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Lower Motor and Primary Sensory Neuron Diseases With Peroneal Muscular Atrophy: I. Neurologic, Genetic, and Electrophysiologic Findings in Hereditary Polyneuropathies

Lower Motor and Primary Sensory Neuron Diseases With Peroneal Muscular Atrophy: I. Neurologic,... Abstract THIS report gives the neurologic, genetic, and electrophysiologic findings of a prospective study of kinships with different hereditary neurologic disorders having symmetric neurogenic weakness and atrophy as an early- and often major manifestation. The study was undertaken to obtain more reliable information about the natural history of these disorders, to test the usefulness of electro physiologic studies in distinguishing affected from nonaffected persons, to compare the results of nerve biopsies from representative affected persons with these disorders, and to develop a more meaningful classification. In this, the first of two articles on the subject, the results of neurologic, genetic, and electrophysiologic studies of 67 persons with hypertrophic neuropathy of the Charcot-Marie-Tooth type, five persons with hypertrophic neuropathy of the Dejerine-Sottas type, and 150 unaffected relatives are given. We also wished to determine whether these syndromes were phenotypic variations of one or several diseases or were separate entities on the References 1. Charcot, J.M., and Marie, P.: Sur une forme particulière d'atrophie musculaire progressive souvent familial débutant par les pieds et les jambes et atteignant plus tard les mains , Rev Med (Paris) 6:97-138 ( (Feb) ) 1886. 2. Tooth, H.H.: The Peroneal Type of Progressive Muscular Atrophy, thesis, London: H. K. Lewis & Co., Ltd., 1886. 3. Virchow, R.: Ein Fall von progressiver Muskelatrophie , Virchow Arch Path Anat 8:537, 1855.Crossref 4. Eulenburg, M.: Ueber progressive Muskelatrophie , Deutsch Klin (Berlin) 8:129-131, 1856. 5. Friedreich, N.: Ueber progressive Muskelatrophie, über wahre und falsche Muskelhypertrophie , Berlin: A. Hirschwald, 1873. 6. Bamberger: cited by Schultze, F.9 7. Hemptenmacher: cited by Schultze, F.9 8. Eichhorst, H.: Ueber Heredität der progressiven Muskelatrophie , Berlin Klin Wschr 10:497-499 (Oct 20); 511-514 ( (Oct 27) ) 1873. 9. Schultze, F.: Über die vererbbare neurale oder neurospinale Muskelatrophie , Deutsch Z Nervenheilk 112:1-19 ( (Jan) ) 1930.Crossref 10. Hoffmann, J.: Ueber chronische spinale Muskelatrophie im Kindesalter, auf familiärer Basis , Deutsch Z Nervenheilk 3:427-470 ( (May) ) 1893.Crossref 11. England, A.C., and Denny-Brown, D.: Severe Sensory Changes, and Trophic Disorder, in Peroneal Muscular Atrophy (Charcot-Marie-Tooth Type) , Arch Neurol 67:1-17 ( (Jan) ) 1952.Crossref 12. Lidge, R.T., and Chandler, F.A.: Charcot-Marie-Tooth Disease , J Pediat 43:152-163 ( (Aug) ) 1953.Crossref 13. Bell, J.: " On the Peroneal Type of Progressive Muscular Atrophy ," in Fisher, R.A. (ed.): Treasury of Human Inheritance: Nervous Diseases and Muscular Dystrophies , London: Cambridge University Press, 1935, vol 4, pp 69-139. 14. Marinesco, M.: Contribution à l'étude de l'amyotrophie Charcot-Marie , Arch Med Exp 6:921-965, 1894. 