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COURSE AND SYMPTOMS OF PROGRESSIVE INFANTILE MUSCULAR ATROPHY: A Follow-Up Study of One Hundred and Twelve Cases in Denmark

COURSE AND SYMPTOMS OF PROGRESSIVE INFANTILE MUSCULAR ATROPHY: A Follow-Up Study of One Hundred... Abstract PROGRESSIVE infantile muscular atrophy was first described by Werdnig1 and Hoffmann2 about fifty years ago. This disorder is defined as a hereditary familial degeneration of the motor cells in the anterior horns of the spinal cord causing progressive atrophy and paralysis of the skeletal muscles. The atrophy begins in the muscles of the back and in the proximal muscles of the limbs and spreads gradually to the whole body. Life is threatened when the nerve cells supplying the respiratory muscles are affected, particularly when this involvement is followed by infection of the respiratory tract. Up to the time of this report, approximately 200 cases, occurring in about 120 families, have been reported. However, the reader will still find the best description of the disease in Hoffmann's papers. Many later single observations and short series of cases have contributed to our knowledge of the disease and its relation to References 1. Werdnig, G.: Zwei frühinfantile hereditäre Fälle von progressiver Muskelatrophie unter dem Bilde der Dystrophie, aber auf neurotischer Grundlage , Arch. f. Psychiat. 22:437, 1891Crossref 2. Die frühinfantile progressive spinale Amyotrophie , Werdnig Arch. f. Psychiat. 26:706, 1894.Crossref 3. Hoffmann, J.: Ueber chronische spinale Muskelatrophie im Kindesalter auf familiärer Basis , Deutsche Ztschr. f. Nervenh. 3:427, 1893Crossref 4. Weitere Beiträge zur Lehre von der hereditären progressiven spinalen Muskelatrophie im Kindesalter , Hoffmann Deutsche Ztschr. f. Nervenh. 10:292, 1897Crossref 5. Dritter Beitrag zur Lehre von der hereditären progressiven spinalen Muskelatrophie im Kindesalter , Hoffmann Deutsche Ztschr. f. Nervenh. 18:217, 1900Crossref 6. Ueber die hereditäre progressive spinale Muskelatrophie im Kindesalter , München. med. Wchnschr. 47:1649, 1900. 7. Batten, F. E.: Progressive Spinal Muscular Atrophy of Infants and Young Children , Brain 33:433, 1910-1911.Crossref 8. Krabbe, K. H.: Congenital Familial Spinal Muscular Atrophies and Their Relation to Amyotonia Congenita , Brain 43:166, 1920.Crossref 9. Slauck, A.: Ueber Myatonia congenita und infantile progressive spinale Muskelatrophie , Deutsche Ztschr. f. Nervenh. 67:1, 1921.Crossref 10. Greenfield, J. C., and Stern, R. O.: The Anatomical Identity of the Werdnig-Hoffmann and Oppenheim Forms of Infantile Muscular Atrophy , Brain 50:652, 1927.Crossref 11. Schildknecht, O.: Ueber die frühinfantile, progressive, spinale Muskelatrophie (Werdnig-Hoffmann) und ihre Beziehung zu Myotonia congenita (Oppenheim) , Deutsche Ztschr. f. Nervenh. 134:163, 1934.Crossref 12. de Lange, C.: Studien ueber angeborene Lähmungen bzw. angeborene Hypotonie , Acta pædiat. 20 ( (supp. 3) ): 1, 1937.Crossref 13. Karlström, F., and Wohlfart, G.: Klinische und histopatologische Studien über infantile spinale Muskelatrophie (Oppenheim'sche und Werdnig-Hoffmann'sche Krankheit) , Acta psychiat. et neurol. 14:453, 1939.Crossref 14. Hanhart, E.: Die infantile progressive spinale Muskelatrophie (Werdnig-Hoffmann) als einfachrezessive, subletale Mutation auf Grund von 29 Fällen in 14 Sippen , Helvet. pædiat. acta 1:110, 1945. 15. Wimmer ( Arch. f. Psychiat. 42:960, 1906)Crossref 16. Krabbe,4 17. Gjørup and Schrøder ( Acta pædiat. 18:211, 1935)Crossref 18. Hilden ( Nord. Med. 9:412, 1941) 19. Oluf Andersen ( Ugesk. f. læger 101:606, 1939). 20. Brandt, S.: Amyotonia Congenital Symptom and Not Separate Disorder: Follow-Up Study of One Hundred and Thirty-One Infants with Muscular Hypotonia , J. Child, Psychiat. 1:266, 1949. 21. Details of this reclassification and additional information will be published later in a monograph entitled "Werdnig-Hoffmann Progressive Infantile Muscular Atrophy." 22. Menges, O.: Ein Beitrag zur Pathologie der Myotonia congenita , Deutsche Ztschr. f. Nervenh. 121:240, 1931.Crossref 23. Fiore, G.: Atonia muscolare congenita e distrofie muscolari fetali , Riv. di clin. pediat. 25:319, 1927. 24. Finkelnburg: Anatomischer Befund bei progressiver Muskeldystrophie in den ersten Lebensjahren , Deutsche Ztschr. f. Nervenh. 35:453, 1908.Crossref 25. Keferstein, G.: Ueber die progressiven Muskelatrophien, Dissertation, Göttingen, 1894. 26. Brandt, S.: Hereditary Factors in Infantile Progressive Muscular Atrophy , Am. J. Dis. Child. 78:226 ( (Aug.) ) 1949.Crossref 27. Wohlfahrt, S., and Wohlfart, G.: Mikroskopische Untersuchungen an progressiven Muskelatrophien , Acta med. Scandinav. , 1935, (supp. 63) , p. 1. 28. Buchtahl, F., and Clemmesen, S.: On the Differentiation of Muscle Atrophy by Electromyography , Acta psychiat. et neurol. 16:144, 1941 29. The Electromyogram of Atrophic Muscles in Cases of Intramedullar Affections , Buchtahl Acta psychiat. et neurol. 18:377, 1943.Crossref 30. Grinker, R. R.: The Pathology of Amytonia Congenita: A Discussion of Its Relation to Infantile Progressive Muscular Atrophy , Arch. Neurol. & Psychiat. 18:982 ( (Dec.) ) 1927. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology & Psychiatry American Medical Association

