Studies of hypothalamic function in Huntington's chorea. 1981

P J Lavin, and I Bone, and P Sheridan

In eight patients with classical Huntington's chorea hypothalamic function was assessed by the insulin tolerance test, the thyrotrophin releasing hormone test, the gonadotrophin releasing hormone test and water deprivation and the results compared with those of 10 control subjects. All patients ceased to have choreiform movements for approximately 60 minutes during the insulin tolerance test. Four of the patients failed to show clinical features of stress in response to hypoglycaemia. The fasting blood glucose level and blood glucose response to insulin were similar for the two groups. However, the response of plasma cortisol (p less than 0.05) and of growth hormone (p less than 0.05) to hypoglycaemia was earlier in patients than controls, though peak responses were the same for each group. The thyrotrophin releasing hormone test revealed no difference in basal levels of thyroid stimulating hormone in either group, or in peak response to thyrotrophin releasing hormone or in the increment at 20 minutes. One of the patients had a delayed response typical of a hypothalamic disorder, whereas none of the controls had such a response. Mean free thyroxine index levels for each group were similar. There was no difference in basal prolactin level, or in the increment or in the peak level in response to thyrotrophin releasing hormone between each group as a whole or when the males and females were analysed separately. Because of small subgroups, the data from the gonadotrophin releasing hormone test were difficult to analyse, but no clear differences or obvious abnormalities emerged. Water deprivation revealed no evidence of inability to concentrate urine in either group and hence no indication of impaired antidiuretic hormone function. The study supports previous findings of altered hypothalamic function in patients with Huntington's chorea but further suggests that serotoninergic rather than dopaminergic mechanisms may be altered.

UI MeSH Term Description Entries
D007031 Hypothalamus Ventral part of the DIENCEPHALON extending from the region of the OPTIC CHIASM to the caudal border of the MAMMILLARY BODIES and forming the inferior and lateral walls of the THIRD VENTRICLE. Lamina Terminalis,Preoptico-Hypothalamic Area,Area, Preoptico-Hypothalamic,Areas, Preoptico-Hypothalamic,Preoptico Hypothalamic Area,Preoptico-Hypothalamic Areas
D007328 Insulin A 51-amino acid pancreatic hormone that plays a major role in the regulation of glucose metabolism, directly by suppressing endogenous glucose production (GLYCOGENOLYSIS; GLUCONEOGENESIS) and indirectly by suppressing GLUCAGON secretion and LIPOLYSIS. Native insulin is a globular protein comprised of a zinc-coordinated hexamer. Each insulin monomer containing two chains, A (21 residues) and B (30 residues), linked by two disulfide bonds. Insulin is used as a drug to control insulin-dependent diabetes mellitus (DIABETES MELLITUS, TYPE 1). Iletin,Insulin A Chain,Insulin B Chain,Insulin, Regular,Novolin,Sodium Insulin,Soluble Insulin,Chain, Insulin B,Insulin, Sodium,Insulin, Soluble,Regular Insulin
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010906 Pituitary Hormone-Releasing Hormones Peptides, natural or synthetic, that stimulate the release of PITUITARY HORMONES. They were first isolated from the extracts of the HYPOTHALAMUS; MEDIAN EMINENCE; PITUITARY STALK; and NEUROHYPOPHYSIS. In addition, some hypophysiotropic hormones control pituitary cell differentiation, cell proliferation, and hormone synthesis. Some can act on more than one pituitary hormone. Hormones, Pituitary Hormone Releasing,Hypophysiotropic Hormones,Hypothalamic Hypophysiotropic Hormone,Hypothalamic Releasing Factor,Hypothalamic Releasing Hormone,Hypothalamic Releasing Hormones,Hormone, Hypothalamic Hypophysiotropic,Hormones, Hypophysiotropic,Hypophysiotropic Hormone, Hypothalamic,Pituitary Hormone Releasing Hormones,Releasing Hormone, Hypothalamic
D005260 Female Females
D006062 Gonadotropins Hormones that stimulate gonadal functions such as GAMETOGENESIS and sex steroid hormone production in the OVARY and the TESTIS. Major gonadotropins are glycoproteins produced primarily by the adenohypophysis (GONADOTROPINS, PITUITARY) and the placenta (CHORIONIC GONADOTROPIN). In some species, pituitary PROLACTIN and PLACENTAL LACTOGEN exert some luteotropic activities. Gonadotropin
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006816 Huntington Disease A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4) Huntington Chorea,Juvenile Huntington Disease,Akinetic-Rigid Variant of Huntington Disease,Chorea, Chronic Progressive Hereditary (Huntington),Chronic Progressive Hereditary Chorea (Huntington),Huntington Chronic Progressive Hereditary Chorea,Huntington Disease, Akinetic-Rigid Variant,Huntington Disease, Juvenile,Huntington Disease, Juvenile-Onset,Huntington Disease, Late Onset,Huntington's Chorea,Huntington's Disease,Juvenile-Onset Huntington Disease,Late-Onset Huntington Disease,Progressive Chorea, Chronic Hereditary (Huntington),Progressive Chorea, Hereditary, Chronic (Huntington),Akinetic Rigid Variant of Huntington Disease,Chorea, Huntington,Chorea, Huntington's,Huntington Disease, Akinetic Rigid Variant,Huntington Disease, Juvenile Onset,Huntington Disease, Late-Onset,Juvenile Onset Huntington Disease,Late Onset Huntington Disease
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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