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At least 19 recordsLinked to original sources

Hypothalamic disease presenting as amenorrhoea: value of computed tomography.

Five women presenting with amenorrhoea (three with secondary amenorrhoea and galactorrhoea, and two with primary amenorrhoea) were shown to have suprasellar abnormalities by computed tomography. Organic hypothalamic disease was confirmed at surgery (craniopharyngioma in three, intra- and suprasellar Rathke's cleft cyst in one, aqueductal stenosis and suprasellar arachnoid cyst in one). Moderate hyperprolactinaemia was present in the first four, while the last had isolated gonadotrophin deficiency of hypothalamic origin. One of the hyperprolactinaemic women had entirely normal residual pituitary function, visual fields and tomography of the sella. Two of the patients with secondary amenorrhoea and galactorrhoea had resumption of menses during bromocriptine therapy before operation. Spontaneous menarche and subsequent pregnancy occurred after ventriculoatrial shunt in the girl with aqueductal stenosis. Organic hypothalamic diseases may present with amenorrhoea (sometimes as the only symptom) more commonly than previously appreciated. Computed tomography should replace conventional tomography of the sella and should be an essential part of the clinical investigation in all patients with amenorrhoea of hypothalamo-pituitary origin.

Adult↗

Medical therapy of hypothalamic diseases.

Hormonal disturbances caused by hypothalamic pathology can be treated effectively by target hormone replacement in the case of failure of glandotropic hormone secretion. Hyposomatotropism in children has to be substituted by parenteral administration of growth hormone. In addition gonadotropins respectively gonadotropin releasing factor have to be given in order to restore fertility in hypothalamic hypogonadism. Posterior pituitary failure can be adequately replaced by administration of analogues of antidiuretic hormone. Hypothalamic pathology causing hypersecretion of anterior pituitary hormones may also be accessable to medical treatment. This pertains particularly to hyperprolactinemia and precocious puberty. However, there is no medical therapy so far for hypothalamic disturbances leading to veterative dysfunction like disturbances of temperature regulation and control of thirst and polyphagia. In this situation symptomatic correction of the abnormality represents the only possibility to keep these patients alive.

Autonomic Nervous System Diseases↗

Hypothalamic disease in association with dysgenesis of the corpus callosum.

We describe two cases of dysgenesis of the corpus callosum demonstrated by magnetic resonance. The first patient presented with chronic hyponatraemia. Investigation demonstrated re-setting of the osmoreceptor and thirst centres. The calculated threshold for arginine vasopressin (AVP) release was reduced at 252 mosmol/kg while severe thirst was perceived at a plasma osmolality of 260 mosmol/kg. Insulin-induced hypoglycaemia produced an exaggerated AVP response. The second patient presented with hypothermia. The calculated threshold of AVP release was 296 mosmol/kg with increased sensitivity of AVP response to hypertonic saline. The plasma AVP response to insulin-induced hypoglycaemia was absent. Both cases had normal anterior pituitary function and psychological assessment showed a similar prefrontal defect. Specific tests of callosal function were normal. These cases illustrate the importance of undertaking complete neuroradiological assessment of cases of unexplained hypothalamic disease regardless of the age of presentation to avoid overlooking this rare congenital association.

Adolescent↗

Neurologic manifestations of hypothalamic disease.

The hypothalamus, in addition to regulating the anterior and posterior pituitary, controls water balance through thirst, regulates food ingestion and body temperature, influences consciousness, sleep, emotion and other behaviors. Much has been learned of these effects in human disease through the clinical manifestations that occur with hypothalamic lesions. This study reviews the clinical pathologic correlations that have been made in recent years showing that regions of the hypothalamus exert functions in humans that are similar to those identified in experimental animals. Clinical pathologic correlations have not always provided precise analysis of hypothalamic function. The hypothalamus is small and often lesions that come to clinical attention achieve considerable size before their recognition, making local anatomic dissections of the effects of the lesions difficult. Nevertheless, the use of modern non-invasive techniques including CT scans and magnetic resonance imaging (MRI) have provided new information not previously available. This paper reviews several cases of hypothalamic disorder recognized recently. (1) A 33-year-old black man with hypothalamic sarcoidosis. Manifestations of hypothalamic dysfunction included panhypopituitarism, aggressive hyperphagia, polydipsia (partially due to hyperglycemia secondary to diabetes mellitus), drowsiness, depression, and irritability. (2) A 37-year-old woman with a large intrahypothalamic tumor (biopsy showed pituitary adenoma), with drowsiness, poikilothermia, lack of satiety, confusion, and memory loss. She becomes depressed when she is transiently more alert (as after hypertonic contrast-dye infusion). (3) A 60-year-old man with hypothalamic compression by a pituitary tumor, associated with syndrome of inappropriate ADH (SIADH), severe anorexia, memory loss, but preserved thirst. After surgical decompression of the tumor his appetite acutely recovered, but he developed severe hypo(poikilo)thermia. (4) A 45-year-old woman with a suprasellar craniopharyngioma presented with severe drowsiness, hyperphagia, depression, and memory loss post-operatively, which responded to antidepressants (except for the memory loss). She had extremely labile blood pressures and serum Na for about 1 week post-operatively.

Adult↗