COURSE AND SYMPTOMS OF PROGRESSIVE INFANTILE MUSCULAR ATROPHY: A Follow-Up Study of One Hundred and Twelve Cases in Denmark

Archives of Neurology & Psychiatry , Volume 63 (2) – Feb 1, 1950

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References (18)

Publisher
American Medical Association
Copyright
Copyright © 1950 American Medical Association. All Rights Reserved.
ISSN
0096-6754
DOI
10.1001/archneurpsyc.1950.02310200026003
Publisher site
See Article on Publisher Site

Abstract

Abstract PROGRESSIVE infantile muscular atrophy was first described by Werdnig1 and Hoffmann2 about fifty years ago. This disorder is defined as a hereditary familial degeneration of the motor cells in the anterior horns of the spinal cord causing progressive atrophy and paralysis of the skeletal muscles. The atrophy begins in the muscles of the back and in the proximal muscles of the limbs and spreads gradually to the whole body. Life is threatened when the nerve cells supplying the respiratory muscles are affected, particularly when this involvement is followed by infection of the respiratory tract. Up to the time of this report, approximately 200 cases, occurring in about 120 families, have been reported. However, the reader will still find the best description of the disease in Hoffmann's papers. Many later single observations and short series of cases have contributed to our knowledge of the disease and its relation to References 1. Werdnig, G.: Zwei frühinfantile hereditäre Fälle von progressiver Muskelatrophie unter dem Bilde der Dystrophie, aber auf neurotischer Grundlage , Arch. f. Psychiat. 22:437, 1891Crossref 2. Die frühinfantile progressive spinale Amyotrophie , Werdnig Arch. f. Psychiat. 26:706, 1894.Crossref 3. Hoffmann, J.: Ueber chronische spinale Muskelatrophie im Kindesalter auf familiärer Basis , Deutsche Ztschr. f. Nervenh. 3:427, 1893Crossref 4. Weitere Beiträge zur Lehre von der hereditären progressiven spinalen Muskelatrophie im Kindesalter , Hoffmann Deutsche Ztschr. f. Nervenh. 10:292, 1897Crossref 5. Dritter Beitrag zur Lehre von der hereditären progressiven spinalen Muskelatrophie im Kindesalter , Hoffmann Deutsche Ztschr. f. Nervenh. 18:217, 1900Crossref 6. Ueber die hereditäre progressive spinale Muskelatrophie im Kindesalter , München. med. Wchnschr. 47:1649, 1900. 7. Batten, F. E.: Progressive Spinal Muscular Atrophy of Infants and Young Children , Brain 33:433, 1910-1911.Crossref 8. Krabbe, K. H.: Congenital Familial Spinal Muscular Atrophies and Their Relation to Amyotonia Congenita , Brain 43:166, 1920.Crossref 9. Slauck, A.: Ueber Myatonia congenita und infantile progressive spinale Muskelatrophie , Deutsche Ztschr. f. Nervenh. 