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HEPATOLENTICULAR DEGENERATION: Analysis of Dyskinetic Phenomena; Relation of Degree of Hepatic Damage to Course of the Disease; Nervous Disorders in Ordinary Disease of the Liver

HEPATOLENTICULAR DEGENERATION: Analysis of Dyskinetic Phenomena; Relation of Degree of Hepatic... Abstract THE CLINICAL diagnosis of hepatolenticular degeneration is made on the basis of the evaluation of the motor phenomena, the evidence of hepatic dysfunction and the presence of the ring of a pigment at the margin of the cornea. The classic descriptions of Wilson,1 Westphal2 and Strümpell3 and the numerous subsequent reports make it possible to delimit this disease from other pathologic entities in the majority of cases. However, a detailed analysis of the dyskinetic symptoms is still wanting and the importance of liver function tests for diagnostic consideration is still under discussion. The specificity of the clinical picture, as well as that of the pathologic changes in the brain stem, needs to be stressed again in the light of recent observations on nervous disorders associated with various hepatic diseases in man and in animals. A comparison of these conditions with hepatolenticular degeneration can be established only after References 1. Wilson, S. A. K.: Progressive Lenticular Degeneration: A Familial Nervous Disease Associated with Cirrhosis of the Liver , Brain 34:295, 1912.Crossref 2. Westphal, C.: Ueber eine dem Bilde der cerebrospinalen Degeneration ähnliche Erkrankung des centralen Nervensystems ohne anatomischen Befund , Arch. f. Psychiat. 14:87, 1883.Crossref 3. Strümpell, A.: (a) Ueber die Westphalsche Pseudosclerose und über diffuse Hirnsclerose, insbes. bei Kindern , Deutsche Ztschr. f. Nervenh. 12:115, 1898Crossref 4. Ein weiterer Beitrag zur Kenntnis der sogen. Pseudosclerose , Strümpell Deutsche Ztschr. f. Nervenh. 14:348, 1899.Crossref 5. Herz, E.: Die amyostatischen Unruheerscheinungen: Klinisch-kinematographische Analyse ihrer Kennzeichen und Begleiterscheinungen , Leipzig, Johannes Ambrosius Barth, 1931. 6. Holmes, G.: On Certain Tremors in Organic Cerebral Lesions , Brain 27:327, 1904.Crossref 7. Holmes, G.: The Cerebellum of Man , Brain 112:1, 1939.Crossref 8. Wilson, S. A. K.: Neurology , Baltimore, William Wood & Company, 1940, vol. 2. 9. Denny-Brown, D.: Diseases of the Basal Ganglia and Subthalamic Nuclei , New York, Oxford University Press, 1946. 10. Homburger, F., and Kozol, H. L.: Hepatolenticular Degeneration , J. A. M. A. 130:6 ( (Jan. 5) ) 1946.Crossref 11. Josephy, H.: Degeneratio hepato-lenticularis , in Bumke, O., and Foerster, O.: Handbuch der Neurologie , Berlin, Julius Springer, 1936, vol. 16. 12. Hunt, J. R.: The Strio-Cerebellar Tremor: A Study of the Nature and Localization of the Combined Form of Organic Tremor , Arch. Neurol. & Psychiat. 8:664 ( (Dec.) ) 1922. 13. Rotter, R.: Beitrag zur Histopathologie und Pathogenese der Wilson-Pseudosclerose Gruppe , Ztschr. f. d. ges. Neurol. & Psychiat. 111:159, 1927. 14. Spielmeyer, W.: Die histopathologische Zusammengehörigkeit der Wilsonsche Krankheit und Pseudosclerose , Ztschr. f. d. ges. Neurol. & Psychiat. 57:312, 1920. 