15. Biemond, A.: Neurotische Muskelatrophie und Friedreichsche Tabes in derselben Familie , Deutsch Z Nervenheilk 104:113-145 ( (June) ) 1928.Crossref 16. Haase, G.R., and Shy, G.M.: Pathological Changes in Muscle Biopsies From Patients With Peroneal Muscular Atrophy , Brain 83:631-637 ( (Dec) ) 1960.Crossref 17. Drachman, D.B., et al: "Myopathic" Changes in Chronically Denervated Muscle , Arch Neurol 19:14-24 ( (Jan) ) 1967.Crossref 18. Dyck, P.J.; Beahrs, O.H.; and Miller, R.H.: Peripheral Nerves in Hereditary Neural Atrophies: Number and Diameters of Myelinated Fibers. Communications from the Sixth International Congress of Electroencephalography and Clinical Neurophysiology, Vienna, Sept 5-10, 1965, pp 673-677. 19. Dyck, P.J., and Lambert, E.H.: Numbers and Diameters of Nerve Fibers and Compound Action Potential of Sural Nerve: Controls and Hereditary Neuromuscular Disorders , Trans Amer Neurol Assoc 91:214-217, 1966. 20. Dyck, P.J.: Histologic Measurements and Fine Structure of Biopsied Sural Nerve: Normal, and in Peroneal Muscular Atrophy, Hypertrophic Neuropathy, and Congenital Sensory Neuropathy , Mayo Clin Proc 41:742-774 ( (Nov) ) 1966. 21. Gutrecht, J.A., and Dyck, P.J.: Segmental Demyelinization in Peroneal Muscular Atrophy: Nerve Fibers Teased From Sural Nerve Biopsy Specimens , Mayo Clin Proc 41:775-777 ( (Nov) ) 1966. 22. Dyck, P.J., et al: Histologic and Teased-Fiber Measurements of Sural Nerve in Disorders of Lower Motor and Primary Sensory Neurons , Mayo Clin Proc 43:81-123 ( (Feb) ) 1968. 23. Dejerine, J., and Sottas, J.: Sur la névrite interstitielle, hypertrophique et progressive de l'enfance , C R Soc Biol 45:63-96, 1893. 24. Dejerine, J.: Névrite interstitielle hypertrophique et progressive de l'enfance , Rev Med 16:881-925 ( (Nov) ) 1896. 25. Boveri, P.: De la nevrite hypertrophique familiale (type Pierre Marie) , Sem Med (Paris) 30:145-150, 1910. 26. Hoffman, J.: Über progressive hypertrophische Neuritis , Deutsch Z Nervenheilk 44:65-94, 1912.Crossref 27. Yokomori, K.: Über Neuritis interstitialis hypertrophica et progressiva (Déjérine and Sottas) mit einem Sektionsbefund , Mitt Med Fakult Univ Tokyo 15:1-48, 1915-1916. 28. Cornil, L.; Chalnot, R.; and Thomas: Névrite hypertrophique progressive non familiale (étude anatomoclinique) , Rev Neurol 1:1187-1192 ( (June) ) 1930. 29. Sears, W.G.: Progressive Hypertrophic Poly-neuritis , J Neurol Psychopath 12:137-147 ( (Oct) ) 1931.Crossref 30. Russell, W.R., and Garland, H.G.: Progressive Hypertrophic Polyneuritis With Case Reports , Brain 53:376-384 ( (Oct) ) 1930.Crossref 31. Villaret, M.; Haguenau, J.; and Klotz, P.H.: Névrite hypertrophique familiale , Rev Neurol 63:211-218 ( (Feb) ) 1935. 32. Cooper, E.L.: Progressive Familial Hypertrophic Neuritis (Dejerine-Sottas) , Brit Med J 1:793-794 ( (April 18) ) 1936.Crossref 33. André-van Leeuwen, M., and Moreau, M.: De la valeur des troubles pupillaires en dehors de la syphilis, comme manifestation d'une affection hérédo-dégénerative: IV. Une souche d'amyotrophie neurale (type névrite hypertrophique) avec troubles pupillaires: Rapports avec la pupillotonie (Adie) et la dystasie aréflexique (Roussy-Lévy) , Mschr Psychiat Neurol 112:121-168, 1946.Crossref 34. Bruns, G.: Zur Kenntnis der hypertrophischen Neuritis (Roussy-Cornil) , Beitr Path Anat 111:407-418 ( (June) ) 1951. 