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Juvenile Amaurotic Idiocy

Juvenile Amaurotic Idiocy Abstract Juvenile amaurotic idiocy (Vogt-Spielmeyer's disease) was first recognized in 1826 by Stengel,1 a Norwegian physician. The descriptions of Batten,2 Vogt,3 and Spielmeyer4 some 50 years ago, clearly established the basic clinical and pathological aspects of the disease and, 25 years later, thorough study of a large number of cases which were observed in Sweden5,6 contributed much to the genetic, clinical and pathological understanding of the disease. In this country, juvenile amaurotic idiocy seems to have attracted much less attention than the infantile type (Tay-Sachs' disease) and only a few reports are available in the medical literature. Yet the disease is not as rare as commonly thought. In fact, from personal observation, the incidence of the juvenile form (9 cases) is not far off from the incidence of the infantile form (15 cases). This ratio of 6:10 was observed in a hospital where there is a References 1. Stengel, C. (1826) Reprinted in; Nissen, A. J. Juvenil amaurotisk idioti i Norge . Nord. Med. 52:1542-1546. 1954. 2. Batten, F. E.: Cerebral degeneration with symmetrical changes in the maculae in two members of a family . Trans, Ophth. Soc. U. Kingdom , 23:386-390, 1903. 3. Vogt, H.: Über familiäre amaurotische idiote and verwandte Krankheitsbilder . Monatschr. f. Psychiat. u. Neurol. , 18:161-171, 310-357, 1906.Crossref 4. Spielmeyer, W.: Histologische und histopathologische Arbeiten uber die Grosshirnrinde. Klinische und anatomische Untersuchurgen über eine besondere Form von Familiarer amaurotischen Idiotie . Nissl Arbeit. , 2:193-213, 1908. 5. Sjögren, T: Die juvenile amaurotische Idiotie . Hereditas , 14:197-426, 1931.Crossref 6. Sjövall, E., and Ericsson, E.: Anatomical type in Swedish cases of juvenile amaurotic idiocy . Acta Path. Scand. (Supp.) , 16:460-471, 1933. 7. Hoffman, J.: Pigmentary retinal lipoid neuronal heredodegeneration (Spielmeye r-Vogt disease) . Electroenceph. Clin. Neurophysiol. , 8: 506-512, 1956.Crossref 8. Cordes, F. C., and Horner, W. D.: Infantile amaurotic idiocy in two Japanese families Am. J. Optholm. , 52:558-561, 1929. 9. DeSilva, C. C., and Tennekoow, G. E.: Tay-Sachs' disease in two Sinahlese children . Brit. Med. Jour. , 11:768-770, 1955.Crossref 10. Corberi, G.: Sulle regressioni mentali nell età infantile e nell età giovanile . Riv. Sper, Freniatria , 50:566-569, 1926. 11. Korey, S. R.: This Symposium. 12. Zierl, F.: Über Skeletveränderungen bei der juvenilen Form der amaurotischen Idiotie . Ztsch. f. ges. Neur. u. Psychiat. , 131:400-420, 1930.Crossref 13. Franceschetti, A.; Klein, D., and Babel, J.: Les manifestations oculaires des troubles primitifs du métabolisme des lipides. Etude clinique, génétique et anatomo-pathologique . Arq. Neuropsiquiat (S. Paolo) , 13:69-160, 1955. 14. Cobb, W.; Martin, F., and Pampiglione, G.: Cerebral lipidoses: electroencephalographic study . Brain , 75:343-357, 1952.Crossref 15. Jefferson, M., and Rutter, M. L.: A report of two cases of the juvenile form of amaurotic familial idiocy (cerebromacular degeneration) . J. Neur. Neurosurg. and Psychiat. , 21:31-37, 1958.Crossref 16. Frenkel, H., and Dide, M.: Retinite pigmentaire avec atrophie papillaire et atoxie céré belleuse familiales , Neur. , 25:729-734, 1913. 17. Liebers, M.: Zur Histopathologie der amaurotischer Idiotie und Myoklonusepilepsie , Ztsch. f. ges. Neur. u. Psychiat. , 111:465-484, 1927.Crossref 18. Wyburn-Mason, R.: On some anomalous forms of amaurotic idiocy and their bearing on relationship of various types . Brit. J. Ophthalm. , 27:145 and 193, 1943.Crossref 19. Rayner, S.: Juvenile amaurotic idiocy: diagnosis of heterozygotes . Acta Gen. et Stat. Med. , 3:1-5, 1952. 20. Diezel, P. B.: Histochemische Untersuchungen an primären Lipoidosen: amaurotische Idiotie, Gargoyhsmus, Niemann-Picksche Krankheit, Gauchersche Krankheit, mit besunderer Berucksichtigung des Zentrolnervensystems . Virchows Arch. , 326:89-118, 1954.Crossref 21. Klenk, E., and Langerbeins, H.: Über die Verteilung der Neuramin Saure in Gehirn (mit einer Mikromekhode zur quantitativen Bestimmung der Substanz in Nervengewebe) . Ztsch. physiol. Chem. , 270:185-193, 1941.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Journal of Diseases of Children American Medical Association

Juvenile Amaurotic Idiocy

A.M.A. Journal of Diseases of Children , Volume 97 (5_PART_II) – May 1, 1959

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References (27)

