S u m m ar y in high eoneentrations, it is relatively low in eoneen-Effect of intravenous infusion of 100 mg of L-Dopa on plas-tration in the median eminenee (Laverty and Sharman ma human growth hormone (HGH) levels was studied in nor-1965). On the eontrary, it was reported that the eonmal subjects and patients with certain central nervous system tent of dopamine is very high in the median eminence diseases. Intravenous infusion of L-Dopa caused a rise in (Laverty and Sharman 1965). Schneider and McCann plasma HGH in most of the normal subjects tested. with peak HGH levels of 15.5 ± 6.1 mpgJml. Plasma HGH rose (1969) suggested the possible role of dopamine in resignificantly during the infusion of L-Dopa in most of the gulating release of luteinizing hormone-releasing faepatients with Parkinson's disease, other degenerative central tor, from their in vitro experiment. L-Dopa, unlike nervous system diseases and anorexia nervosa, although ap-exogenous dopamine, erosses the blood brain barrier parently less marked than in normal subjects. Oral admin-d istration of glucose did not affect the magnitude of HGH where it is believed to be eonverted to opamine. response to L-Dopa but delayed the peak of plasma HGH The present study is designed to investigate the efslightly. Combined administration 01" propranolol, a beta feet of the intravenous infusion of L-Dopa on plasadrenergic blocking agent, significantly enhanced plasma HGH ma HGH levels. response to L-Dopa, whereas phentolamme, an alpha blocking agent, had no signiticant elleCL Materials and Methods

A 19-year-old man was admitted to our hospital for delayed puberty. At birth, he had macrocephalia and showed delayed physical and mental development. At 9 years of age, right cryptorchism was diagnosed. His parents had noticed that he could not recognize any smells since his infancy. Physical examination on admission revealed ocular hypertelorism, high myopia, high arched palate, and intermittent external strabismus. Sense of smell was scaled out by olfactometry. External genitalia were infantile. Neurological examination showed on IQ of 83, and mild truncal ataxia. Magnetic resonance imaging (MRI) showed a cystic distension of the IV ventricle, partial aplasia of the cerebellar vermis, elevation of the tentorium cerebelli, enlargement of the III ventricle, and agenesis of the corpus callosum. These findings revealed that the patient had Dandy-Walker malformation. The basal FSH, LH, and testosterone levels were all low compared with normal adult reference values. The serial LH-RH provocation tests showed stepwise LH and FSH elevation. After the fifth day of LH-RH administration, both LH and FSH responses clearly improved. Olfactory tracts were defective in MRI findings. These findings were consistent with hypogonadotropic hypogonadism of hypothalamic origin with anosmia, and the patient was therefore diagnosed with Kallmann syndrome. Sequence analysis of the KAL1 gene showed no mutation in the coding region. To our knowledge, this is the first case report of the coexistence of Kallmann syndrome and Dandy-Walker malformation in the same patient.

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Release of plasma ACTH- and beta-endorphin (beta-EP)-like immunoreactivity (LI) was studied in vivo in a patient with an ectopic ACTH-producing malignant thymoma. Administration of lysin vasopressin stimulated concomitant release of plasma ACTH- and beta-EP-LI. Administration of cyproheptadine, naloxone, and somatostatin significantly suppressed plasma levels of ACTH- and beta-EP-LI, while saline infusion did not. Gel exclusion chromatography of the plasma extracts revealed that ACTH-LI consisted of two components, large and small molecular weight form, while beta-EP-LI consisted of three components, large molecular weight, beta-lipotropin-, and beta-EP-sized form; each of these components was incompletely suppressed by somatostatin infusion. It is suggested that certain tumors may have acquired aberrant multiple receptors during malignant transformation which may lead to the paradoxical hormone response as demonstrated in this case.

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