15. von Lehoczky, T.: Zur Anatomie und Klinik der Wilson-Pseudosclerose Gruppe , Arch. f. Psychiat. 95:481, 1931. 16. Miskolczy, D.: Wilsonsche Krankheit und Kleinhirn , Arch. f. Psychiat. 97:27, 1932. 17. Stadtler, H.: Histopathologische Untersuchungen zur Frage der Beziehung zwischen Leber- und Gehirnveränderungen , Ztschr. f. d. ges. Neurol. u. Psychiat. 154:626, 1936 18. Die Erkrankung der Westphal-Wilsonschen Pseudosclerose als Stoffwechselproblem , Deutsche med. Wchnschr. 66:1325, 1940. 19. Waggoner, R. W., and Malamud, N.: Wilson's Disease in Light of Cerebral Changes Following Ordinary Acquired Liver Disorders , J. Nerv. & Ment. Dis. 96:410, 1942. 20. Crandall, L. A., Jr., and Weil, A.: Pathology of Central Nervous System in Diseases of the Liver , Arch. Neurol. & Psychiat. 29:1066 ( (May) ) 1933. 21. Woods, A. H., and Pendleton, L.: Fourteen Simultaneous Cases of an Acute Degenerative Striatal Disease , Arch. Neurol. & Psychiat. 13:549 ( (May) ) 1925. 22. Alexander, L.: The Fundamental Types of Histopathologic Changes Encountered in Cases of Athetosis and Paralysis Agitans , A. Research Nerv. & Ment. Dis. Proc. (1940) 21:334, 1942. 23. Baker, A. B.: The Central Nervous System in Hepatic Disease , J. Neuropath. & Exper. Neurol. 8:283, 1949. 24. Adams, R. D., and Foley, J. M.: The Neurological Changes in the More Common Types of Severe Liver Disease , Tr. Am. Neurol. A. 74:217, 1949. 25. Newman, J. L.: Infective Hepatitis: History of Outbreak in Lavant Valley , Brit. M. J. 1:61, 1942.Crossref 26. Cameron, J. D. S.: Infective Hepatitis , Quart. J. Med. 12:139, 1943. 27. Lescher, F. G.: Nervous Complications of Infective Hepatitis , Brit. M. J. 1:554, 1944.Crossref 28. Byrne, E. A. J., and Taylor, G. F.: An Outbreak of Jaundice with Signs in Nervous System , Brit. M. J. 1:477, 1945.Crossref 29. Weinstein, L., and Davison, W. T.: Neurologic Manifestations in Preicteric Phase of Infectious Hepatitis , Am. Practitioner 1:191, 1946. 30. Stokes, J. F.; Owen, J. R., and Holmes, E. G.: Neurological Complications of Infectious Hepatitis , Brit. M. J. 2:642, 1945.Crossref 31. Zimmerman, H. M., and Yannet, H.: Kernicterus: Jaundice of the Nuclear Masses of the Brain , Am. J. Dis. Child. 45:740 ( (April) ) 1933Crossref 32. Cerebral Sequelae of Icterus Gravis Neonatorum and Their Relation to Kernicterus , Zimmerman Am. J. Dis. Child. 49:418 ( (Feb.) ) 1935. 33. Leonard, M. F.: Hemolytic Disease of the Newborn , J. Pediat. 27:249, 1945.Crossref 34. Docter, J. M.: Kernicterus: Neurological Sequelae of Erythroblastosis Fetalis , J. Pediat. 27:327, 1945.Crossref 35. Stiller, R.: Kernicterus: A Follow-Up Study of 35 Erythroblastotic Infants , Am. J. Dis. Child. 73:651 ( (June) ) 1947.Crossref 36. Lande, L.: Clinical Signs and Development of Survivors of Kernicterus Due to Rh Sensitization , J. Pediat. 32:692, 1948. 37. Hutyra, F., Marek, J., and Manninger, R.: Special Pathology and Therapeutics of the Diseases of Domestic Animals , ed. 4, London, Baillière, Tindall & Cox, 1938, vol. 2, 388-397. 38. Dobberstein, in Joest's Handbuch der speziellen pathologischen Anatomie der Haustiere , Berlin, Richard Schoetz, 1926, vol. 2, p. 594. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology & Psychiatry American Medical Association

