal therapy was administered. The patient gained 19 kg body weight during 4 months follow up because of hyperphagia. This solitary tumor, either a breast cancer metastasis or a primary tumor, involving the STN is extremely unusual. ( info)

10/88. kleine-levin syndrome.

    kleine-levin syndrome (KLS) is a rare disorder of uncertain etiology, characterized by recurring episodes of undue sleepiness lasting for days. We report a case of young female presenting with episodes of undue sleep along with hypersexuality and excessive food intake, who improved significantly on lithium and valproate. KLS should be considered in young patients, who present with episodes of undue somnolence. ( info)
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