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Progressive Bulbar Paralysis in Childhood (Fazio-Londe's Disease): Report of a Case with Pathologic Evidence of Nuclear Atrophy

Progressive Bulbar Paralysis in Childhood (Fazio-Londe's Disease): Report of a Case with... Abstract Bailey, Buchanan, and Bucy1 have said: "Whenever in our experience a child has developed in a chronic manner multiple palsies of the bulbar nerves, the cause has always been a neoplasm." To this rule there seem to be very few exceptions. We have had the opportunity of studying a child with progressive dissolution of the motor functions of the brain stem and of confirming by pathologic examination that there was, indeed, degeneration of the corresponding motor nuclei. This was the most striking manifestation of a progressive muscular atrophy. The following presentation is, to the best of our knowledge, the first clinical and pathological study of progressive nuclear amyotrophy chiefly affecting the brain stem in a child (progressive bulbar paralysis of childhood, Fazio-Londe's disease). Report of Case A white girl, the only child of healthy young parents, was born on April 7, 1956. The father had been hospitalized in early References 1. Roche Laboratories, Nutley, N.J. 2. Bailey, P.; Buchanan, D. N., and Bucy, P. C.: Intracranial Tumors of Infancy and Childhood , Chicago, University of Chicago Press, 1939, p. 239. 3. Duchenne (de Boulogne), G. B. A.: Paralysie musculaire progressive de la langue du voile du palais et des levres , Arch. Gen. Med. 16:283-296, 431-445, 1860. 4. Berger: Cited by van Bogaert.15 5. Hoffmann, J.: Ein Fall von chronischer progressiver Bulbärparalyse im Kindlichen , Deutsch. Z. Nervenh. 2:169-172, 1891.Crossref 6. Thomson, J.: Transactions of the Medico-Chirurgical Society, Session 70, Meeting 10, June 17, 1891, Edinburgh , Med. J. 37:262-263, 1891. 7. Fazio, M.: Ereditarietá della paralisi bulbare progressiva , Riforma Med . 8:327, 1892. 8. Remak, E.: Zur Pathologie der Bulbárparalyse , Arch. Psychiat. Nervenh. 23:919-960, 1892. 9. Londe, P.: Paralysie bulbaire progressive infantile et familiale , Rev. Med. 13:1020-1030, 1893. 10. Londe, P.: Paralysie bulbaire progressive infantile et familiale , Rev. Med. 14:212-254, 1894. 11. Filatow: Cited by van Bogaert.15 12. Brown, C. H.: Infantile Amyotrophic Lateral Sclerosis of the Family Type , J. Nerv. Ment. Dis. 21:707-716, 1894.Crossref 13. Trömner: Infantile Progressive Bulbärparalyse , Neurol. Cbl. 24:729, 1905. 14. Marinesco, G.: Sur deux cas de paralysie bulbaire progressive, infantile et familiale , C.R. Soc. Biol. 78:481, 1915. 15. Paulian, E. D.: Contributions clinique a l'étude de la paralysie bulbaire infantile familiale , Rev. Neurol. 22:275-278, 1922. 16. van Bogaert, L.: La sclérose latérale amyotrophique et la paralysie bulbaire progressive chez l'enfant , Rev. Neurol. 1:180-192, 1925. 17. Kurland, L. T., and Mulder, D. W.: Epidemiological Investigations of Amyotrophic Lateral Sclerosis: 2. Familial Aggregations Indicative of a Dominant Inheritance , Neurology 5:182-196, 249-268, 1955.Crossref 18. Gowers, W. R.: A Manual of Diseases of the Nervous System , Philadelphia, P. Blakiston, Son & Company, 1893, p. 564. 19. Bernhardt, M.: Über eine hereditäre Form der progressiven spinalen mit Bulbärparalyse complicisten Muskelatrophie , Arch. Path. Anat. 115:197-216, 1889.Crossref 20. Lovell, H. W.: Familial Progressive Bulbar Paralysis , Arch. Neurol. Psychiat. 28:394-398, 1932.Crossref 21. Robertson, E. E.: Progressive Bulbar Paralysis Showing Heredo-Familial Incidence and Intellectual Impairment , Arch. Neurol. Psychiat. 69:197-207, 1953.Crossref 22. Walton, J. N.: The Amyotonia Congenita Syndrome , Proc. Roy. Soc. Med. 50:301-306, 1957. 23. de Lange, C.: Zur Klinik der frühinfantilen und kongenitalen spinalen Lähmungen , Psychiat. Neurol. Bl. 20:1-14, 1916. 24. Grinker, R. R.: The Pathology of Amyotonia Congenita: Discussion of Its Relation to Infantile Progressive Muscular Atrophy , Arch. Neurol. Psychiat. 18:982-997, 1927.Crossref 25. Brandt, S.: Werdnig-Hoffmann's Infantile Progressive Muscular Atrophy , Copenhagen, Ejnar Munksgaards Forlag, 1950. 26. Thieffry, S.; Arthuis, M., and Bargeton, E.: Quarante cas de maladie de Werdnig-Hoffmann avec onze examens anatomiques , Rev. Neurol. 93:621-644, 1955. 27. Byers, R. K., and Banker, B. Q.: Infantile Muscular Atrophy , Arch. Neurol. 5:140-164, 1961.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Progressive Bulbar Paralysis in Childhood (Fazio-Londe's Disease): Report of a Case with Pathologic Evidence of Nuclear Atrophy