HEPATOLENTICULAR DEGENERATION: Analysis of Dyskinetic Phenomena; Relation of Degree of Hepatic Damage to Course of the Disease; Nervous Disorders in Ordinary Disease of the Liver

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Publisher
American Medical Association
Copyright
Copyright © 1950 American Medical Association. All Rights Reserved.
ISSN
0096-6754
DOI
10.1001/archneurpsyc.1950.02310240002001
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Abstract

Abstract THE CLINICAL diagnosis of hepatolenticular degeneration is made on the basis of the evaluation of the motor phenomena, the evidence of hepatic dysfunction and the presence of the ring of a pigment at the margin of the cornea. The classic descriptions of Wilson,1 Westphal2 and Strümpell3 and the numerous subsequent reports make it possible to delimit this disease from other pathologic entities in the majority of cases. However, a detailed analysis of the dyskinetic symptoms is still wanting and the importance of liver function tests for diagnostic consideration is still under discussion. The specificity of the clinical picture, as well as that of the pathologic changes in the brain stem, needs to be stressed again in the light of recent observations on nervous disorders associated with various hepatic diseases in man and in animals. A comparison of these conditions with hepatolenticular degeneration can be established only after References 1. Wilson, S. A. K.: Progressive Lenticular Degeneration: A Familial Nervous Disease Associated with Cirrhosis of the Liver , Brain 34:295, 1912.Crossref 2. Westphal, C.: Ueber eine dem Bilde der cerebrospinalen Degeneration ähnliche Erkrankung des centralen Nervensystems ohne anatomischen Befund , Arch. f. Psychiat. 14:87, 1883.Crossref 3. Strümpell, A.: (a) Ueber die Westphalsche Pseudosclerose und über diffuse Hirnsclerose, insbes. bei Kindern , Deutsche Ztschr. f. Nervenh. 12:115, 1898Crossref 4. Ein weiterer Beitrag zur Kenntnis der sogen. Pseudosclerose , Strümpell Deutsche Ztschr. f. Nervenh. 14:348, 1899.Crossref 5. Herz, E.: Die amyostatischen Unruheerscheinungen: Klinisch-kinematographische Analyse ihrer Kennzeichen und Begleiterscheinungen , Leipzig, Johannes Ambrosius Barth, 1931. 6. Holmes, G.: On Certain Tremors in Organic Cerebral Lesions , Brain 27:327, 1904.Crossref 7. Holmes, G.: The Cerebellum of Man , Brain 112:1, 1939.Crossref 8. Wilson, S. A. K.: Neurology , Baltimore, William Wood & Company, 1940, vol. 2. 9. Denny-Brown, D.: Diseases of the Basal Ganglia and Subthalamic Nuclei , New York, Oxford University Press, 1946. 10. Homburger, F., and Kozol, H. L.: Hepatolenticular Degeneration , J. A. M. A. 130:6 ( (Jan. 5) ) 1946.Crossref 11. Josephy, H.: Degeneratio hepato-lenticularis , in Bumke, O., and Foerster, O.: Handbuch der Neurologie , Berlin, Julius Springer, 1936, vol. 16. 12. Hunt, J. R.: The Strio-Cerebellar Tremor: A Study of the Nature and Localization of the Combined Form of Organic Tremor , Arch. Neurol. & Psychiat. 8:664 ( (Dec.) ) 1922. 13. Rotter, R.: Beitrag zur Histopathologie und Pathogenese der Wilson-Pseudosclerose Gruppe , Ztschr. f. d. ges. Neurol. & Psychiat. 111:159, 1927. 14. Spielmeyer, W.: Die histopathologische Zusammengehörigkeit der Wilsonsche Krankheit und Pseudosclerose , Ztschr. f. d. ges. Neurol. & Psychiat. 