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BILATERAL ACOUSTIC NEUROFIBROMAS: FURTHER CLINICAL AND PATHOLOGIC DATA ON HEREDITARY DEAFNESS AND RECKLINGHAUSEN'S DISEASE

BILATERAL ACOUSTIC NEUROFIBROMAS: FURTHER CLINICAL AND PATHOLOGIC DATA ON HEREDITARY DEAFNESS AND... Abstract In 1930, Gardner and Frazier1 reported a family in which von Recklinghausen's disease, in the form of bilateral tumors of the acoustic nerve, had been transmitted as a dominant mendelian trait through five generations. At the time of the original report, 38 of the 217 members of the family were affected. Necropsy was performed on 2 members of the family and revealed bilateral acoustic neurofibromas. After the publication of the first report, symptoms of a tumor of the spinal cord developed in 1 of the affected members. This tumor was removed and likewise proved to be a neurofibroma; the case was subsequently reported by Gardner.2 The present report, the third concerning this family, deals chiefly with the pathologic material obtained from 4 members since the last publication.3 One of these members (case 4) represents the only case thus far observed in which the disease has been recognized References 1. Gardner, W. J., and Frazier, C. H.: Bilateral Acoustic Neurofibromas: A Clinical Study and Field Survey of a Family of Five Generations with Bilateral Deafness in Thirty-Eight Members , Arch. Neurol. & Psychiat. 23:266-300 ( (Feb.) ) 1930 2. Hereditary Deafness Due to Bilateral Acoustic Tumors: Clinical Study and Field Survey of a Family of Five Generations with a History of Bilateral Deafness in Thirty-Eight Members , Ann. Otol., Rhin. & Laryng. 39:974-986 ( (Dec.) ) 1930. 3. Gardner, W. J.: Tumor of the Spinal Cord Associated with Bilateral Acoustic Tumors: Report of a Case , Arch. Neurol. & Psychiat. 24:1014-1022 ( (Nov.) ) 1930. 4. The histologic sections were stained with hematoxylin and eosin and phosphotungstic acid hematoxylin and by Perdrau's silver impregnation method for connective tissue, Fincher's silver technic, Cajal's and Bielschowsky's silver methods for nerve fibers and, in some instances, by various stains for myelin sheaths. Some of the preparations, particularly those of necropsy material, suffered from the fact that the tissue was obtained long after death. However, in no instance were the postmortem changes so great as to interfere with a relatively detailed study of the tissue or to prevent recognition of the tumor type. 5. Gardner, W. J., and Turner, O. A.: Multiple Intracranial Tumors: A Discussion of the Relationship of Meningeal to Acoustic Tumors and a Report of a Case , J. A. M. A. 113:111-113 ( (July 8) ) 1939. 6. Cushing, H., and Eisenhardt, L.: Meningiomas: Their Classification, Regional Behavior, Life History, and Surgical End Results , Springfield, Ill., Charles C. Thomas, Publisher, 1938. 7. von Recklinghausen, F.: Ueber die multiplen Fibrome der Haut und ihre Beziehung zu den multiplen Neuromen , Berlin, A. Hirschwald, 1882. 8. Thomson, A.: On Neuroma and Neurofibromatosis , Edinburgh, Turnbull & Spears, 1900. 9. Struwe, F., and Steuer, E. J.: Eine Recklinghausen-Familie. Klinische und anatomische Untersuchungen , Ztschr. f. d. ges. Neurol. u. Psychiat. 125:748-790, 1930. 10. Minski, L.: Familial Bilateral Acoustic Tumours , J. Neurol. & Psychopath. 12:289-308 ( (April) ) 1932. 11. Schaltenbrand, G.: Sobre una familia con enfermedad de Recklinghausen , Prensa méd. argent. 20:1011-1026 ( (May 10) ) 1933. 12. Roger, H.; Alliez, J., and Sarradon, P.: Tumeur bilatérale de l'acoustique à forme familiale et héréditaire , Bull. et mém. Soc. méd. d. hôp. de Paris 50:235-240 ( (Feb. 19) ) 1934. 13. Stallard, H. B.: A Case of Intra-Ocular Neuroma (von Recklinghausen's Disease) of the Left Optic Nerve Head , Brit. J. Ophth. 22:11-18 ( (Jan.) ) 1938. 14. Turner, O. A., and Gardner, W. J.: Familial Involvement of the Nervous System by Multiple Tumors of the Sheaths and Enveloping Membranes: Hereditary, Clinical and Pathological Study of Central and Peripheral Neurofibromatosis , Am. J. Cancer 32:339-360 ( (March) ) 1938. 15. Penfield, W.: Tumors of the Sheaths of the Nervous System , in Cytology and Cellular Pathology of the Nervous System , New York, Paul B. Hoeber, Inc., 1932, vol. 3, pp. 955-990. 16. Footnote deleted. 17. Berggrün, E.: Ein Fall von allgemeiner Neurofibromatose bei einem 11jährigen Knaben , Arch. f. Kinderh. 21:89-113, 1896. 18. Hardy, M., and Crowe, S. J.: Early Asymptomatic Acoustic Tumor: Report of Six Cases , Arch. Surg. 32:292-301 ( (Feb.) ) 1936. 19. Fowler, E. P.: Acoustic Tumors Within Internal Auditory Meatus , Laryngoscope 46:616-627 ( (Aug.) ) 1936. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology & Psychiatry American Medical Association

