[A rare case of high dorsal radicular-medullary compression in a patient with ochronotic arthropathy. Clinico-radiological features].
Alkaptonuria is a rare, hereditary, metabolic disease in which homogentisic acid, an intermediary product in the metabolism of phenylalanine and tyrosine, cannot be further metabolized. The metabolic defect causes a characteristic triad of homogentisic aciduria, ochronosis, and arthritis. The cause of this disease is a constitutional lack of the enzyme homogentisic acid oxidase. The condition is inherited as an autosomal recessive disease. The ochronotic arthritis affects mainly male subjects after fourty. Authors present a case of a 60 years old man suffering from a generalized ochronotic arthritis since 1976. From 1992 summer to the beginning of 1993 the patient developed a progressive weakness in the lower limbs associated with thoracic back pain and subsequently a spastic paraparesis. These symptoms were related to a vertebral compression due to D4-D5 pathologic fracture. In spite of a decompressive operation the neurologic symptoms didn't improve.
Marsile
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Ospedale Predabissi, Vizzolo Predabissi, Milano.
Menozzi
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C
Menozzi
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C
D000368:Aged; D001168:Arthritis / Q000150:complications* / Q000000981:diagnostic imaging; D005260:Female; D005598:Fractures, Spontaneous / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D008159:Lumbar Vertebrae / Q000000981:diagnostic imaging; D008297:Male; D009408:Nerve Compression Syndromes / Q000209:etiology*; D009794:Ochronosis / Q000150:complications* / Q000235:genetics; D010375:Pedigree; D011859:Radiography; D013117:Spinal Cord Compression / Q000000981:diagnostic imaging / Q000209:etiology*; D016103:Spinal Fractures / Q000150:complications* / Q000000981:diagnostic imaging; D013126:Spinal Nerve Roots
Un raro caso di compressione radicolo-midollare dorsale alta in soggetto con artropatia ocronotica. Osservazioni clinico-radiologiche.