57:312, 1920. 15. von Lehoczky, T.: Zur Anatomie und Klinik der Wilson-Pseudosclerose Gruppe , Arch. f. Psychiat. 95:481, 1931. 16. Miskolczy, D.: Wilsonsche Krankheit und Kleinhirn , Arch. f. Psychiat. 97:27, 1932. 17. Stadtler, H.: Histopathologische Untersuchungen zur Frage der Beziehung zwischen Leber- und Gehirnveränderungen , Ztschr. f. d. ges. Neurol. u. Psychiat. 154:626, 1936 18. Die Erkrankung der Westphal-Wilsonschen Pseudosclerose als Stoffwechselproblem , Deutsche med. Wchnschr. 66:1325, 1940. 19. Waggoner, R. W., and Malamud, N.: Wilson's Disease in Light of Cerebral Changes Following Ordinary Acquired Liver Disorders , J. Nerv. & Ment. Dis. 96:410, 1942. 20. Crandall, L. A., Jr., and Weil, A.: Pathology of Central Nervous System in Diseases of the Liver , Arch. Neurol. & Psychiat. 29:1066 ( (May) ) 1933. 21. Woods, A. H., and Pendleton, L.: Fourteen Simultaneous Cases of an Acute Degenerative Striatal Disease , Arch. Neurol. & Psychiat. 13:549 ( (May) ) 1925. 22. Alexander, L.: The Fundamental Types of Histopathologic Changes Encountered in Cases of Athetosis and Paralysis Agitans , A. Research Nerv. & Ment. Dis. Proc. (1940) 21:334, 1942. 23. Baker, A. B.: The Central Nervous System in Hepatic Disease , J. Neuropath. & Exper. Neurol. 8:283, 1949. 24. Adams, R. D., and Foley, J. M.: The Neurological Changes in the More Common Types of Severe Liver Disease , Tr. Am. Neurol. A. 74:217, 1949. 25. Newman, J. L.: Infective Hepatitis: History of Outbreak in Lavant Valley , Brit. M. J. 1:61, 1942.Crossref 26. Cameron, J. D. S.: Infective Hepatitis , Quart. J. Med. 12:139, 1943. 27. Lescher, F. G.: Nervous Complications of Infective Hepatitis , Brit. M. J. 1:554, 1944.Crossref 28. Byrne, E. A. J., and Taylor, G. F.: An Outbreak of Jaundice with Signs in Nervous System , Brit. M. J. 1:477, 1945.Crossref 29. Weinstein, L., and Davison, W. T.: Neurologic Manifestations in Preicteric Phase of Infectious Hepatitis , Am. Practitioner 1:191, 1946. 30. Stokes, J. F.; Owen, J. R., and Holmes, E. G.: Neurological Complications of Infectious Hepatitis , Brit. M. J. 2:642, 1945.Crossref 31. Zimmerman, H. M., and Yannet, H.: Kernicterus: Jaundice of the Nuclear Masses of the Brain , Am. J. Dis. Child. 45:740 ( (April) ) 1933Crossref 32. Cerebral Sequelae of Icterus Gravis Neonatorum and Their Relation to Kernicterus , Zimmerman Am. J. Dis. Child. 49:418 ( (Feb.) ) 1935. 33. Leonard, M. F.: Hemolytic Disease of the Newborn , J. Pediat. 27:249, 1945.Crossref 34. Docter, J. M.: Kernicterus: Neurological Sequelae of Erythroblastosis Fetalis , J. Pediat. 27:327, 1945.Crossref 35. Stiller, R.: Kernicterus: A Follow-Up Study of 35 Erythroblastotic Infants , Am. J. Dis. Child. 73:651 ( (June) ) 1947.Crossref 36. Lande, L.: Clinical Signs and Development of Survivors of Kernicterus Due to Rh Sensitization , J. Pediat. 32:692, 1948. 37. Hutyra, F., Marek, J., and Manninger, R.: Special Pathology and Therapeutics of the Diseases of Domestic Animals , ed. 4, London, Baillière, Tindall & Cox, 1938, vol. 2, 388-397. 38. Dobberstein, in Joest's Handbuch der speziellen pathologischen Anatomie der Haustiere , Berlin, Richard Schoetz, 1926, vol. 2, p. 594.

Journal

Archives of Neurology & PsychiatryAmerican Medical Association

Published: Jun 1, 1950

References