BILATERAL ACOUSTIC NEUROFIBROMAS: FURTHER CLINICAL AND PATHOLOGIC DATA ON HEREDITARY DEAFNESS AND RECKLINGHAUSEN'S DISEASE

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References (21)

Publisher
American Medical Association
Copyright
Copyright © 1940 American Medical Association. All Rights Reserved.
ISSN
0096-6754
DOI
10.1001/archneurpsyc.1940.02280070084004
Publisher site
See Article on Publisher Site

Abstract

Abstract In 1930, Gardner and Frazier1 reported a family in which von Recklinghausen's disease, in the form of bilateral tumors of the acoustic nerve, had been transmitted as a dominant mendelian trait through five generations. At the time of the original report, 38 of the 217 members of the family were affected. Necropsy was performed on 2 members of the family and revealed bilateral acoustic neurofibromas. After the publication of the first report, symptoms of a tumor of the spinal cord developed in 1 of the affected members. This tumor was removed and likewise proved to be a neurofibroma; the case was subsequently reported by Gardner.2 The present report, the third concerning this family, deals chiefly with the pathologic material obtained from 4 members since the last publication.3 One of these members (case 4) represents the only case thus far observed in which the disease has been recognized References 1. Gardner, W. J., and Frazier, C. H.: Bilateral Acoustic Neurofibromas: A Clinical Study and Field Survey of a Family of Five Generations with Bilateral Deafness in Thirty-Eight Members , Arch. Neurol. & Psychiat. 23:266-300 ( (Feb.) ) 1930 2. Hereditary Deafness Due to Bilateral Acoustic Tumors: Clinical Study and Field Survey of a Family of Five Generations with a History of Bilateral Deafness in Thirty-Eight Members , Ann. Otol., Rhin. & Laryng. 39:974-986 ( (Dec.) ) 1930. 3. Gardner, W. J.: Tumor of the Spinal Cord Associated with Bilateral Acoustic Tumors: Report of a Case , Arch. Neurol. & Psychiat. 24:1014-1022 ( (Nov.) ) 1930. 4. The histologic sections were stained with hematoxylin and eosin and phosphotungstic acid hematoxylin and by Perdrau's silver impregnation method for connective tissue, Fincher's silver technic, Cajal's and Bielschowsky's silver methods for nerve fibers and, in some instances, by various stains for myelin sheaths. Some of the preparations, particularly those of necropsy material, suffered from the fact that the tissue was obtained long after death. However, in no instance were the postmortem changes so great as to interfere with a relatively detailed study of the tissue or to prevent recognition of the tumor type. 5. Gardner, W. J., and Turner, O. A.: Multiple Intracranial Tumors: A Discussion of the Relationship of Meningeal to Acoustic Tumors and a Report of a Case , J. A. M. A. 113:111-113 ( (July 8) ) 1939. 6. Cushing, H., and Eisenhardt, L.: Meningiomas: Their Classification, Regional Behavior, Life History, and Surgical End Results , Springfield, Ill., Charles C. Thomas, Publisher, 1938. 7. von Recklinghausen, F.: Ueber die multiplen Fibrome der Haut und ihre Beziehung zu den multiplen Neuromen , Berlin, A. Hirschwald, 1882. 8. Thomson, A.: On Neuroma and Neurofibromatosis , Edinburgh, Turnbull & Spears, 1900. 9. Struwe, F., and Steuer, E. J.: Eine Recklinghausen-Familie. Klinische und anatomische Untersuchungen , Ztschr. f. d. ges. Neurol. u. Psychiat. 125:748-790, 1930. 10. Minski, L.: Familial Bilateral Acoustic Tumours , J. Neurol. & Psychopath. 12:289-308 ( (April) ) 1932. 11. Schaltenbrand, G.: Sobre una familia con enfermedad de Recklinghausen , Prensa méd. argent. 20:1011-1026 ( (May 10) ) 1933. 12. Roger, H.; Alliez, J., and Sarradon, P.: Tumeur bilatérale de l'acoustique à forme familiale et héréditaire , Bull. et mém. Soc. méd. d. hôp. de Paris 50:235-240 ( (Feb. 19) ) 1934. 13. Stallard, H. B.: A Case of Intra-Ocular Neuroma (von Recklinghausen's Disease) of the Left Optic Nerve Head , Brit. J. Ophth. 22:11-18 ( (Jan.) ) 1938. 14. Turner, O. A., and Gardner, W. J.: Familial Involvement of the Nervous System by Multiple Tumors of the Sheaths and Enveloping Membranes: Hereditary, Clinical and Pathological Study of Central and Peripheral Neurofibromatosis , Am. J. Cancer 32:339-360 ( (March) ) 1938. 15. Penfield, W.: Tumors of the Sheaths of the Nervous System , in Cytology and Cellular Pathology of the Nervous System , New York, Paul B. Hoeber, Inc., 1932, vol. 3, pp. 955-990. 16. Footnote deleted. 17. Berggrün, E.: Ein Fall von allgemeiner Neurofibromatose bei einem 11jährigen Knaben , Arch. f. Kinderh. 21:89-113, 1896. 18. Hardy, M., and Crowe, S. J.: Early Asymptomatic Acoustic Tumor: Report of Six Cases , Arch. Surg. 32:292-301 ( (Feb.) ) 1936. 19. Fowler, E. P.: Acoustic Tumors Within Internal Auditory Meatus , Laryngoscope 46:616-627 ( (Aug.) ) 1936.

Journal

Archives of Neurology & PsychiatryAmerican Medical Association

Published: Jul 1, 1940

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