Effect of Naloxone in a Previously Undescribed Hypothalamic Syndrome

Wolff, O. H.; Leonard, J. V.; Preece, M A

doi:10.1016/s0140-6736(80)91735-3

Cited by 68 publications

(26 citation statements)

References 21 publications

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“…In this case, opiate blockers might attenuate SIB by increasing pain (hyperalgesia) during the abusive act. Consistent with the possibility that blocking the opioid system reversed chronic ''hypoalgesia'' or sensory depression in patients with SIB, it was reported that the opiate blockers naloxone and naltrexone (NTX) reversed congenital insensitivity to pain [Dehen et al, 1977], reversed hypothalamic dysfunction coexisting with elevated pain threshold [Dunger et al,1980], and increased brain sensory-evoked potentials [Arnsten et al, 1983].…”

Section: Opiate Blockers and Sibmentioning

confidence: 84%

Proopiomelanocortin (POMC) disregulation and response to opiate blockers

Sandman

Spence

Smith

1999

Ment. Retard. Dev. Disabil. Res. Rev.

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Autism is a collection of disorders or subtypes with distinctive or prominent phenotypes and genotypes. The study of adults with autism offers a unique opportunity to examine its phenotypic diversity. Recent evidence has identified disturbances in specific neurochemical systems that are associated with primary autistic symptoms. Establishing biological markers, such as specific neurochemical disturbances, not only confers greater precision in phenotyping individuals but also provides the basis for rational intervention. Our initial studies in adult individuals exhibiting self-injurious behavior (SIB) generated evidence that the proopiomelanocortin (POMC) system, specifically the endogenous opioid system, may be disregulated in subgroups of autistic patients. These findings, corroborated in at least 15 other laboratories, indicated that treatment with an opiate blocker, naltrexone (NTX), reduced SIB in 30-70% of individuals observed. However, the effects of NTX on SIB were not simple. We and others have found that concentration of plasma POMC fragments, specifically opioid fragments, contributed to the symptoms of autism and to the response to treatment. Uncoupling of the release of POMC products predicted the efficacy of NTX treatment on the expression of SIB. Uncoupling of POMC fragments among autistic and SIB patients suggested a basic, underlying defect, perhaps in the POMC gene. The findings of a maternal influence on the C-terminal BE fragment among individuals with autism (Leboyer et al. [1999] Soc Biol Psychiatry 45:158-163) and our preliminary findings, reported here, of a mutation in the opioid region of the POMC gene in an autistic individual, were consistent with the prospect that a subgroup of patients will be identified who share a POMC genetic defect.1999 Wiley-Liss, Inc.

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Section: Opiate Blockers and Sibmentioning

confidence: 84%

Proopiomelanocortin (POMC) disregulation and response to opiate blockers

Sandman

Spence

Smith

1999

Ment. Retard. Dev. Disabil. Res. Rev.

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“…Eight cases, including ours, developed central hypoventilation (3,5,(7)(8)(9). Six out of the eight had a poor respiratory prognosis.…”

Section: T H E O Rg An Ic C a Us E O F Hy P O T H Al Am I C Dysfunctimentioning

confidence: 71%

“…There are fourteen previously reported cases of idiopathic hypothalamic syndrome in children (1)(2)(3)(4)(5)(6)(7)(8)(9). Most patients (12/14) had the onset between one and 6 yr of age.…”

Section: T H E O Rg An Ic C a Us E O F Hy P O T H Al Am I C Dysfunctimentioning

confidence: 99%

“…Pharmacological therapy should be attempted when central hypoventilation is documented. Clomipramine HCl, theophyline, naloxone, and progesterone have been tried (3,5,7), and the former two drugs showed some effect (7). If the effect is not sufficient or severe symptoms are documented, several ventilatory support options such as mask ventilation or nasal intermittent positive pressure ventilation should be considered to keep the oxygen saturation with pulse oxymetry above 95% (9,10).…”

Section: T H E O Rg An Ic C a Us E O F Hy P O T H Al Am I C Dysfunctimentioning

confidence: 99%

“…Hypernatremia and hypothalamic hypopituitarism are also specific features of this disorder. Clinical onset usually appears between two and six years of age (1)(2)(3)(4)(5)(6)(7)(8)(9). At least fourteen cases have been reported in the literature.…”

Section: Introductionmentioning

confidence: 99%

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A Sudden Death Due to Central Hypoventilation in A 3-Year-Old Boy with Idiopathic Hypothalamic Dysfunction

Kobayashi

Miyamoto

Hasegawa

2003

Clin Pediatr Endocrinol

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Abstract. Idiopathic hypothalamic dysfunction is a rare disorder associated with adipsia, obesity and other symptoms such as central hypoventilation without any hypothalamic structural lesion. We report the case of a 3-year-old boy who died suddenly due to central hypoventilation. His obesity index increased from 0% to 75% during the 7 months prior to 3 yr 0 mo. During this clinical course, adipsia, hyperthermia, sudoresis, mild central hypoventilation, blepharoptosia, a change of character, hypernatremia, and hypothalamic hypopituitarism were observed. No treatment was given for hypoventilation. He died from sudden respiratory arrest because of central hypoventilation at 3 yr 8 mo. MRI examination was performed twice at 3 yr 3 mo and 3 yr 4 mo and revealed neither specific lesion nor invisible pituitary stalk. At autopsy, local inflammation and gliosis of the hypothalamus were present. So far at least 14 patients with idiopathic hypothalamic dysfunction have been reported. Eight cases including ours had central hypoventilation. Six out of the eight had poor respiratory prognosis, and central hypoventilation in hypothalamic dysfunction could be potentially fatal. In conclusion the patients with hypothalamic dysfunction should be treated intensively if they are suspected of having central hypoventilation.

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