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[A rare case of high dorsal radicular-medullary compression in a patient with ochronotic arthropathy. Clinico-radiological features].

作者信息

Marsile C, Menozzi C, Menozzi C

机构信息

Ospedale Predabissi, Vizzolo Predabissi, Milano.

出版信息

Minerva Med. 1995 Jan-Feb;86(1-2):61-6.

PMID:7753441
Abstract

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.

摘要

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