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Dictionnaire des maladies éponymiques et des observations princeps : Lenticulaire (dégénération )
KINNIER WILSON, SA / EDIN / LOND.
Progressive lenticular degeneration : a familial nervous disease assiociated with cirrhosis of the liver
In : Brain, 1912, Vol. 34, part IV pp. 296-509
Exemplaire numérisé : BIU Santé (Paris)
Adresse permanente : https://www.biusante.parisdescartes.fr/histmed/medica/cote?epo1241
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 [sans numérotation]  
 295  [Sommaire]
 296  Introduction
 297  
 298  
 299  
 300  Chapter I.- Historical
 301  
 302  
Image : Fig. 1. S.M (Gowers.) The tremors, spasticity, contractures, &co, are well seen. From one of the original silver prints (date 1886) kindly lent to me by Dr. Thomas Wilson
 303  
Image : Fig. 2-. S.M (Gowers.) Note the apparent indifference of the patient to the fact that his mouth is widely open. From the original in the notes at the National Hospital/ Fig. 3-. S.M (Gowers.) From the original drawing in the notes of the case at the National Hospital
 304  
Image : Fig. 4. S. M (Gowers) From the original drawing in the notes of the case at the National Hospital / Fig. 5. S. M (Gowers) Photograph taken shortly before death, showing spastic smile, ontractures, and profound emaciation. From the original silver print (dated 1886), kindly lent to me by Dr.Thomas Wilson
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Image : Fig. 9. W.S.(Ormerod.) Bilateral softening in the putamen (From St. Bartholomew's Hospital Reports, 1890.)
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Image : Fig. 10. Wilhelm K (Homén.) Note the fixed, smiling expression, open mouth, contractures, and some emanciation ( From Archiv f. Psychiat., 1892.)
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 320  Chapter II.- Clinical cases
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Image : Fig. 11. Handwriting of S.T. when in hospital
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Image : Fig. 12. Photograph of S.T. before the onset of the symptoms of progressive lenticular degeneration
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Image : Fig. 13. Photographs of S. T., taken at Virginia Water. Characteristic appearance of face and upper limbs
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 329  
Image : Fig. 14. Right arm and hand of S.T., showing contractures, and ressemblance of hand to that paralysis agitans
 330  
 331  Résumé of the clinical History of case 1
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Image : Fig. 15. D.P. before the onset of the symptoms of progressive lenticular degeneration
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Image : Fig. 16. D.P.(national hospital, May 1906). Note how patient is leaning over the right side
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 338  Résumé of the clinical History of case 2
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Image : Fig. 17. Normal handwriting of E.P
 342  
Image : Fig. 18. Handwriting of E.P. after the onset of progressive lenticular degeneration
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Image : Fig. 19. E.P (June 4 , 1910). Note mouth, contractures, tremor of the hands
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Image : Fig. 20. E.P in his school days
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Image : Fig. 21. E.P (june 4, 1910). Note vacant expression, open mouth, sialorrhoea, contractures
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Image : Fig. 22 (june 4, 1910). Contracture-attitude of right hand
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 352  Résumé of the clinical History of case 3
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Image : Fig. 23. M. To (National Hospital, October, 1911). Characteristic fixed smile and retracted lips. Note intelligent expression of eyes. The patient is falling stiffly to the right
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Image : Fig. 24. M. To. Note characteristic contacture-attitudes, tremor of right hand (the patient is grasping her nightdress with that hand in the endeavour to keep the limb as steady as possible), open mouth, vacant expression
 360  Résumé of the clinical History of case 4
 361  
 362  