67:1, 1921.Crossref 10. Greenfield, J. C., and Stern, R. O.: The Anatomical Identity of the Werdnig-Hoffmann and Oppenheim Forms of Infantile Muscular Atrophy , Brain 50:652, 1927.Crossref 11. Schildknecht, O.: Ueber die frühinfantile, progressive, spinale Muskelatrophie (Werdnig-Hoffmann) und ihre Beziehung zu Myotonia congenita (Oppenheim) , Deutsche Ztschr. f. Nervenh. 134:163, 1934.Crossref 12. de Lange, C.: Studien ueber angeborene Lähmungen bzw. angeborene Hypotonie , Acta pædiat. 20 ( (supp. 3) ): 1, 1937.Crossref 13. Karlström, F., and Wohlfart, G.: Klinische und histopatologische Studien über infantile spinale Muskelatrophie (Oppenheim'sche und Werdnig-Hoffmann'sche Krankheit) , Acta psychiat. et neurol. 14:453, 1939.Crossref 14. Hanhart, E.: Die infantile progressive spinale Muskelatrophie (Werdnig-Hoffmann) als einfachrezessive, subletale Mutation auf Grund von 29 Fällen in 14 Sippen , Helvet. pædiat. acta 1:110, 1945. 15. Wimmer ( Arch. f. Psychiat. 42:960, 1906)Crossref 16. Krabbe,4 17. Gjørup and Schrøder ( Acta pædiat. 18:211, 1935)Crossref 18. Hilden ( Nord. Med. 9:412, 1941) 19. Oluf Andersen ( Ugesk. f. læger 101:606, 1939). 20. Brandt, S.: Amyotonia Congenital Symptom and Not Separate Disorder: Follow-Up Study of One Hundred and Thirty-One Infants with Muscular Hypotonia , J. Child, Psychiat. 1:266, 1949. 21. Details of this reclassification and additional information will be published later in a monograph entitled "Werdnig-Hoffmann Progressive Infantile Muscular Atrophy." 22. Menges, O.: Ein Beitrag zur Pathologie der Myotonia congenita , Deutsche Ztschr. f. Nervenh. 121:240, 1931.Crossref 23. Fiore, G.: Atonia muscolare congenita e distrofie muscolari fetali , Riv. di clin. pediat. 25:319, 1927. 24. Finkelnburg: Anatomischer Befund bei progressiver Muskeldystrophie in den ersten Lebensjahren , Deutsche Ztschr. f. Nervenh. 35:453, 1908.Crossref 25. Keferstein, G.: Ueber die progressiven Muskelatrophien, Dissertation, Göttingen, 1894. 26. Brandt, S.: Hereditary Factors in Infantile Progressive Muscular Atrophy , Am. J. Dis. Child. 78:226 ( (Aug.) ) 1949.Crossref 27. Wohlfahrt, S., and Wohlfart, G.: Mikroskopische Untersuchungen an progressiven Muskelatrophien , Acta med. Scandinav. , 1935, (supp. 63) , p. 1. 28. Buchtahl, F., and Clemmesen, S.: On the Differentiation of Muscle Atrophy by Electromyography , Acta psychiat. et neurol. 16:144, 1941 29. The Electromyogram of Atrophic Muscles in Cases of Intramedullar Affections , Buchtahl Acta psychiat. et neurol. 18:377, 1943.Crossref 30. Grinker, R. R.: The Pathology of Amytonia Congenita: A Discussion of Its Relation to Infantile Progressive Muscular Atrophy , Arch. Neurol. & Psychiat. 18:982 ( (Dec.) ) 1927.

Journal

Archives of Neurology & PsychiatryAmerican Medical Association

Published: Feb 1, 1950

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