35. Luban, B.: 2. Neurale Muskelatrophie und hypertrophische Neuritis: Klinischer und genealogischer Beitrag anhand einer bernischen Sippe , Schweiz Arch Neurol Psychiat 68:34-63, 1952. 36. Creutzfeldt, H.G.; Curtius, F.; and Krüger, K.H.: Zur Klinik, Histologie und Genealogie der Déjerine-Sottasschen Krankheit , Arch Psychiat Nervenkr 186:341-360, 1951.Crossref 37. Austin, J.H.: Observations on the Syndrome of Hypertrophic Neuritis (The Hypertrophic Interstitial Radiculoneuropathies) , Medicine (Balt) 35:187-237 ( (Sept) ) 1956.Crossref 38. Bedford, P.D., and James, F.E.: A Family With the Progressive Hypertrophic Polyneuritis of Dejerine and Sottas , J Neurol Neurosurg Psychiat 19:46-51 ( (Feb) ) 1956.Crossref 39. Isaacs, H.: Familial Chronic Hypertrophic Polyneuropathy With Paralysis of the Extremities in Cold Weather , S Afr Med J 34:758-761 ( (Sept 3) ) 1960. 40. Webster, H.deF., et al: The Role of Schwann Cells in the Formation of "Onion Bulbs" Found in Chronic Neuropathies , J Neuropath Exp Neurol 26:276-299 ( (April) ) 1967.Crossref 41. Lambert, E.H.: Clinical Examinations in Neurology , Philadelphia: W. B. Saunders Company, 1956, pp 287-317. 42. Gilliatt, R.W., and Thomas, P.K.: Extreme Slowing of Nerve Conduction in Peroneal Muscular Atrophy , Ann Phys Med 4:104-106 ( (Aug) ) 1957. 43. Lambert, E.H.; Bastron, J.A.; and Mulder, D.W.: Conduction Velocity of Motor Fibers of Peripheral Nerves in Peroneal Muscular Atrophy (C.M.T. Disease), read before the annual meeting of the American Academy of Neurology, Philadelphia, April 19, 1958. 44. Christie, B.G.: Electrodiagnostic Features of Charcot-Marie-Tooth Disease , Proc Roy Soc Med 54:321-324 ( (April) ) 1961. 45. Blom, S.; Hagbarth, K.E.; and Lundberg, P.O.: Motor Conduction Velocities in Amyotrophic Lateral Sclerosis, Polyradiculoneuritis and Charcot-Marie-Toooth's Disease , Acta Neurol Scand 40:6-12, 1964.Crossref 46. Dyck, P.J.; Lambert, E.H.; and Mulder, D.W.: Charcot-Marie-Tooth Disease: Nerve Conduction and Clinical Studies of a Large Kinship , Neurology (Minneap) 13:1-11 ( (Jan) ) 1963.Crossref 47. Amick, L.D., and Lemmi, H.: Electromyographic Studies in Peroneal Muscular Atrophy: Charcot-Marie-Tooth Disease , Arch Neurol 9:273-384 ( (Sept) ) 1963.Crossref 48. Myrianthopoulos, N.C., et al: Nerve Conduction and Other Studies in Families With Charcot-Marie-Tooth Disease , Brain 87( (pt 4) ):589-608, 1964.Crossref 49. Roussy, G., and Levy, G.: Sept cas d'une maladie familiale particuliere: Trouble de la marche, pieds bots et aréfleéxie tendineuse généralisée, avec accessoirement, légère maladresse des mains , Rev Neurol 1:427-450 ( (April) ) 1926. 50. Yudell, A.; Dyck, P.J.; and Lambert, E.H.: A Kinship With the Roussy-Levy Syndrome , Arch Neurol 13:432-440 ( (Oct) ) 1965.Crossref 51. Slauck: Über progressive hypertrophische Neuritis (Hoffmannsche Krankheit) , Z Neurol Psychiat 92:34-77, 1924.Crossref 52. Wolf, A.; Rubinowitz, A.H.; and Burchell; S.C.: Interstitial Hypertrophic Neuritis of Déjérine and Sottas: A Report of Three Cases , Bull Neurol Inst New York 2:373-428 ( (Nov) ) 1932. 