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Familial Lumbosacral Syringomyelia

Familial Lumbosacral Syringomyelia Familial Lumbosacral Syringomyelia 1 Clarence Van Epps , M.D. and H. Dabney Kerr , M.D. Iowa City, Iowa ↵ 1 Presented before the Fifth International Congress of of Radiology, at Chicago, Sept. 13–17, 1937. Excerpt THE appearance in our clinic of two or more members of four different families with trophic changes in the soft and bony tissues of the feet has led us to review the literature of similar cases. One of our cases was sporadic, but as the picture was similar to the familial cases we have included it in our report—all the more so since one case, at first considered sporadic, was found to be one of seven in the family. We have hesitated long as to the proper title to use. It is our belief that the various etiologic factors of the syndrome are of the developmental type and that the terms, status dysraphicus, myelodysplasia, syringomyelia , and trophopathia pedis myelo-dysplastica , all have some basis for being suggested as the preferable term. Since there is no reported autopsy on a case of this type, except one incomplete report by Schlesinger on the case of Verhoogen and Vandervelde (65) and considered by him not to be syringomyelia, the choice of a term rests almost entirely on a clinical basis. Status dysraphicus (Bremer, 7) is especially frequent in relatives of persons with syringomyelia. He found that spinal cords of persons with funnel chest revealed gliotic changes around the central canal in six of eight cases, these changes differing in degree but not in nature from those found in syringomyelia. The most striking and frequent manifestations of status dysraphicus are anomalies of the sternum, kyphoscoliosis, differences in the mammary glands, body span exceeding body length, livid cold hands, tendency to bending or curvature of the fingers, disturbances of sensation of circular limitation, and nocturnal enuresis. Myelodysplasia (Fuchs, 17) is characterized by syndactyly, dissociated sensory loss, open sacral canal, anomalies of the superficial and deep reflexes of the abdomen and legs, deformities of the feet often with peroneal weakness and trophic and vasomotor disturbances of the feet. Trophopathia pedis Myelodysplastica. —Under this title Kienböck (31) has reported four sporadic cases, in three of which the changes in the feet closely resembled those in our series. In only one case was a radiograph made of the spine and this showed a spina bifida. He collected 17 cases of occult spina bifida with trophic changes in the skin and bones of the feet and classified his cases among that group. There exists a closely allied group of cases with symmetrical gangrene of the hands and feet (Table I). Of these, 18 familial and one sporadic case have been reported. The largest series was one of eight cases reported by Nékám (44). Syringomyelia rarely occurs as a familial disease, only 44 cases being recorded in which the distribution was of the usual cervico-thoracic type (Table II). In only four families did the number of cases exceed two. The exceptional series was one of 13 cases reported by Barraquer and de Gispert (4). These authors examined five cases and had a history of eight others. Copyrighted 1940 by the Radiological Society of North America, Inc. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Radiology Radiological Society of North America, Inc.

Familial Lumbosacral Syringomyelia

Radiology , Volume 35 (2): 160 – Aug 1, 1940

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References (7)

Publisher
Radiological Society of North America, Inc.
Copyright
Copyright © 1940 by Radiological Society of North America
ISSN
1527-1315
eISSN
0033-8419
DOI
10.1148/35.2.160
Publisher site
See Article on Publisher Site

Abstract

Familial Lumbosacral Syringomyelia 1 Clarence Van Epps , M.D. and H. Dabney Kerr , M.D. Iowa City, Iowa ↵ 1 Presented before the Fifth International Congress of of Radiology, at Chicago, Sept. 13–17, 1937. Excerpt THE appearance in our clinic of two or more members of four different families with trophic changes in the soft and bony tissues of the feet has led us to review the literature of similar cases. One of our cases was sporadic, but as the picture was similar to the familial cases we have included it in our report—all the more so since one case, at first considered sporadic, was found to be one of seven in the family. We have hesitated long as to the proper title to use. It is our belief that the various etiologic factors of the syndrome are of the developmental type and that the terms, status dysraphicus, myelodysplasia, syringomyelia , and trophopathia pedis myelo-dysplastica , all have some basis for being suggested as the preferable term. Since there is no reported autopsy on a case of this type, except one incomplete report by Schlesinger on the case of Verhoogen and Vandervelde (65) and considered by him not to be syringomyelia, the choice of a term rests almost entirely on a clinical basis. Status dysraphicus (Bremer, 7) is especially frequent in relatives of persons with syringomyelia. He found that spinal cords of persons with funnel chest revealed gliotic changes around the central canal in six of eight cases, these changes differing in degree but not in nature from those found in syringomyelia. The most striking and frequent manifestations of status dysraphicus are anomalies of the sternum, kyphoscoliosis, differences in the mammary glands, body span exceeding body length, livid cold hands, tendency to bending or curvature of the fingers, disturbances of sensation of circular limitation, and nocturnal enuresis. Myelodysplasia (Fuchs, 17) is characterized by syndactyly, dissociated sensory loss, open sacral canal, anomalies of the superficial and deep reflexes of the abdomen and legs, deformities of the feet often with peroneal weakness and trophic and vasomotor disturbances of the feet. Trophopathia pedis Myelodysplastica. —Under this title Kienböck (31) has reported four sporadic cases, in three of which the changes in the feet closely resembled those in our series. In only one case was a radiograph made of the spine and this showed a spina bifida. He collected 17 cases of occult spina bifida with trophic changes in the skin and bones of the feet and classified his cases among that group. There exists a closely allied group of cases with symmetrical gangrene of the hands and feet (Table I). Of these, 18 familial and one sporadic case have been reported. The largest series was one of eight cases reported by Nékám (44). Syringomyelia rarely occurs as a familial disease, only 44 cases being recorded in which the distribution was of the usual cervico-thoracic type (Table II). In only four families did the number of cases exceed two. The exceptional series was one of 13 cases reported by Barraquer and de Gispert (4). These authors examined five cases and had a history of eight others. Copyrighted 1940 by the Radiological Society of North America, Inc.

Journal

RadiologyRadiological Society of North America, Inc.

Published: Aug 1, 1940

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