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Publisher
American Medical Association
Copyright
Copyright © 1962 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1962.00450220059009
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Abstract

Abstract Bailey, Buchanan, and Bucy1 have said: "Whenever in our experience a child has developed in a chronic manner multiple palsies of the bulbar nerves, the cause has always been a neoplasm." To this rule there seem to be very few exceptions. We have had the opportunity of studying a child with progressive dissolution of the motor functions of the brain stem and of confirming by pathologic examination that there was, indeed, degeneration of the corresponding motor nuclei. This was the most striking manifestation of a progressive muscular atrophy. The following presentation is, to the best of our knowledge, the first clinical and pathological study of progressive nuclear amyotrophy chiefly affecting the brain stem in a child (progressive bulbar paralysis of childhood, Fazio-Londe's disease). Report of Case A white girl, the only child of healthy young parents, was born on April 7, 1956. The father had been hospitalized in early References 1. Roche Laboratories, Nutley, N.J. 2. Bailey, P.; Buchanan, D. N., and Bucy, P. C.: Intracranial Tumors of Infancy and Childhood , Chicago, University of Chicago Press, 1939, p. 239. 3. Duchenne (de Boulogne), G. B. A.: Paralysie musculaire progressive de la langue du voile du palais et des levres , Arch. Gen. Med. 16:283-296, 431-445, 1860. 4. Berger: Cited by van Bogaert.15 5. Hoffmann, J.: Ein Fall von chronischer progressiver Bulbärparalyse im Kindlichen , Deutsch. Z. Nervenh. 2:169-172, 1891.Crossref 6. Thomson, J.: Transactions of the Medico-Chirurgical Society, Session 70, Meeting 10, June 17, 1891, Edinburgh , Med. J. 37:262-263, 1891. 7. Fazio, M.: Ereditarietá della paralisi bulbare progressiva , Riforma Med . 8:327, 1892. 8. Remak, E.: Zur Pathologie der Bulbárparalyse , Arch. Psychiat. Nervenh. 23:919-960, 1892. 9. Londe, P.: Paralysie bulbaire progressive infantile et familiale , Rev. Med. 13:1020-1030, 1893. 10. Londe, P.: Paralysie bulbaire progressive infantile et familiale , Rev. Med. 14:212-254, 1894. 11. Filatow: Cited by van Bogaert.15 12. Brown, C. H.: Infantile Amyotrophic Lateral Sclerosis of the Family Type , J. Nerv. Ment. Dis. 21:707-716, 1894.Crossref 13. Trömner: Infantile Progressive Bulbärparalyse , Neurol. Cbl. 24:729, 1905. 14. Marinesco, G.: Sur deux cas de paralysie bulbaire progressive, infantile et familiale , C.R. Soc. Biol. 78:481, 1915. 15. Paulian, E. D.: Contributions clinique a l'étude de la paralysie bulbaire infantile familiale , Rev. Neurol. 22:275-278, 1922. 16. van Bogaert, L.: La sclérose latérale amyotrophique et la paralysie bulbaire progressive chez l'enfant , Rev. Neurol. 1:180-192, 1925. 17. Kurland, L. T., and Mulder, D. W.: Epidemiological Investigations of Amyotrophic Lateral Sclerosis: 2. Familial Aggregations Indicative of a Dominant Inheritance , Neurology 5:182-196, 249-268, 1955.Crossref 18. Gowers, W. R.: A Manual of Diseases of the Nervous System , Philadelphia, P. Blakiston, Son & Company, 1893, p. 564. 19. Bernhardt, M.: Über eine hereditäre Form der progressiven spinalen mit Bulbärparalyse complicisten Muskelatrophie , Arch. Path. Anat. 115:197-216, 1889.Crossref 20. Lovell, H. W.: Familial Progressive Bulbar Paralysis , Arch. Neurol. Psychiat. 28:394-398, 1932.Crossref 21. Robertson, E. E.: Progressive Bulbar Paralysis Showing Heredo-Familial Incidence and Intellectual Impairment , Arch. Neurol. Psychiat. 69:197-207, 1953.Crossref 22. Walton, J. N.: The Amyotonia Congenita Syndrome , Proc. Roy. Soc. Med. 50:301-306, 1957. 23. de Lange, C.: Zur Klinik der frühinfantilen und kongenitalen spinalen Lähmungen , Psychiat. Neurol. Bl. 20:1-14, 1916. 24. Grinker, R. R.: The Pathology of Amyotonia Congenita: Discussion of Its Relation to Infantile Progressive Muscular Atrophy , Arch. Neurol. Psychiat. 18:982-997, 1927.Crossref 25. Brandt, S.: Werdnig-Hoffmann's Infantile Progressive Muscular Atrophy , Copenhagen, Ejnar Munksgaards Forlag, 1950. 26. Thieffry, S.; Arthuis, M., and Bargeton, E.: Quarante cas de maladie de Werdnig-Hoffmann avec onze examens anatomiques , Rev. Neurol. 93:621-644, 1955. 27. Byers, R. K., and Banker, B. Q.: Infantile Muscular Atrophy , Arch. Neurol. 5:140-164, 1961.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Apr 1, 1962

References