53. Thomas, A., and Chausseblanche, L.: Un cas de névrite hypertrophique et progressive de l'enfance (maladie de Déjérine et Sottas) , Encephale 28:504-530 ( (July-Aug) ) 1933. 54. Massion-Verniory, L., and Radermecker, J.: Névrite hypertrophique interstitielle , Mschr Psychiat Neurol 113-114:246-257, 1947.Crossref 55. Marie, P.: Forme spéciale de névrite intersitielle hypertrophique progressive de l'enfance , Rev Neurol 14:557-560 ( (June) ) 1906. 56. Gombault, A., and Mallet: Un cas de tabès ayant débuté dans l'enfance—autopsie , Arch Med Exp Anat Path (Paris) 1:385-415, 1889. 57. Störring, E.: Über Pupillenstörungen bei neuralen Muskelatrophein: Zugleich ein Beitrag zur Kenntnis der hypertrophischen Neuritis Déjérine-Sottas , Z Neurol Psychiat 171:95-116 ( (Feb) ) 1941.Crossref 58. Long, E.: Atrophie musculaire progressive des membres supérieurs, type Aran-Duchenne par névrite interstitielle hypertrophique (contribution a l'étude des maladies d'evolution) , Nouv Iconog Salpetriere 20:46-65, 1907. 59. Long, E.: Atrophie musculaire progressive type Aran-Duchenne de nature névritique (second cas suivi d'autopsie) , Nouv Iconog Salpetriere 25:281-308, 1912. 60. Roussy, G., and Cornil, L.: Névrite hypertrophique progressive non-familial de l'adulte , Ann Med (Paris) 6:296-305, 1919. 61. Achard, C.H., and Thiers, J.: Polynévrite chronique hypertrophique de l'adulte , Rev Neurol 39:146-150, 1924. 62. Schaller, W.F., and Newmann, H.W.: Névrite interstitielle hypertrophique: Relation d'un cas avec suggestion de traitement , Rev Neurol 1:529-539 ( (April) ) 1935. 63. Tarassiéwitch, I.J., and Michéjew, W.: Nevrite hypertrophique et progressive (neuromegalia peripherica progressiva) , Rev Neurol 2:18-40 ( (July) ) 1935. 64. Sloane, P.: Progressive Interstitial Hypertrophic Neuritis , J Nerv Ment Dis 90:429-438 ( (Oct) ) 1939.Crossref 65. Garcin, R., et al: Sur deux cas sporadiques de névrite hypertrophique progressive de Dejerine-Sottas avec contrôle biopsique , Rev Neurol 82:204-210 ( (March) ) 1950. 66. Roger, H., et al: Polynévrite hypertrophique de l'adulte (non-familiale) avec paraparésie spasmodique , Rev Neurol 86:695-698 ( (July) ) 1952. 67. Juliao, O.F.: Etude sur la névrite hypertrophique progressive de Déjerine-Sottas , Arq Neuropsiguiat 10:221-246, 1952. 68. Ritz, O.: Zur Kenntnis der hypertrophischen Neuritis , Confin Neurol 21:257-271, 1961.Crossref 69. Andermann, F., et al: Observations on Hypertrophic Neuropathy of Déjerine and Sottas , Neurology (Minneap) 12:712-724 ( (Oct) ) 1962.Crossref 70. Rossolimo, G.: Sur une forme récurrente de la polynévrite interstitielle hypertrophique progressive de l'enfance (Dejerine) avec participation du nerf oculo-moteur externe , Rev Neurol 7:558-564, 1899. 