SciELO - Scientific Electronic Library Online

 
vol.54 issue4Dementia in Parkinson's disease: a critical review of literatureDeath concepts and brain death diagnostic criteria author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand

Journal

Article

Indicators

Related links

Share


Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.54 no.4 São Paulo Dec. 1996

http://dx.doi.org/10.1590/S0004-282X1996000400024 

Considerações a respeito da doença de Parkinson de início precoce revisão crítica da literatura

 

A comprehensive critical review on early onset Parkinson's disease

 

 

Luiz Augusto Franco de Andrade

Professor Adjunto e Livre-Docente de Neurologia, Chefe da Disciplina de Neurologia da Escola Paulista de Medicina (Universidade Federal de São Paulo)

 

 


RESUMO

Desde sua descrição, em 1817, a doença de Parkinson sempre foi entendida como enfermidade que afeta predominantemente indivíduos acima dos 55 anos. Entretanto, desde o final do século passado descreveram-se casos com início em idades precoces, abaixo dos 40 anos. Inúmeras séries de pacientes têm sido apresentadas, com as mais numerosas contando em torno de 60 casos. Tem havido muita controvérsia a respeito de alguns aspectos. Um deles diz respeito à própria denominação, sendo chamado por uns como parkinsonismo de início precoce, parkinsonismo jovem por outros e, ainda, doença de Parkinson de início precoce por outros tantos. Isto denota outra controvérsia sobre a verdadeira situação nosológica: trata-se da mesma doença de Parkinson, com as inclusões dos corpos de Lewy, que ocorre nos indivíduos mais idosos ou, diferentemente, trata-se de outras entidades, com anatomopatologia diversa? Esta a razão principal dos inúmeros estudos clínicos realizados nos pacientes deste grupo de início precoce, pois o número de estudos anatomopatológicos em tais pacientes é muito reduzido. Há evidências de que a maioria dos pacientes seja de doença de Parkinson clássica com início em idades mais jovens, apesar de uma série de descrições patológicas diferentes do habitual para esta enfermidade. Há maior encontro de casos familiares entre os pacientes jovens. Uma das características clínicas mais marcantes é o precoce e invariável aparecimento de complicações da levodopaterapia, como discinesias e flutuações. Por outro lado, parece haver uma evolução clínica mais lenta e favorável. São apresentadas, revistas e comentadas grande parte das séries que apareceram na literatura.

Palavras-chave: doença de Parkinson, início precoce, parkinsonismo precoce, juvenil.


ABSTRACT

Since its original description Parkinson's disease has been considered as a clinical condition which affects older people. Nonetheless, since late in the last century, cases starting in very young age have been described. A great controversy has arisen concerning the real pathology in these cases and, consequently, how should they be named. Early or young onset parkinsonism, early or young onset Parkinson's disease, juvenile parkinsonism, all these terms have been used indistinguishable. There have been few pathological descriptions in early onset parkinsonism. Some show striking differences from the cases of older patients but others are very similar to what has been considered classical Parkison's disease. Younger starting age usually corresponds to greater possibility of other family members being affected. Dyskinesias and fluctuations due to chronic levodopa treatment are an early and almost invariable complication in the course of young patients. Comments on several aspects based on an extensive literature review are presented.

Key words:Parkinson's disease, young onset, juvenile, early onset parkinsonism.


