A syndrome of periodic hypothalamic discharge

doi:10.1016/0002-9343(64)90123-8

The American Journal of Medicine

Volume 36, Issue 6, June 1964, Pages 956-967

https://doi.org/10.1016/0002-9343(64)90123-8 Get rights and content

Abstract

A fourteen year old boy is described who has had cyclical manifestations of nausea, vomiting, fever, emotional disturbance and marked weight change. Associated findings during the attacks were facial plethora, hypertension, abnormal glucose tolerance, elevated plasma ACTH and adrenal hyperfunction. The results of roentgenologic and physiologic studies are compatible with a lesion of the central nervous system, with periodic release of ACTH resulting in a clinical disease secondary to hyperadrenocorticism. The studies reported reaffirm the role of the hypothalamus in certain homeostatic mechanisms.

The disease process was effectively suppressed with exogenous dexamethasone treatment during two periods of administration.

References (51)

J.H. Bragdon
The colorimetric determination of blood lipids
J. Biol. Chem.
(1951)
J.J. Pisano et al.
Determinations of 3-methoxy-4-hydroxy mandelic acid in urine
Clin. chim. acta
(1962)
E.R. Buskirk et al.
Human energy expenditure studies in the National Institute of Arthritis and Metabolic Diseases metabolic chamber. I. Interaction of cold environment and specific dynamic effect
Am. J. Clin. Nutrition
(1960)
E.R. Buskirk et al.
Human energy expenditure studies in the National Institute of Arthritis and Metabolic Diseases metabolic chamber. II. Sleep
Am. J. Clin. Nutrition
(1960)
T.H. Allen et al.
Prediction of total adiposity from skinfolds and the curvilinear relationship between external and internal adiposity
Metabolism
(1956)
R.E. Peterson
The miscible pool and turnover rate of adrenocortical steroids in man
Recent Progr. Hormone Res.
(1959)
R.S. Gordon et al.
Unesterified fatty acid in human blood plasma
J. Clin. Invest.
(1956)
A. Kappas et al.
Steroid fever studies: physiological differences between bacterial pyrogens and endogenous steroid pyrogens of man
Tr. A. Am. Physicians
(1960)
C.M. Riley
Familial dysautonomia
S.M. Wolff et al.
A syndrome of periodic hypothalamic discharge
Clin. Res.
(1962)
H.G. Bauer
Endocrine and metabolic conditions related to pathology in the hypothalamus: a review
J. Nerv. & Ment. Dis.
(1959)

H.G. Bauer

Endocrine and other clinical manifestations of hypothalamic disease

J. Clin. Endocrinol.

(1954)

B. Hökfelt et al.

The effect of suprasellar tumours on the regulation of adrenocortical function

Acta endocrinol.

(1959)

L. Kahana et al.

Endocrine manifestations of intracranial extracellar lesions

J. Clin. Endocrinol.

(1962)

P. Heinbecker

The pathogenesis of Cushing's syndrome

Medicine

(1944)

W.G. Penfield

Diencephalic autonomic epilepsy

Arch. Neurol. & Psychiat.

(1929)

H.M. Rubinstein et al.

The colorimetric estimation of alpha-amino nitrogen in tissue fluids

J. Clin. Path.

(1959)

S. Udenfriend et al.

Assay of serotonin and related metabolites, enzymes and drugs

Methods Biochem. Anal.

(1958)

J. Uriel

Colorimetric detection of human ceruloplasmin oxidase activity after electrophoresis in agar gel or after immunoelectrophoresis

Nature, London

(1958)

J.M. Sperry et al.

Revision of Schoenheimer-Sperry method for cholesterol determination

J. Biol. Chem.

(1950)

C.P. Stewart et al.

The phospholipids of blood

Biochem. J.

(1935)

V.P. Dole

A relation between non-esterified fatty acids in plasma and the metabolism of glucose

J. Clin. Invest.

(1956)

A. Carlsson et al.

A fluorimetric method for the determination of dopamine (3-hydroxytyrbamine)

Acta physiol. scandinav.

(1958)

J.R. Crout

J.J. Pisano

A simple analysis for normetanephrine and metanephrine in urine

Clin. chim. acta

(1960)

E.R. Buskirk et al.

Metabolic response to cold air in men and women in relation to total body fat content

J. Appl. Physiol.

(1963)

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^☆: Part of this manuscript was presented at the meeting of the American Federation for Clinical Research, Atlantic City, New Jersey, April 29, 1962 [1].

¹: From the Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, and the Metabolic Diseases Branch, National Institute of Arthritis and Metabolic Diseases, National Institutes of Health, Bethesda, Maryland.

^†: Present address: Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland.

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Case reportA syndrome of periodic hypothalamic discharge☆

Abstract

J. Biol. Chem.

Clin. chim. acta

Am. J. Clin. Nutrition

Am. J. Clin. Nutrition

Metabolism

Recent Progr. Hormone Res.

J. Clin. Invest.

Tr. A. Am. Physicians