71. Schaller, W.F.: Progressive Interstitial Hypertrophic Neuritis of Childhood of Dejerine and Sottas: Report of a Case , Arch Intern Med 10:399-404 ( (Oct) ) 1912.Crossref 72. Hoestermann, E.: Über rekurrierende Polyneuritis , Deutsch Z Nervenheilk 51:116-123 ( (May) ) 1914.Crossref 73. Nattrass, F.J.: Recurrent Hypertrophic Neuritis , J Neurol Psychopath 2:159-165 ( (Aug) ) 1921.Crossref 74. Harris, W., and Newcomb, W.D.: A Case of Relapsing Interstitial Hypertrophic Polyneuritis , Brain 52:108-116 ( (April) ) 1929.Crossref 75. Diddle, A.W., and Stephens, R.L.: Hypertrophic Interstitial Neuritis With Papilledema , Arch Neurol 40:151-157 ( (July) ) 1938.Crossref 76. Reisner, H., and Spiel, W.: Zur Frage der Polyneuritis hypertrophicans , Wein Z Nervenheilk 5:388-403, 1952. 77. Symonds, C.P., and Blackwood, W.: Spinal Cord Compression in Hypertrophic Neuritis , Brain 85:251-260 ( (June) ) 1962.Crossref 78. Green, L.; Herzog, I.; and Aberfeld, D.: A Case of Hypertrophic Interstitial Neuritis Coexisting With Dementia and Cerebellar Degeneration , J Neuropath Exp Neurol 24:682-689 ( (Oct) ) 1965.Crossref 79. Stewart, B.M.: The Hypertrophic Neuropathy of Acromegaly: A Rare Neuropathy Associated With Acromegaly , Arch Neurol 14:107-110 ( (Jan) ) 1966.Crossref 80. Thomas, P.K., and Lascelles, R.G.: The Pathology of Diabetic Neuropathy , Quart J Med 35:489-509 ( (Oct) ) 1966. 81. De Bruyn, R.S., and Stern, R.O.: A Case of the Progressive Hypertrophic Polyneuritis of Dejerine and Sottas, With Pathological Examination , Brain 52:84-107 ( (April) ) 1929.Crossref 82. Refsum, S.: Heredoataxia hemeralopica polyneuritiformis—et tidligere ikke beskrevet familiaert syndrom? Nord Med 28:2682-2685 ( (Dec) ) 1945. 83. Refsum, S.: Heredopathia Atactica Polyneuritiformis: A Familial Syndrome Not Hitherto Described , Acta Psychiat Neurol (suppl 38) , pp 1-303, 1946. 84. Cammermeyer, J.: Neuropathologic Changes in Hereditary Neuropathies: Manifestation of the Syndrome Heredopathia Atactica Polyneuritiformis in the Presence of Interstitial Hypertrophic Polyneuropathy , J Neuropath Exp Neurol 15:340-361 ( (July) ) 1956.Crossref 85. Reese, H., and Bareta, J.: Heredopathia Atactica Polyneuritiformis , J Neuropath Exp Neurol 9:385-395 ( (Oct) ) 1950.Crossref 86. Clark, D.B.: Heredopathia Atactica Polyneuritiformis (Refsum's Syndrome) , Proc Roy Soc Med 44:689-694, 1951. 87. Ashenhurst, E.M.; Millar, J.H.D.; and Milliken, T.G.: Refsum's Syndrome Affecting a Brother and Two Sisters , Brit Med J 2:415-417 ( (Aug 16) ) 1958.Crossref 88. Toussaint, D.; Coërs, C.; and Toppet, N.: Heredopathia atactica polyneuritiformis (syndrome de Refsum): Constatations cliniques et biopsiques , Bull Soc Belg Ophtal 122:383-402, 1959. 89. Klenk, E., and Kahlke, W.: Über das Vorkommen der 3.7.11.15-Tetramethyl-hexadecansäure Phytansäure) in den Cholesterinestern und anderen Lipoidfraktionen der Organe bei einem Krankheitsfall unbekannter Genese (Verdacht auf Heredopathia atactica polyneuritiformis [Refsum-Syndrom]) , Hoppe Seyler Z Physiol Chem 333:133-139, 1963.Crossref 90. Kahlke, W.: Über das Vorkommen von 3,7,11,15-Tetramethyl-hexadecansäure im Blutserum bei Refsum-Syndrom , Klin Wschr 41:783-785 ( (Aug) ) 1963.Crossref 91. Richterich, R.; van Mechelen, P.; and Rossi, E.: Refsum's Disease (Heredopathia Atactica Polyneuritiformis): An Inborn Error of Lipid Metabolism With Storage of 3,7,11,15-Tetramethyl