 

 

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

 

 

REFERÊNCIAS

1. Andrade LAF. The role of the environmental factors in the genesis of Parkinson's disease. In Caraceni T, Nappi G, (eds) Focus on Parkinson's disease Milano: Masson, 1991:67-78.         [ Links ]

2. Andrade LAF. Contribuição ao estudo da doença de Parkinson de início precoce: análise clínica de 58 pacientes. Tese de Livre-Docência. Escola Paulista de Medicina (Universidade Federal de São Paulo).São Paulo, 1994.         [ Links ]

3. Araújo AQC, Miranda SBM. Doença de Segawa: distonia progressiva sensível à L-Dopa: relato de um caso. Arq Neuropsiquiatr 1993;51:532-536.         [ Links ]

4. Askenazy JJM, Mendelson L, Keren O, Braun Z. Juvenile Parkinson's disease and its response to L-dopa therapy. J Neural Transm 1990;2:23-30.         [ Links ]

5. Ballard PA, Tetrud JW, Langston JW. Permanent human parkinsonism due to 1 -methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP):seven cases. Neurology 1985;35:949-56.         [ Links ]

6. Barbeau A, Cloutier RT, Plasse L, Paris S. Environmental and genetic factors in the etiology of Parkinson's disease. In Yahr MD, Bergmann KJ (eds) Adv Neurol.Vol. 45. New York: Raven Press 1986;299-306.         [ Links ]

7. Barbeau A, Pourcher E. New data on the genetics of Parkinson's disease. Can J Neurol Sei 1982;9:53-66.         [ Links ]

8. Barbeau A, Roy M. Genetic susceptibility, environmental factors and Parkinson's disease. Proceedings of the VIII International Symposium on Parkinson's Disease, New York: June, 9-12,1985:13.         [ Links ]

9. Barbeau A, Roy M, Bernier G, Campanella G, Paris S. Ecogenetics of Parkinson's disease: prevalence and environmental aspects in rural areas. Can J Neurol Sei 1987;14:36-41.         [ Links ]

10. Barbeau A, Roy M, Paris S, Cloutier T, Plasse L, Poirier J. Ecogenetics of Parkinson's disease: 4-hydroxylation of debrisoquine. Lancet 1985;2:1213-6.         [ Links ]

11. Bury JS. Two cases of paralysis agitans in the same family in which improvement followed the administration of hyoscine. Lancet 1902; 1:1097.         [ Links ]

12. Butterfield PG, Valanis BG, Spencer PS, Lindeman CA, Nutt JG. Environmental antecedents of young-onset Parkinson's disease. Neurology 1993;43:1150-1158.         [ Links ]

13. Carlier G, Dubru JM. Familial juvenile parkinsonism. Acta Paediat Belg 1979;32:123-127.         [ Links ]

14. Clough CG, Mendoza M, Yahr MD. A case of sporadic juvenile Parkinson's disease. Arch Neurol, 1981; 38:730-731.         [ Links ]

15. Davison, D. Pallido-pyramidal disease. J Neuropath Exp Neurol, 1947;13:50-59.         [ Links ]

16. Dwork AI, Balmaceda C, Fazzini EA, Mac Collin M, Côté LA, Fahn S. Dominantly inherited, early-onset parkinsonism: neuropathology of a new form. Neurology 1993 ;43:69-74.         [ Links ]

17. Ferraz HB, Bertolucci PHF, Pereira JS, Lima JGC, Andrade LAF. Chronic exposure to the fungicide maneb may produce symptoms and signs of CNS manganese intoxication. Neurology 1988;38:550-553.         [ Links ]

18. Ferreiro JL, Pugliese MI, Cari de AE. Parkinsonismo juvenil: a proposito de 18 casos. Medicina (Buenos Aires) 1991;51:204-208.         [ Links ]

19. Furukawa Y, Nishi K, Kondo T, Mizuno Y.Narabayashi H. Juvenile parkinsonism: ventricular CSF biopterin levels and clinical features. J Neurol Sei, 1992;108:207-213.         [ Links ]

20. Gershanik OS. Parkinsonism of early onset. In Jankovic J, Tolosa E (eds). Parkinson's disease and movement disorders. Baltimore: Urban & Schwarzenberg, 1988:191-204.         [ Links ]

21. Gershanik OS. Parkinsonism of early onset. In Jankovic J, Tolosa E (eds) Parkinson's disease and movement disorders. Baltimore: Williams & Wilkins, 1993:235-252.         [ Links ]