 363  Chapter III.- Pathological finding in the personal cases
 364  
 365  
Image : Fig. 25. Liver (S.T., Case 1), from above/ Fig. 26. Liver (S.T., Case 1). Transverse section
 366  
Image : Fig. 27. Basal surface of brain (S.T., Case 1)
 367  
Image : Fig. 28. Upper surface of brain (S.T., Case 1)
 368  
Image : Fig. 29. Horizontal section through hemispheres (S.T., Case 1)
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Image : Fig. 30.Cervical cord (Case 1). Weigert/ Fig. 31. Dorsal cord. Weigert/ Fig. 32. Lumbar cord (Case 1). Weigert
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Image : Fig. 33. Anterior horn-cells, cervical region (Case 1). Nissl
 373  
Image : Fig. 34. Medulla (Case 1). Weigert-Pal/ Fig. 35 (Case 1). Hypoglossal nucleus, right side (Case 1). Nissl
 374  
Image : Fig. 36. Crus cerebri (Case 1). Weigert-Pal
 375  
Image : Fig. 37. Shows method of subdiving the right hemisphere (case 1) for examination in serial section. Each block is 1cm. Thick. X-y is the line of Marie's coupe d'election
 376  
Image : Fig. 38.Slabs into which thr lrft hemisphere (case 1) was subdivided for the method of serial sections. Each block is 1cm. Thick. X-y as in fig.37
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Image : Fig. 39. Correspond to lin 4-4 of fig. 37. Vertico-transverse section. Cavity in place of lenticular nucleus. Internal capsule intact
 378  
Image : Fig. 40. Correspond to lin 5-5 of fig. 37. Vertico-transverse. Posterior end of lenticular cavity is well shown. Note the normal sized optic thalamus
 379  
Image : Fig. 41. Vertico transverse section n°148 (case 1) Weigert-Pal
 380  
Image : Fig. 42. Vertico transverse section n°184 (case 1) Weigert-Pal. Posterior end of lenticular area
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Image : Fig. 43. Horizontal section n° B31, left hemisphere (case 1) Weigert
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Image : Fig. 44. Horizontal section n° C 39 (case 1) Weigert
 384  
Image : Fig. 45. Photograph of maximum degenerated area (section imperfectly stained) to show situation of figs.46 and 47 (1=47; 2=46)
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Image : Fig. 46. Margin of lenticular cavity (x60) Haematoxylin-van gieson/ Fig. 47. Degenerated fibres in globus pallidus. Weigert-Pal
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Image : Fig. 48. Artery from globus pallidus. Weigert-Pal. Haematoxylin-van gieson. Normal walls (It has plug of bacilli in its lumen.)
 387  
Image : Fig. 49. Degenerated blood-vessel from near margin of lenticular cavity (case 1) / Fig. 50. Oblique section from righte leg centre, paracentral lobule (case 1). Nissl. Normal Betz-cells
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Image : Fig. 51. Liver (case 1). Haematoxylin-van gieson. Irregular size of liver-nodules/ Fig. 52. Liver-cell divinding by an amitotic process.Haematoxylin eosin
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Image : Fig. 53. Clumps of bacilli in cirrhotic liver tissue (case 1). Carbol gentian violet/ Fig. 54. Bacilli in neighbourhood of portal tract (case 1)
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Image : Fig. 55. Liver under surface (D.P.case 2)
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Image : Fig. 56. Liver, transverse section (D.P.case 2). Natural size
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Image : Fig. 57. Medula (case 2). Weigert-pal
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Image : Fig. 58. Vessels from the right putamen (case 2). Haematoxylin-van gieson. Normal walls. No small-cell infiltration/ Fig. 59. Transverse section of lenticulo-striate artery, removed from degenerated area and cut separately (case 2)
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Image : Fig. 60. Liver (E.P., Case 3). Under surface
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Image : Fig. 61. Liver, transverse section (E.P., Case 3)
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Image : Fig. 62. Upper surface of cerebrum (case 3)/ Fig. 63. Under surface of cerebral hemispheres (case 3)
 404  
Image : Fig. 64. Right hemisphere (case 3)
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Image : Fig. 65. Coupe d'éléction of hemispheres (case 3)