Hexadecanoic Acid: I. Report of a Case , Amer J Med 39:230-236 ( (Aug) ) 1965.Crossref 92. Kahlke, W., and Richterich, R.: Refsum's Disease (Heredopathia Atactica Polyneuritiformis): An Inborn Error of Lipid Metabolism With Storage of 3,7,11,15-Tetramethyl Hexadecanoic Acid: II. Isolation and Identification of the Storage Product , Amer J Med 39:237-241 ( (Aug) ) 1965.Crossref 93. Kolodny, E.H., et al: Refsum's Syndrome: Report of a Case Including Electron Microscopic Studies of the Liver , Arch Neurol 12:583-596 ( (June) ) 1965.Crossref 94. Dawson, G.D., and Scott, J.W.: Recording of Nerve Action Potentials Through the Skin in Man , J Neurol Neurosurg Psychiat 12:259-267 ( (Nov) ) 1959.Crossref 95. Dyck, P.J., and Gomez, M.R.: Segmental Demyelinization in Dejerine-Sottas Disease: Light, Phase-Contrast, and Electron Microscopic Studies, to be published. 96. Thomas, P.K., and Lascelles, R.G.: Hypertrophic Neuropathy , Quart J Med 36:223-238 ( (April) ) 1967. 97. Nichols, P.C.; Dyck, P.J.; and Miller, D.R.: Experimental Hypertrophic Neuropathy: Change in Fascicular Area and Fiber Spectrum After Acute Crush Injury, to be published. 98. Symonds, C.P., and Shaw, M.E.: Familial Claw-Foot With Absent Tendon-Jerks: A "Forme Fruste" of the Charcot-Marie-Tooth Disease , Brain 49:387-403 ( (Sept) ) 1926.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Lower Motor and Primary Sensory Neuron Diseases With Peroneal Muscular Atrophy: I. Neurologic, Genetic, and Electrophysiologic Findings in Hereditary Polyneuropathies

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American Medical Association
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Copyright © 1968 American Medical Association. All Rights Reserved.
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0003-9942
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10.1001/archneur.1968.00470360025002
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Abstract

Abstract THIS report gives the neurologic, genetic, and electrophysiologic findings of a prospective study of kinships with different hereditary neurologic disorders having symmetric neurogenic weakness and atrophy as an early- and often major manifestation. The study was undertaken to obtain more reliable information about the natural history of these disorders, to test the usefulness of electro physiologic studies in distinguishing affected from nonaffected persons, to compare the results of nerve biopsies from representative affected persons with these disorders, and to develop a more meaningful classification. In this, the first of two articles on the subject, the results of neurologic, genetic, and electrophysiologic studies of 67 persons with hypertrophic neuropathy of the Charcot-Marie-Tooth type, five persons with hypertrophic neuropathy of the Dejerine-Sottas type, and 150 unaffected relatives are given. We also wished to determine whether these syndromes were phenotypic variations of one or several diseases or were separate entities on the References 1. Charcot, J.M., and Marie, P.: Sur une forme particulière d'atrophie musculaire progressive souvent familial débutant par les pieds et les jambes et atteignant plus tard les mains , Rev Med (Paris) 6:97-138 ( (Feb) ) 1886. 