22. Gershanik OS, Leist A. Juvenile onset Parkinson's disease. In Yahr MD, Bergmann KJ. Adv Neurol Vol. 45. New York: Raven Press 1986:213-216.         [ Links ]

23. Gershanik OS, Nygaard TG. Parkinson's disease beginning before age 40. In Streifler MB, Korczyn AD, Melamed E, Youdim MBH (eds). Adv Neurol Vol 53 New York: Raven Press, 1990:251-258.         [ Links ]

24. Gibb WRG, Lees AJ. A comparison of clinical and pathological features of young- and old-onset Parkinson's disease. Neurology, 1988;38:1402-1406.         [ Links ]

25. Gibb W, Narabayashi H, Yokochi M, Iizuka R. Additional pathological observations in juvenile onset parkinsonism with dystonia (Abstr.). Neurology 1989;39(Suppl 1.): 139.         [ Links ]

26. Gibb WRG, Narabayashi H, Yokochi M, Iizuka R, Lees AJ. New pathologic observations in juvenile onset parkinsonism with dystonia. Neurology 1991;41:820-822.         [ Links ]

27. Giovannini P, Piccolo S, Genitrini P, Soliveri F, Girotti G, Geminiani G, Scigliano G, Caraceni T. Early-onset Parkinson's disease. Mov Disord 1991;6:36-42.         [ Links ]

28. Golbe L.I. Young-onset Parkinson's disease: a clinical review. Neurology 1991;41:168-173.         [ Links ]

29. Golbe L.I. Risk factors in young-onset Parkinson's disease. Neurology 1993;43:1641-1643.         [ Links ]

30. Hoehn MM. Age distribution of patients with parkinsonism. J Am Geriat Soc 1976;24:79-85.         [ Links ]

31. Hunt J.R. Progressive atrophy of the globus pallidus. Brain 1917;40:58-148.         [ Links ]

32. Jenner P, Marsden CD. MPTP-induced parkinsonism in primates and its use in the assessment of novel strategies for the treatment of Parkinson's disease. In Rose FC (ed). Current problems in neurology (6): Parkinson's disease clinical and experimental advances. London: John Libbey, 1988:149-162.         [ Links ]

33. KollerW, O'Hara R, Weiner W, Lang A, Nutt J, Agid I, Bonnet A.M. Jankovic J. Relationship of aging to Parkinson's disease. In Yahr MD, Bergmann KJ (eds). Adv Neurol, Vol.45.New York: Raven Press, 1986:317-321.         [ Links ]

34. Kostic V, Przedborski S, Flaster MS, Sternic N. Early development of levodopa-induced dyskinesias and response fluctuations in young-onset Parkinson's disease. Neurology 1991 ;41:202-205.         [ Links ]

35. Krabbe KH. Nogle Undersogelser over Paralysis Agitans. Bibliotek for Laeger, 1923; 115:355-363.         [ Links ]

36. Langston J.W. MPTP-induced parkinsonism: how good a model is it? In Fahn S (ed). Recent developments in Parkinson's disease. New York: Raven Press, 1986:119-126.         [ Links ]

37. Langston JW, Ballard PA. Parkinson's disease in a chemist working with 1 -methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). N Engl J Med 1983:309:310.         [ Links ]

38. Langston JW, Ballard P, Tetrud JW, Irwin I. Chronic parkinsonism in humans due to a product of meperidine analog synthesis. Science 1983;219:979-980.         [ Links ]

39. Langston JW, Langston EB, Irwin I. MPTP-induced parkinsonism in human and non-human primates: clinical and experimental aspects. Acta Neurol Scand 1984;70:49-54.         [ Links ]

40. Lima AB, Levy A, Castro Caldas A, Neves G, Lopes G, Sampaio C. Parkinson's disease before age 30. In Narabayashi H, Nagatsu T, Yanagizawa N, Mizuno Y. Adv Neurol, Vol 60. New York: Raven Press, 1993:553-557.         [ Links ]

