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Year : 1968  |  Volume : 2  |  Issue : 1  |  Page : 12-23

Achondroplasia With Paraplegia

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P Chandra

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Achondroplastic dwarfs have generally been employed as court jesters and have been known for their strength and wit. These are persons with odd body built but do not differ from other human beings in morbidity and life expectancy. However, contrary to general belief that achondroplastic changes are limited to the bones of extremities, it has been shown that the spine is also involved (Donath and Vogl 1925). This involvement of the spine rarely may predispose growing achondroplastics to progressive crippling lesion of the spinal cord, causing paraparesis and even quadriplegia. In the literature, about forty-eight cases of achondroplasia who developed neurological deficit varying from nerve root irritation of complete paraplegia or even quadriplegia have been reported upto 1967, including some unpublished cases and personal communications cited by Vogl in 1962. Although eight reports of paraplegia in achondroplastics appeared between 1905 and 1925, most of the remaining cases have been recognized during the last two decades. Vogl and Osborne (1949) reviewed nine case reported till then and added one more case of their own. Spillane (1952) reported three cases and pointed out another case reported by Poynton in 1907 which had not been included in the case reviewed by Vogl and Osborne. Vogl (1962) stated that twenty three cases of achondroplasia with neurological involvement had been reported between 1949 and 1960 including some unpublished cases and personal communications. These twenty-three cases included three cases of spillane (1952), two cases of Schreiber and Rosenthal (1952), one case of Epstein and Malis (1955) and one case of Vogl and Osborne (1949). This brought the total number of cases reported till 1960 to thirty-three. Report of five cases by Duvoisin and Yahr in 1962, eight cases by Hancock and Phillips in 1965 and two cases by Cohen, Rosenthal and Matson in 1967 has brought the total number to forty-eight upto date. The purpose of this paper is to stress the need to be aware of the possibility of cord and root compression in achondroplastic dwarfs specially because early treatment many offer the only chance for recovery, to review the available literature and to discuss certain important features of the syndrome in the light of our own observations in one case.

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