2. Tooth, H.H.: The Peroneal Type of Progressive Muscular Atrophy, thesis, London: H. K. Lewis & Co., Ltd., 1886. 3. Virchow, R.: Ein Fall von progressiver Muskelatrophie , Virchow Arch Path Anat 8:537, 1855.Crossref 4. Eulenburg, M.: Ueber progressive Muskelatrophie , Deutsch Klin (Berlin) 8:129-131, 1856. 5. Friedreich, N.: Ueber progressive Muskelatrophie, über wahre und falsche Muskelhypertrophie , Berlin: A. Hirschwald, 1873. 6. Bamberger: cited by Schultze, F.9 7. Hemptenmacher: cited by Schultze, F.9 8. Eichhorst, H.: Ueber Heredität der progressiven Muskelatrophie , Berlin Klin Wschr 10:497-499 (Oct 20); 511-514 ( (Oct 27) ) 1873. 9. Schultze, F.: Über die vererbbare neurale oder neurospinale Muskelatrophie , Deutsch Z Nervenheilk 112:1-19 ( (Jan) ) 1930.Crossref 10. Hoffmann, J.: Ueber chronische spinale Muskelatrophie im Kindesalter, auf familiärer Basis , Deutsch Z Nervenheilk 3:427-470 ( (May) ) 1893.Crossref 11. England, A.C., and Denny-Brown, D.: Severe Sensory Changes, and Trophic Disorder, in Peroneal Muscular Atrophy (Charcot-Marie-Tooth Type) , Arch Neurol 67:1-17 ( (Jan) ) 1952.Crossref 12. Lidge, R.T., and Chandler, F.A.: Charcot-Marie-Tooth Disease , J Pediat 43:152-163 ( (Aug) ) 1953.Crossref 13. Bell, J.: " On the Peroneal Type of Progressive Muscular Atrophy ," in Fisher, R.A. (ed.): Treasury of Human Inheritance: Nervous Diseases and Muscular Dystrophies , London: Cambridge University Press, 1935, vol 4, pp 69-139. 14. Marinesco, M.: Contribution à l'étude de l'amyotrophie Charcot-Marie , Arch Med Exp 6:921-965, 1894. 15. Biemond, A.: Neurotische Muskelatrophie und Friedreichsche Tabes in derselben Familie , Deutsch Z Nervenheilk 104:113-145 ( (June) ) 1928.Crossref 16. Haase, G.R., and Shy, G.M.: Pathological Changes in Muscle Biopsies From Patients With Peroneal Muscular Atrophy , Brain 83:631-637 ( (Dec) ) 1960.Crossref 17. Drachman, D.B., et al: "Myopathic" Changes in Chronically Denervated Muscle , Arch Neurol 19:14-24 ( (Jan) ) 1967.Crossref 18. Dyck, P.J.; Beahrs, O.H.; and Miller, R.H.: Peripheral Nerves in Hereditary Neural Atrophies: Number and Diameters of Myelinated Fibers. Communications from the Sixth International Congress of Electroencephalography and Clinical Neurophysiology, Vienna, Sept 5-10, 1965, pp 673-677. 19. Dyck, P.J., and Lambert, E.H.: Numbers and Diameters of Nerve Fibers and Compound Action Potential of Sural Nerve: Controls and Hereditary Neuromuscular Disorders , Trans Amer Neurol Assoc 91:214-217, 1966. 20. Dyck, P.J.: Histologic Measurements and Fine Structure of Biopsied Sural Nerve: Normal, and in Peroneal Muscular Atrophy, Hypertrophic Neuropathy, and Congenital Sensory Neuropathy , Mayo Clin Proc 41:742-774 ( (Nov) ) 1966. 21. Gutrecht, J.A., and Dyck, P.J.: Segmental Demyelinization in Peroneal Muscular Atrophy: Nerve Fibers Teased From Sural Nerve Biopsy Specimens , Mayo Clin Proc 41:775-777 ( (Nov) ) 1966. 22. Dyck, P.J., et al: Histologic and Teased-Fiber Measurements of Sural Nerve in Disorders of Lower Motor and Primary Sensory Neurons , Mayo Clin Proc 43:81-123 ( (Feb) ) 1968. 