PMID: 9438162Nov 25, 1996Paper

Autonomic neuropathy in a patient with adrenomyeloneuropathy

European Journal of Medical Research
W J Schulte-MattlerS Zierz

Abstract

Adrenomyeloneuropathy (AMN) is a clinical subtype of X-linked adrenoleukodystrophy (ALD). In ALD, the accumulation of very long chain fatty acids (VLCFA) in the nervous system, adrenal cortex, and testes due to impaired degradation of VLCFA is thought to be pathogenic. Demonstration of abnormally high levels of saturated VLCFA is diagnostic of ALD. The symptoms of AMN include spastic paraparesis, impaired vibration sense in the distal legs, urinary disturbances, and mild peripheral neuropathy. About further involvement of the autonomic nervous system, no data are available. We report an AMN patient with clinically suspected and electrophysiologically verified autonomic neuropathy, supporting the hypothesis that neuropathy in AMN is at least partially caused by primary axonal degeneration.

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Adrenoleukodystrophy

Adrenoleukodystrophy (ALD), the most frequent peroxisomal disorder, is an X-linked disorder caused by a defect in the metabolism of long chain fatty acids leading to demyelination, neurodegeneration, and death. Here is the latest research.

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