Lower Motor Neuron Deficit in Arthrogryposis: Title and subTitle BreakAn EMG Study

EDWIN M. SMITH, M.D.; LEONARD F. BENDER, M.D.; CORNELIUS N. STOVER, M.D.

[+] Author Affiliations

Received for publication Aug. 13, 1962.

Read at the Meeting of the American Association of Electromyography and Electrodiagnosis, Aug. 27, 1961.

Medical School, The University of Michigan.

This study was supported in part by a grant from the Sister Elizabeth Kenny Foundation.

Arch Neurol. 1963;8(1):97-100. doi:10.1001/archneur.1963.00460010113013

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ABSTRACT

ABSTRACT | REFERENCES

The pathogenesis of arthrogryposis multiplex congenita has continued to be obscure since the disorder was first described by Otto in 1841. Although there is fairly uniform agreement today that the multiplex congenital contractures found in this disease are related to abnormality of muscle and to reduced intrauterine movement, the nature and cause of the muscle abnormality remain in doubt.^1-3

After reviewing the literature, Drachman and Banker⁴ concluded that all cases labeled arthrogryposis which have had microscopic examination of muscle showed changes attributable either to denervation or to a primary nonprogressive myopathy. They point out that in 12 of the 13 known cases in which the spinal cords of arthrogrypotic patients have also been studied, the one consistent finding has been significant reduction in the number of anterior horn cells, particularly at cervical and lumbosacral levels. In 1 of the 12 spinal cords neuronophagia was observed, and this was

REFERENCES

ABSTRACT | REFERENCES

Steindler, A.: Arthrogryposis , J. Int. Coll. Surg. 12:21-25, 1949;.

Sheldon, W.: Multiple Congenital Articular Rigidity: Arthrogryposis Multiplex Congenita , Arch. Dis. Child 7:117-136, 1932;.

Adams, R. D.; Denny-Brown, D., and Pearson, C. H.: Diseases of Muscle: A Study in Pathology , New York, Paul B. Hoeber, Inc., Medical Book Department of Harper & Brothers, 1953;.

Drachman, D. B., and Banker, B. Q.: Arthrogryposis Multiplex Congenita , Arch. Neurol. 5:77-93, 1961;.

Lefebvre, J., and Chaumont, P.: Résultats de l'examen électrique et électromyographique dans l'arthrogrippose , J. Radiol. Electrol. 39:214-216, 1958;.

Banker, B. Q.; Victor, M., and Adams, R. D.: Arthrogryposis Multiplex Due to Congenital Muscular Dystrophy , Brain 80:319-334, 1957;.

Badgley, C.: American Academy of Orthopaedic Surgeons , Instructional Course Lectures, Vol. X, Ann Arbor, Mich., J. W. Edwards, 1953;.

Mead, N. G.; Lithgow, W. C., and Sweeney, H. J.: Arthrogryposis Multiplex Congenita , J. Bone Joint Surg . 40A:1285-1309, 1958;.

Johnson, E. W.; Guyton, J. D., and Olsen, K. J.: Motor Nerve Conduction Velocity Studies in Poliomyelitis , Arch. Phys. Med. 41:185-190, 1960;.

Lambert, E. H., et al.: Clinical Examinations in Neurology , Philadelphia, W. B. Saunders Company, 1956;.

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