23. Dejerine, J., and Sottas, J.: Sur la névrite interstitielle, hypertrophique et progressive de l'enfance , C R Soc Biol 45:63-96, 1893. 24. Dejerine, J.: Névrite interstitielle hypertrophique et progressive de l'enfance , Rev Med 16:881-925 ( (Nov) ) 1896. 25. Boveri, P.: De la nevrite hypertrophique familiale (type Pierre Marie) , Sem Med (Paris) 30:145-150, 1910. 26. Hoffman, J.: Über progressive hypertrophische Neuritis , Deutsch Z Nervenheilk 44:65-94, 1912.Crossref 27. Yokomori, K.: Über Neuritis interstitialis hypertrophica et progressiva (Déjérine and Sottas) mit einem Sektionsbefund , Mitt Med Fakult Univ Tokyo 15:1-48, 1915-1916. 28. Cornil, L.; Chalnot, R.; and Thomas: Névrite hypertrophique progressive non familiale (étude anatomoclinique) , Rev Neurol 1:1187-1192 ( (June) ) 1930. 29. Sears, W.G.: Progressive Hypertrophic Poly-neuritis , J Neurol Psychopath 12:137-147 ( (Oct) ) 1931.Crossref 30. Russell, W.R., and Garland, H.G.: Progressive Hypertrophic Polyneuritis With Case Reports , Brain 53:376-384 ( (Oct) ) 1930.Crossref 31. Villaret, M.; Haguenau, J.; and Klotz, P.H.: Névrite hypertrophique familiale , Rev Neurol 63:211-218 ( (Feb) ) 1935. 32. Cooper, E.L.: Progressive Familial Hypertrophic Neuritis (Dejerine-Sottas) , Brit Med J 1:793-794 ( (April 18) ) 1936.Crossref 33. André-van Leeuwen, M., and Moreau, M.: De la valeur des troubles pupillaires en dehors de la syphilis, comme manifestation d'une affection hérédo-dégénerative: IV. Une souche d'amyotrophie neurale (type névrite hypertrophique) avec troubles pupillaires: Rapports avec la pupillotonie (Adie) et la dystasie aréflexique (Roussy-Lévy) , Mschr Psychiat Neurol 112:121-168, 1946.Crossref 34. Bruns, G.: Zur Kenntnis der hypertrophischen Neuritis (Roussy-Cornil) , Beitr Path Anat 111:407-418 ( (June) ) 1951. 35. Luban, B.: 2. Neurale Muskelatrophie und hypertrophische Neuritis: Klinischer und genealogischer Beitrag anhand einer bernischen Sippe , Schweiz Arch Neurol Psychiat 68:34-63, 1952. 36. Creutzfeldt, H.G.; Curtius, F.; and Krüger, K.H.: Zur Klinik, Histologie und Genealogie der Déjerine-Sottasschen Krankheit , Arch Psychiat Nervenkr 186:341-360, 1951.Crossref 37. Austin, J.H.: Observations on the Syndrome of Hypertrophic Neuritis (The Hypertrophic Interstitial Radiculoneuropathies) , Medicine (Balt) 35:187-237 ( (Sept) ) 1956.Crossref 38. Bedford, P.D., and James, F.E.: A Family With the Progressive Hypertrophic Polyneuritis of Dejerine and Sottas , J Neurol Neurosurg Psychiat 19:46-51 ( (Feb) ) 1956.Crossref 39. Isaacs, H.: Familial Chronic Hypertrophic Polyneuropathy With Paralysis of the Extremities in Cold Weather , S Afr Med J 34:758-761 ( (Sept 3) ) 1960. 40. Webster, H.deF., et al: The Role of Schwann Cells in the Formation of "Onion Bulbs" Found in Chronic Neuropathies , J Neuropath Exp Neurol 26:276-299 ( (April) ) 1967.Crossref 41. Lambert, E.H.: Clinical Examinations in Neurology , Philadelphia: W. B. Saunders Company, 1956, pp 287-317. 42. 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Journal

Archives of NeurologyAmerican Medical Association

Published: Jun 1, 1968

References