A variant of adrenomyeloneuropathy with hypothalamic-pituitary dysfunction and neurologic remission after glucocorticoid replacement therapy

doi:10.1016/0002-9343(82)90606-4

The American Journal of Medicine

Volume 72, Issue 1, January 1982, Pages 173-176

https://doi.org/10.1016/0002-9343(82)90606-4 Get rights and content

Abstract

Adrenomyeloneuropathy is a syndrome comprising spastic paraparesis, polyneuropathy, primary adrenocortical insufficiency and variable hypogonadism. We describe a 32 year old man who presented with contractures, peripheral neuropathy, primary adrenocortical insufficiency and secondary hypogonadism. Abnormal responses of growth hormone, gonadotropins, prolactin and thyrotropin to provocative stimuli were found, without radiographic evidence of a pituitary or hypothalamic lesion. Almost complete recovery from the neurologic abnormalities occurred with glucocorticoid replacement therapy. The clinical features of this patient support a diagnosis of adrenomyeloneuropathy. The hypothalamic-pituitary dysfunction extends the clinical spectrum of this disease. Remission of the paraparesis coincident with glucocorticoid replacement has not been reported previously.

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^☆: This work was supported in part by the Veterans Administration.

^∗: Present address: Department of Medicine, Yale University, New Haven, Connecticut 06510.

¹: From the Department of Medicine, SUNY Downstate Medical Center, and the Medical Service, Veterans Administration Medical Center, Brooklyn, New York.

^†: Present address: Veterans Administration Medical Center, 30th and Euclid Streets, Des Moines, Iowa 50370.

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Case reportA variant of adrenomyeloneuropathy with hypothalamic-pituitary dysfunction and neurologic remission after glucocorticoid replacement therapy☆

Abstract

Am J Med

Die erkrankungen der nebennieren

Familial Addison's disease with spastic paraplegia

J Clin Endocrinol

Addison's disease in association with spastic paraplegia

Br Med J

Progressive spastic paraparesis and adrenal insufficiency

Arch Neurol

Adrenomyeloneuropathy: a probable variant of adrenoleukodystrophy. 1. Clinical and endocrinologic aspects

Neurology

Spastic paraplegia associated with Addison's disease: adult variant of adrenoleukodystrophy

J Neurol

Adrenomyeloneuropathie: forme adulte d'adrenoleukodystrophie

Rev Neurol (Paris)

Case report
A variant of adrenomyeloneuropathy with hypothalamic-pituitary dysfunction and neurologic remission after glucocorticoid replacement therapy☆