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Klippel-Feil Syndrome: A Study of Mirror Movement Detected by Electromyography

Klippel-Feil Syndrome: A Study of Mirror Movement Detected by Electromyography Abstract A TAILOR with pleurisy and nephritis was described by Klippel and Feil1 in 1912 because his neck was so short that his head seemed to rest directly on his shoulders. Head movement was very limited and the hairline was low. At postmortem examination there were only four cervical vertebrae fused into one column of bone. Many similar cases have been reported. The Klippel-Feil syndrome is usually defined as the triad of short neck, low hairline, and limitation of neck movement. The radiological examination of the cervical spine shows a failure of segmentation of variable degree. Patients with this syndrome may be otherwise normal or may have a variety of additional anomalies. Some of these include sensorineural and conductive hearing loss, scoliosis, cleft palate, Sprengel's deformity, congenital heart malformation, facial asymmetry, nystagmus, micrognathia, and cranial nerve palsies. In 1932 Bauman2 noticed that four of six patients with the syndrome References 1. Klippel, M., and Feil, A.: Un cas d'absence des vertebres cervicales avec cage thoracique remontant jusqu'a la base du crane (cage thoracique cervicale) , Nouv Iconogr Salpet 25:223, 1912. 2. Bauman, G.I.: Absence of the Cervical Spine: Klippel-Feil Syndrome , JAMA 98:129, 1932.Crossref 3. Mitchell, H.S.: Klippel-Feil Syndrome (Congenital Webbed Neck) , Arch Dis Child 9:213, 1934.Crossref 4. Erskine, C.A.: Analysis of Klippel-Feil Syndrome , Arch Path 41:269, 1946. 5. Ford, F.R.: Diseases of the Nervous System in Infancy, Childhood and Adolescence , ed 3, Springfield Ill: Charles C Thomas, Publishers, 1952, p 308. 6. Spillane, J.D.; Pallis, C.; and Jones, A.M.: Developmental Abnormalities in the Region of the Foramen Magnum , Brain 80:11, 1957.Crossref 7. Willard, D.P., and Nicholson, J.T.: Klippel-Feil Syndrome , Ann Surg 99:561, 1934.Crossref 8. Abercrombie, M.L.J.; Lindon, R.L.; and Tyson, M.C.: Associated Movements in Normal and Physically Handicapped Children , Develop Med Child Neurol 6:573, 1964.Crossref 9. Tascon-Alonzo, M.: The Amplitude of Evoked Potentials in Human Hand Muscles in Health and Disease, (thesis, University of Alberta, Alberta, Canada, 1963). 10. Mainland, D.; Herrera, L.; and Sutcliffe, M.I.: Statistical Tables for Use with Binomial Samples: Contingency Tests, Confidence Limits, and Sample Size Estimates, Dept Medical Statistics, New York University College of Medicine, New York, 1956. 11. Mosberg, W.H.: The Klippel-Feil Syndrome: Etiology and Treatment of Neurologic Signs , J Nerv Ment Dis 117:479, 1953.Crossref 12. Holtzer, H., and Detwiler, S.R.: An Experimental Analysis of the Development of the Spinal Column: III. Induction of Skeletogenous Cells , J Exp Zool 123:335, 1953.Crossref 13. Avery, G.B., and Guandolo, V.L.: (Status Dysraphicus): Clinical Proceedings. Grand Rounds Transcript , Children's Hospital (Wash) : 22:51, 1966. 14. Guttman, E.; Maclay, W.S.; and Stokes, A.B.: Persistent Mirror Movements as a Heredo-Familial Disorder , J Neurol Neurosurg Psychiat 2:13, 1939.Crossref 15. Johnston, P.W.: Hereditary Mirror Movements: Report of a Case , Bull Los Angeles Neurol Soc 13:119, 1948. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Klippel-Feil Syndrome: A Study of Mirror Movement Detected by Electromyography

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Publisher
American Medical Association
Copyright
Copyright © 1967 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1967.02090200078006
Publisher site
See Article on Publisher Site

Abstract

Abstract A TAILOR with pleurisy and nephritis was described by Klippel and Feil1 in 1912 because his neck was so short that his head seemed to rest directly on his shoulders. Head movement was very limited and the hairline was low. At postmortem examination there were only four cervical vertebrae fused into one column of bone. Many similar cases have been reported. The Klippel-Feil syndrome is usually defined as the triad of short neck, low hairline, and limitation of neck movement. The radiological examination of the cervical spine shows a failure of segmentation of variable degree. Patients with this syndrome may be otherwise normal or may have a variety of additional anomalies. Some of these include sensorineural and conductive hearing loss, scoliosis, cleft palate, Sprengel's deformity, congenital heart malformation, facial asymmetry, nystagmus, micrognathia, and cranial nerve palsies. In 1932 Bauman2 noticed that four of six patients with the syndrome References 1. Klippel, M., and Feil, A.: Un cas d'absence des vertebres cervicales avec cage thoracique remontant jusqu'a la base du crane (cage thoracique cervicale) , Nouv Iconogr Salpet 25:223, 1912. 2. Bauman, G.I.: Absence of the Cervical Spine: Klippel-Feil Syndrome , JAMA 98:129, 1932.Crossref 3. Mitchell, H.S.: Klippel-Feil Syndrome (Congenital Webbed Neck) , Arch Dis Child 9:213, 1934.Crossref 4. Erskine, C.A.: Analysis of Klippel-Feil Syndrome , Arch Path 41:269, 1946. 5. Ford, F.R.: Diseases of the Nervous System in Infancy, Childhood and Adolescence , ed 3, Springfield Ill: Charles C Thomas, Publishers, 1952, p 308. 6. Spillane, J.D.; Pallis, C.; and Jones, A.M.: Developmental Abnormalities in the Region of the Foramen Magnum , Brain 80:11, 1957.Crossref 7. Willard, D.P., and Nicholson, J.T.: Klippel-Feil Syndrome , Ann Surg 99:561, 1934.Crossref 8. Abercrombie, M.L.J.; Lindon, R.L.; and Tyson, M.C.: Associated Movements in Normal and Physically Handicapped Children , Develop Med Child Neurol 6:573, 1964.Crossref 9. Tascon-Alonzo, M.: The Amplitude of Evoked Potentials in Human Hand Muscles in Health and Disease, (thesis, University of Alberta, Alberta, Canada, 1963). 10. Mainland, D.; Herrera, L.; and Sutcliffe, M.I.: Statistical Tables for Use with Binomial Samples: Contingency Tests, Confidence Limits, and Sample Size Estimates, Dept Medical Statistics, New York University College of Medicine, New York, 1956. 11. Mosberg, W.H.: The Klippel-Feil Syndrome: Etiology and Treatment of Neurologic Signs , J Nerv Ment Dis 117:479, 1953.Crossref 12. Holtzer, H., and Detwiler, S.R.: An Experimental Analysis of the Development of the Spinal Column: III. Induction of Skeletogenous Cells , J Exp Zool 123:335, 1953.Crossref 13. Avery, G.B., and Guandolo, V.L.: (Status Dysraphicus): Clinical Proceedings. Grand Rounds Transcript , Children's Hospital (Wash) : 22:51, 1966. 14. Guttman, E.; Maclay, W.S.; and Stokes, A.B.: Persistent Mirror Movements as a Heredo-Familial Disorder , J Neurol Neurosurg Psychiat 2:13, 1939.Crossref 15. Johnston, P.W.: Hereditary Mirror Movements: Report of a Case , Bull Los Angeles Neurol Soc 13:119, 1948.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: May 1, 1967

References