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CLINICAL AND PATHOLOGICAL FINDINGS IN A CASE OF DYSTROPHIA MYOTONICA

CLINICAL AND PATHOLOGICAL FINDINGS IN A CASE OF DYSTROPHIA MYOTONICA Abstract IN 1944 I1 described a typical case of dystrophia myotonica in a man aged 44. Death occurred in 1948, and autopsy was performed. An anatomical examination was made of the endocrine glands, the muscles and the central and peripheral nervous systems. CLINICAL FINDINGS The patient was extremely bald; the left eye was artificial, and the right eye revealed the remains of iridectomy and an operation for cataract. He presented the characteristic aspect of the facies myopathica, and the general appearance was that of typical Jammergestalt. Speech was unintelligible; the thyroid gland was not palpable. There was fairly general adiposity. The left testis was very small; the right testis was still smaller, being about the size of a marble. Both were soft. There was male distribution of hair. The extremities were thin and showed atrophy of the muscles. The left leg was thinner than the right. The calves and the References 1. den Hartog Jager, W. A.: Over dystrophia myotonica , Psychiat. en neurol. bl. , p. 1 ( (Jan.) ) 1944. 2. Rasmussen, A. T.: Percentage of Different Types of Cells in Male Adult Human Hypophysis , Am. J. Path. 5:263 ( (May) ) 1929. 3. Ruyter, J. H. C.: A New Staining Method for the Hypophysis , Acta neerl. Morph. , p. 180, 1943. 4. Clark, W. E. Le Gros; Beattie, J.; Riddoch, G., and Dott, N. M.: The Hypothalamus: Morphological, Functional, Clinical and Surgical Aspects , The Henderson Trust Lectures, nos. XIII-XVI, published for the William Ramsay Henderson Trust, Edinburgh, Oliver & Boyd, 1938. 5. Jakob, A.: Die extrapyramidalen Erkrankungen , Berlin, Julius Springer, 1923. 6. Steinert, H.: Myopathologische Beiträge , Deutsche Ztschr. Nervenh. 37:58, 1909.Crossref 7. Hoffmann, J.: Vergaderingsbericht , München, med. Wchnschr. 66:519, 1919. 8. Fischer, L.: Klinische, psychopathologische und anatomische Beiträge zur Dystrophia myotonica , Ztschr. ges. Neurol. u. Psychiat. 58:254, 1920.Crossref 9. Hitzenberger, R.: Über myotonische Dystrophie , Monatsschr. Psychiat. u. Neurol. 47:249, 1920.Crossref 10. Bramwell, E.: Case of Myotonia Atrophica with Autopsy , Proc. Roy. Soc. Med. 16(Sec. Neurol.): 11, 1923. 11. Adie, W. J., and Greenfield, J. G.: Dystrophia Myotonica , Brain 46:73, 1923.Crossref 12. Weil, A., and Keschner, M.: Ein Beitrag zur Klinik und Pathologie der Dystrophia myotonica , Ztschr. ges. Neurol. u. Psychiat. 108:686, 1927. 13. Guillain, G.; Bertrand, I., and Roquès, L.: Les lésions de la myotonie atrophique , Ann. de méd. 31:180 ( (Jan.) ) 1932 14. Étude neurologique, series 6, p. 386, 1936. 15. Keschner, M., and Davison, C.: Dystrophia Myotonica, Clinicopathologic Study , Arch. Neurol. & Psychiat. 30:1259 ( (Dec.) ) 1933. 16. Bielschowsky, M.; Maas, O., and Ostertag, B.: Über Dystrophia myotonica (Myotonie atrophique), Vol. Jubilaire Marinesco, pp. 71-89, 1933. 17. d'Antona, L., cited, Zentralbl. ges. Neurol. u. Psychiat. 101:137, 1942. 18. Sinnige, J. L. M., and den Hartog, B. J. C.: A Case of Dystrophia Myotonica Developing 15 Years After Poliomyelitis , Folia psychiat, neurol. et neurochir. neerl. (Brouwer Memorial Volume) , p. 421, 1950. 19. Greving, R., in Müller, L. R.: Lebensnerven und Lebenstriebe: Dritte wesentlich erweiterte Auflage des vegetativen Nervensystems , Berlin, Julius Springer, 1931, p. 115. 20. Spatz, H.; Diepen, R., and Gaupp, V.: Zur Anatomie des Infundibulum und des Tuber cinereum beim Kaninchen , Deutsche Ztschr. Nervenh. 159:229, 1948. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Neurology & Psychiatry American Medical Association

