A variant of adrenomyeloneuropathy with hypothalamic-pituitary dysfunction and neurologic remission after glucocorticoid replacement therapy.
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 adn 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 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.
D000303:Adrenal Cortex Diseases / Q000188:drug therapy*; D000304:Adrenal Cortex Function Tests; D000328:Adult; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use*; D007006:Hypogonadism / Q000209:etiology; D008297:Male; D009128:Muscle Spasticity / Q000188:drug therapy; D010264:Paraplegia / Q000188:drug therapy*; D010523:Peripheral Nervous System Diseases / Q000188:drug therapy*; D010901:Pituitary Function Tests; D013577:Syndrome
D006854:Hydrocortisone