Objective and Importance: Myelopathy secondary to alcaptonuria or ochronosis has been reported in only a few patients, but no reports on alcaptonuria and neuropathy have been available. Clinical Presentation: We report a case of alcaptonuria in a 55-year-old patient who presented with neuropathy and myelopathy after sustaining injuries in a traffic accident. Alcaptonuria, which is a rare autosomal recessive disorder characterized by the inability to metabolize homogentisic acid, was confirmed by the presence of homogentisic acid in the urine of the patient and by typical radiological changes in the spine, hips and knees. Conclusion: The association of alcaptonuria and peripheral neuropathy in this patient may remain accidental; nevertheless, the possibility exists that it is a further disorder of amino acid metabolism associated with neuropathy.

Keywords:
Alcaptonuria, Myelopathy, Neuropathy
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1999
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