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Nerve conduction studies in adrenomyeloneuropathy

V Chaudhry1, H W Moser, D R Cornblath

  • 1Department of Neurology, Johns Hopkins Hospital, Baltimore, MD 21287, USA.

Journal of Neurology, Neurosurgery, and Psychiatry
|August 1, 1996
PubMed
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Nerve conduction studies reveal frequent abnormalities in adrenomyeloneuropathy (AMN), indicating both axonal loss and demyelination. These findings correlate with patient disability and serum very long chain fatty acids (VLCFAs), aiding treatment evaluation.

Area of Science:

  • Neurology
  • Genetics
  • Metabolic Disorders

Background:

  • Adrenomyeloneuropathy (AMN) is an X-linked metabolic disorder.
  • It causes progressive spastic paraparesis and peripheral neuropathy.
  • Pyramidal signs can mask neuropathy, complicating diagnosis.

Purpose of the Study:

  • To characterize peripheral neuropathy in AMN patients using nerve conduction studies (NCS).

Main Methods:

  • NCS were performed on 99 men and 38 heterozygous women with AMN and neurological disabilities.

Main Results:

  • Abnormalities were found in 82% of patients, more common in men, legs, motor nerves, and involving latency/velocity.
  • Demyelinating range values were observed in 26 patients.

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  • NCS variables correlated with disability status and serum very long chain fatty acids (VLCFAs).

    • Conclusions:

    • NCS in AMN patients frequently show mixed axonal loss and multifocal demyelination.
    • NCS measures may help evaluate future AMN treatments due to correlations with disability and VLCFAs.