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Image : Fig. 66. Coupe d'éléction, left hemispheres (case 3). Atrophic degeneration of lenticular nucleus
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Image : Fig. 67. Coupe d'éléction. Normal brain of a youth who died of cirrhosis of the liver without nervous symptoms
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Image : Fig. 68. Coupe d'éléction, hemispheres (case 3). Soft friable, disintegrated nucleus lenticularis on each side
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Image : Fig. 69. Cevical cord (case 3). Weigert/ Fig. 70. Dorsal cord (case 3). Weigert/ Fig. 71. Lumbard cord (case 3). Weigert
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Image : Fig. 72. Anterior horn cells, lumbar cord (case 3). Nissl / Fig. 73. Anterior horn cells, cervical cord (case 3). Nissl
 414  
Image : Fig. 74. Right hypoglossal nucleus (case 3). Nissl /
 415  
Image : Fig. 75. Crus cerebri (case 3). Weigert. Normal appearance/ Fig. 76. Cells of red nucleus (case 3). Nissl. Numbers of parasite glial cells round many of the nerve-cells
 416  
Image : Fig. 77. Drawings of cut slabs of right basal ganglia (case 3). Natural size
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 418  
Image : Fig. 78. Degenerated area of right putamen (case 3) / Fig. 79. Key to fig. 78
 419  
Image : Fig. 80. Degenerated area, right putamen (case 3). Haematoxylin-eosin. A mass of amorphous neurological overgrowth, breaking down in some places
 420  
Image : Fig. 81. Degenerated area, right putamen (case 3). Nissl.(x 500) Two degenrated nerve-cells, neuroglial cells, "Körnchenzellen", &c. Tissue breaking down
 421  
Image : Fig. 82. A single lenticulo-striate vessel, removed and cut separately (case 3)/ Fig. 83. Macrophages from the degenerating area, left lenticular nucleus (case 3). Marchi. They are crammed with fatty granular débris
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Image : Fig. 84. Horizontal section, right hemisphere (case 3)
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Image : Fig. 85. Horizontal section, right hemisphere (case 3). Weigert. Grossly atrophic putamen and globus pallidus
 424  
Image : Fig. 86. Section of normal brain corresponding to that of fig. 85. Weigert/ Fig. 87. Sunthalamic region, left hemisphere (case 3). Weigert
 425  
Image : Fig. 88. Horizontal section, right crus, somewhat lower than that of fig. 87 (case 3). Weigert
 426  
Image : Fig. 89. Strip from precentral gyrus, left leg centre (case 3). Nissl. Normal betz-cells/ Fig. 90. Strip from precentral gyrus, left arm centre (case 3). Nissl. Normal betz-cells
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 428  
Image : Fig. 91. Liver (case 3). Haematoxylin-eosin. Healthy and degenerated liver-tissue
 429  
Image : Fig. 92. So-called hypertrophying bile ducts in cirrhotic tissue of liver (case 3) / Fig. 93. Regenerating liver-cells (case 3). Division of nuclei
 430  
Image : Fig. 94. Continuity of so-called hypertrophying bilde-ducts xith columns of liver-cells (case 3)
 431  
Image : Fig. 95. Thyroid (case 3)/ Fig. 96. A minute vessel removed from degenerating lenticular area and cut separately (case 3)
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Image : Fig. 97. Naked-eye appearence of liver (case 6, National Hospital, 1869)/ Fig. 98. Transverse section of liver (case 6)
 435  Chapter IV.- Synthetic study of the disease
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 [sans numérotation]  Table summarizing all the known cases of progressive lenticular degeneration
 [page blanche]  
 455  Chapter V.- Nature and pathogenesis of the disease
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 465  Chapter VI.- Pathological physiology of the more important symptoms
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Image : Fig. 99. Diagram to show the relations of the pyramidal and extrapyramidal systems, and the afferent cerebello-cortical system
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 478  Chapter VII.- Syndrome of the corpus striatum
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 486  Chapter VIII.- Clinical conclusions
 487  Pathological conclusions
 488  Physiological conclusions
 489  Appendix
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 504  Bibliography
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 508  Addendum
 509