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Author Legare, Janet M. ♦ Modaff, Peggy ♦ Iskandar, Bermans J. ♦ Pauli, Richard M.
Source World Health Organization (WHO)-Global Index Medicus
Content type Text
Publisher Wiley-Blackwell
File Format HTM / HTML
Language English
Difficulty Level Medium
Subject Domain (in DDC) Technology ♦ Medicine & health
Abstract We describe five children with Hereditary Multiple Exostosis (HME) who also had syringomyelia. Of these, four had a tethered cord/fibrolipoma. No spinal osteochondromas were found in these patients. All had antecedent neurological signs or symptoms that prompted spinal imaging with MRI. Of all patients with HME seen in the Midwest Regional Bone Dysplasia Clinic from 1982 to present, 44% (17/39) of patients had signs or symptoms concerning for possible cord-related neurological findings. However, only 10 of 39 had spinal imaging. Assuming that all individuals with syringomyelia were identified, then 5/39 (13%) were in that way affected. This, of course, is a minimal estimate given that many were not imaged. The incidence of syringomyelia appears to be increased in this population, and seems to be unrelated to spinal osteochondromas. A low threshold for obtaining spinal MRI in patients with Hereditary Multiple Exostosis seems rational. © 2016 Wiley Periodicals, Inc.
Description Author Affiliation: Legare JM ( Division of Genetics and Metabolism, Department of Pediatrics, University of Wisconsin, Madison, Wisconsin. jmlegare@pediatrics.wisc.edu.); Modaff P ( Division of Genetics and Metabolism, Department of Pediatrics, University of Wisconsin, Madison, Wisconsin.); Iskandar BJ ( Department of Neurosurgery, University of Wisconsin, Madison, Wisconsin.); Pauli RM ( Division of Genetics and Metabolism, Department of Pediatrics, University of Wisconsin, Madison, Wisconsin.)
ISSN 15524825
Educational Role Student ♦ Teacher
Age Range above 22 year
Educational Use Reading ♦ Research ♦ Self Learning
Interactivity Type Expositive
Education Level UG and PG
Learning Resource Type Article
Publisher Date 2016-11-01
Publisher Place United States
e-ISSN 15524833
Journal American Journal of Medical Genetics Part A
Volume Number 170
Issue Number 11


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Source: WHO-Global Index Medicus