Prevalence of Syndrome of ACTH-ADH Discharge in Japan

Abstract

Abstract.The prevalence of the syndrome of ACTH -ADH discharge in Japan was studied by means of a questionnaire to all members of the Japanese Society for Pediat ric Endocrinology.Ninety-one patients were reported by 107 members , and clinical details of 46 cases were obtained. The age of the patients was widely distributed , from one to 15 yrs (median, eight yrs), while the age of onset was usually between zero and four yrs , indicating a long duration of the symptoms.Eight patients had various congenital diseases . As for the three cardinal symptoms of vomiting ,h ypertension and psychotic depression, five cases (10 .8%) did not have hypertension . Association of autonomic symptoms was common (85%) . Laboratory data showed a consistent rise in ACTH and ADH at the initial phase of the attack , whereas hyponatremia did not occur in 30% of patients. Elevation of plasma norepinephrine and a concomitant decrease of dopamine were also constantly found. Eleven (26.8%) of 41 cases had an abnormal EEG , showing high voltage slow bursts. A wide variety of drugs was used for treatment , but the most effective were anticonvulsants, especially phenytoin, while antagonists of catecholamine were only transiently effective .These data indicate that (1) the syndrome of ACTH-ADH discharge is a fairly common disorder in Japan, (2) it is a quite different syndrome from cyclic vomiting in childhood , (3) the symptoms last for a long time, in some cases beyond adolescence , and (4) the most effective drugs are anticonvulsants. It is suggested that this syndrome may be due to periodic epileptic discharge in the hypothalamus involving paraventricular CRH-and ADH-neurons and the emetic center in the medulla.

Cyclic vomiting syndrome in adults

“…A rare syndrome of recurrent vomiting associated with hypertension and profound listlessness during episodes has been reported in children with CVS‐like conditions by Sato et al 19–21 . Similarly, Pasricha et al.…”

“…A rare syndrome of recurrent vomiting associated with hypertension and profound listlessness during episodes has been reported in children with CVS-like conditions by Sato et al [19][20][21] Similarly, Pasricha et al 22 have described an adult with cyclic vomiting episodes associated with hypertension and depression during the episodes.…”

“…The importance of catecholamine and prostaglandin pathways in the genesis of this variant is evidenced by the reduction in symptom produced by the a-adrenoceptor agonist clonidine and the prevention of vomiting episodes by indomethacin pretreatment. [19][20][21] In 1964, Wolff et al 60 described a patient with cyclic nausea and vomiting associated with hypertension, abnormal glucose tolerance, elevated plasma ACTH and cortisol that could be attenuated by high doses of dexamethasone. Underlying hypothalamic derangements have also been suggested in other paediatric CVS cases.…”

“…They found a rise in peripheral levels of ACTH, cortisol, catecholamines (noradrenaline and adrenaline), antidiuretic hormone (ADH) and prostaglandin E 2 (PGE 2 ) preceding and during the early part of the vomiting episode. The importance of catecholamine and prostaglandin pathways in the genesis of this variant is evidenced by the reduction in symptom produced by the α‐adrenoceptor agonist clonidine and the prevention of vomiting episodes by indomethacin pretreatment 19–21 . In 1964, Wolff et al.…”

Type 1 diabetes complicated with uncontrollable adult cyclic vomiting syndrome: a case report

“…That study particularly focused on patients with an increase in ACTH and ADH during the vomiting period, characterized by improvement and prompt remission, also known as ACTH-ADH hypersecretory syndrome [ 12 – 14 ]. Others [ 15 ] have reported that a family history of migraine is commonly recognized in patients with CVS. Another pathophysiologic possibility is mitochondrial dysfunction caused by mutations in mitochondrial DNA in which energy deficiency might trigger the onset of vomiting during increased energy demand at the time of infection or stress, as has been reported in pediatric patients [ 15 – 17 ].…”