CLINICAL AND PATHOLOGICAL FINDINGS IN A CASE OF DYSTROPHIA MYOTONICA

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Publisher
American Medical Association
Copyright
Copyright © 1951 American Medical Association. All Rights Reserved.
ISSN
0096-6886
DOI
10.1001/archneurpsyc.1951.02320080062006
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Abstract

Abstract IN 1944 I1 described a typical case of dystrophia myotonica in a man aged 44. Death occurred in 1948, and autopsy was performed. An anatomical examination was made of the endocrine glands, the muscles and the central and peripheral nervous systems. CLINICAL FINDINGS The patient was extremely bald; the left eye was artificial, and the right eye revealed the remains of iridectomy and an operation for cataract. He presented the characteristic aspect of the facies myopathica, and the general appearance was that of typical Jammergestalt. Speech was unintelligible; the thyroid gland was not palpable. There was fairly general adiposity. The left testis was very small; the right testis was still smaller, being about the size of a marble. Both were soft. There was male distribution of hair. The extremities were thin and showed atrophy of the muscles. The left leg was thinner than the right. The calves and the References 1. den Hartog Jager, W. A.: Over dystrophia myotonica , Psychiat. en neurol. bl. , p. 1 ( (Jan.) ) 1944. 2. Rasmussen, A. T.: Percentage of Different Types of Cells in Male Adult Human Hypophysis , Am. J. Path. 5:263 ( (May) ) 1929. 3. Ruyter, J. H. C.: A New Staining Method for the Hypophysis , Acta neerl. Morph. , p. 180, 1943. 4. Clark, W. E. Le Gros; Beattie, J.; Riddoch, G., and Dott, N. M.: The Hypothalamus: Morphological, Functional, Clinical and Surgical Aspects , The Henderson Trust Lectures, nos. XIII-XVI, published for the William Ramsay Henderson Trust, Edinburgh, Oliver & Boyd, 1938. 5. Jakob, A.: Die extrapyramidalen Erkrankungen , Berlin, Julius Springer, 1923. 6. Steinert, H.: Myopathologische Beiträge , Deutsche Ztschr. Nervenh. 37:58, 1909.Crossref 7. Hoffmann, J.: Vergaderingsbericht , München, med. Wchnschr. 66:519, 1919. 8. Fischer, L.: Klinische, psychopathologische und anatomische Beiträge zur Dystrophia myotonica , Ztschr. ges. Neurol. u. Psychiat. 58:254, 1920.Crossref 9. Hitzenberger, R.: Über myotonische Dystrophie , Monatsschr. Psychiat. u. Neurol. 47:249, 1920.Crossref 10. Bramwell, E.: Case of Myotonia Atrophica with Autopsy , Proc. Roy. Soc. Med. 16(Sec. Neurol.): 11, 1923. 11. Adie, W. J., and Greenfield, J. G.: Dystrophia Myotonica , Brain 46:73, 1923.Crossref 12. Weil, A., and Keschner, M.: Ein Beitrag zur Klinik und Pathologie der Dystrophia myotonica , Ztschr. ges. Neurol. u. Psychiat. 108:686, 1927. 13. Guillain, G.; Bertrand, I., and Roquès, L.: Les lésions de la myotonie atrophique , Ann. de méd. 31:180 ( (Jan.) ) 1932 14. Étude neurologique, series 6, p. 386, 1936. 15. Keschner, M., and Davison, C.: Dystrophia Myotonica, Clinicopathologic Study , Arch. Neurol. & Psychiat. 30:1259 ( (Dec.) ) 1933. 16. Bielschowsky, M.; Maas, O., and Ostertag, B.: Über Dystrophia myotonica (Myotonie atrophique), Vol. Jubilaire Marinesco, pp. 71-89, 1933. 17. d'Antona, L., cited, Zentralbl. ges. Neurol. u. Psychiat. 101:137, 1942. 18. Sinnige, J. L. M., and den Hartog, B. J. C.: A Case of Dystrophia Myotonica Developing 15 Years After Poliomyelitis , Folia psychiat, neurol. et neurochir. neerl. (Brouwer Memorial Volume) , p. 421, 1950. 19. Greving, R., in Müller, L. R.: Lebensnerven und Lebenstriebe: Dritte wesentlich erweiterte Auflage des vegetativen Nervensystems , Berlin, Julius Springer, 1931, p. 115. 20. Spatz, H.; Diepen, R., and Gaupp, V.: Zur Anatomie des Infundibulum und des Tuber cinereum beim Kaninchen , Deutsche Ztschr. Nervenh. 159:229, 1948.

Journal

A.M.A. Archives of Neurology & PsychiatryAmerican Medical Association

Published: Aug 1, 1951

References