Publisher
American Medical Association
Copyright
Copyright © 1959 American Medical Association. All Rights Reserved.
ISSN
0096-6916
DOI
10.1001/archpedi.1959.02070010665003
Publisher site
See Article on Publisher Site

Abstract

Abstract Juvenile amaurotic idiocy (Vogt-Spielmeyer's disease) was first recognized in 1826 by Stengel,1 a Norwegian physician. The descriptions of Batten,2 Vogt,3 and Spielmeyer4 some 50 years ago, clearly established the basic clinical and pathological aspects of the disease and, 25 years later, thorough study of a large number of cases which were observed in Sweden5,6 contributed much to the genetic, clinical and pathological understanding of the disease. In this country, juvenile amaurotic idiocy seems to have attracted much less attention than the infantile type (Tay-Sachs' disease) and only a few reports are available in the medical literature. Yet the disease is not as rare as commonly thought. In fact, from personal observation, the incidence of the juvenile form (9 cases) is not far off from the incidence of the infantile form (15 cases). This ratio of 6:10 was observed in a hospital where there is a References 1. Stengel, C. (1826) Reprinted in; Nissen, A. J. Juvenil amaurotisk idioti i Norge . Nord. Med. 52:1542-1546. 1954. 2. Batten, F. E.: Cerebral degeneration with symmetrical changes in the maculae in two members of a family . Trans, Ophth. Soc. U. Kingdom , 23:386-390, 1903. 3. Vogt, H.: Über familiäre amaurotische idiote and verwandte Krankheitsbilder . Monatschr. f. Psychiat. u. Neurol. , 18:161-171, 310-357, 1906.Crossref 4. Spielmeyer, W.: Histologische und histopathologische Arbeiten uber die Grosshirnrinde. Klinische und anatomische Untersuchurgen über eine besondere Form von Familiarer amaurotischen Idiotie . Nissl Arbeit. , 2:193-213, 1908. 5. Sjögren, T: Die juvenile amaurotische Idiotie . Hereditas , 14:197-426, 1931.Crossref 6. Sjövall, E., and Ericsson, E.: Anatomical type in Swedish cases of juvenile amaurotic idiocy . Acta Path. Scand. (Supp.) , 16:460-471, 1933. 7. Hoffman, J.: Pigmentary retinal lipoid neuronal heredodegeneration (Spielmeye r-Vogt disease) . Electroenceph. Clin. Neurophysiol. , 8: 506-512, 1956.Crossref 8. Cordes, F. C., and Horner, W. D.: Infantile amaurotic idiocy in two Japanese families Am. J. Optholm. , 52:558-561, 1929. 9. DeSilva, C. C., and Tennekoow, G. E.: Tay-Sachs' disease in two Sinahlese children . Brit. Med. Jour. , 11:768-770, 1955.Crossref 10. Corberi, G.: Sulle regressioni mentali nell età infantile e nell età giovanile . Riv. Sper, Freniatria , 50:566-569, 1926. 11. Korey, S. R.: This Symposium. 12. Zierl, F.: Über Skeletveränderungen bei der juvenilen Form der amaurotischen Idiotie . Ztsch. f. ges. Neur. u. Psychiat. , 131:400-420, 1930.Crossref 13. Franceschetti, A.; Klein, D., and Babel, J.: Les manifestations oculaires des troubles primitifs du métabolisme des lipides. Etude clinique, génétique et anatomo-pathologique . Arq. Neuropsiquiat (S. Paolo) , 13:69-160, 1955. 14. Cobb, W.; Martin, F., and Pampiglione, G.: Cerebral lipidoses: electroencephalographic study . Brain , 75:343-357, 1952.Crossref 15. Jefferson, M., and Rutter, M. L.: A report of two cases of the juvenile form of amaurotic familial idiocy (cerebromacular degeneration) . J. Neur. Neurosurg. and Psychiat. , 21:31-37, 1958.Crossref 16. Frenkel, H., and Dide, M.: Retinite pigmentaire avec atrophie papillaire et atoxie céré belleuse familiales , Neur. , 25:729-734, 1913. 17. Liebers, M.: Zur Histopathologie der amaurotischer Idiotie und Myoklonusepilepsie , Ztsch. f. ges. Neur. u. Psychiat. , 111:465-484, 1927.Crossref 18. Wyburn-Mason, R.: On some anomalous forms of amaurotic idiocy and their bearing on relationship of various types . Brit. J. Ophthalm. , 27:145 and 193, 1943.Crossref 19. Rayner, S.: Juvenile amaurotic idiocy: diagnosis of heterozygotes . Acta Gen. et Stat. Med. , 3:1-5, 1952. 20. Diezel, P. B.: Histochemische Untersuchungen an primären Lipoidosen: amaurotische Idiotie, Gargoyhsmus, Niemann-Picksche Krankheit, Gauchersche Krankheit, mit besunderer Berucksichtigung des Zentrolnervensystems . Virchows Arch. , 326:89-118, 1954.Crossref 21. Klenk, E., and Langerbeins, H.: Über die Verteilung der Neuramin Saure in Gehirn (mit einer Mikromekhode zur quantitativen Bestimmung der Substanz in Nervengewebe) . Ztsch. physiol. Chem. , 270:185-193, 1941.Crossref

Journal

A.M.A. Journal of Diseases of ChildrenAmerican Medical Association

Published: May 1, 1959

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