41. Lima B, Neves G, Nora M. Juvenile parkinsonism: clinical and metabolic characteristics. Proceedings of the VIII International Symposium on Parkinson's Disease, New York: June 9-12,1985 P6.         [ Links ]

42. Lima B, Neves G, Nora M. Juvenile parkinsonism: clinical and metabolic characteristics. J Neurol Neurosurg Psychiatry 1987;50:345-348.         [ Links ]

43. Martin WE, Resch JA, Baker AB. Juvenile parkinsonism. Arch Neurol 1971 ;25:494-500.         [ Links ]

44. Mayer JM, Mikol J, Haguenau M, Dellanave J, Pépin B. Familial juvenile parkinsonism with multiple systems degenerations: a clinicopathological study. J Neurol Sci 1986;72:91-101.         [ Links ]

45. Mjones H. Paralysis agitans: a clinical and genetic study. Acta Psychiatr Neurol 1949;54(Suppl): 1 -195.         [ Links ]

46. Nygaard TG, Marsden CD, Duvoisin, RC. Dopa-responsive dystonia. In Fahn S, Marsden CD, Calne DB (eds) Adv Neurol, Vol 50. New York: Raven Press, 1988:377-384.         [ Links ]

47. Ota Y, Miyoshi S, Ueda O, Mukai T, Maeda A. Familial paralysis agitans juvenilis: clinical, anatomical and genetic study. Folia Psychiat Neurol Japónica 1958; 12:112-121.         [ Links ]

48. Parkinson J. An essay on the shaking palsy. Sherwood, Neely & Jones, 1817. Ed. fac-simile in Med Class 1938;3:964-997.         [ Links ]

49. Quinn N, Critchley P, Marsden CD. Young onset Parkinson's disease. Mov Disord, 1987;2:73-91.         [ Links ]

50. Rajput AH, Stern W, Christ A, Laverty W. Etiology of Parkinson's disease: environmental factor(s). Neurology 1984;34:(Suppl)207.         [ Links ]

51. Rajput AH, Uitti RJ, Stern W, Laverty W Early onset Parkinson's disease and childhood environment. In Yahr MD, Bergmann KJ. Adv Neurol, Vol 45. New York: Raven Press, 1986:295-297.         [ Links ]

52. Rajput AH, Uitti RJ, Stern W, Laverty W, O'Donell K, Yuen WK, Dua A. Geography, drinking water chemistry, pesticides and herbicides and the etiology of Parkinson's disease. Can J Neurol Sci 1987;14:414-418.         [ Links ]

53. Sachdev KK, Singh N, Krishnamoorty MS. Juvenile parkinsonism treated with levodopa. Arch Neurol, 1977;34:244-245.         [ Links ]

54. Sardinha LA, Braga AA, Quagliato EMAB. Parkinsonismo juvenil familiar: apresentação de 3 casos e revisão da literatura. Arq Neuropsiquiatrl 988; 46(Supl):223.         [ Links ]

55. Scaff M, Barbosa ER, Assis JL, Canelas HM. Parkinsonismo juvenil: considerações a respeito de 10 casos. Arq Neuropsiquiatr, 1980;38:385- 390.         [ Links ]

56. Scott RM, Brody JA. Benign early onset Parkinson's disease: a syndrome distinct from classic postencephalitic parkinsonism. Neurology 1971;21:366-368.         [ Links ]

57. Segawa M, Hosaka A, Miyagawa F, Nomura Y, Imai H. Hereditary progressive dystonia with marked diurnal fluctuation. In Eldridge R, Fahn S.Adv.Neurol, Vol.14. New York: Raven Press, 1976:215-233.         [ Links ]

58. Segawa M, Nomura Y, Kase M. Hereditary progressive dystonia with marked diurnal fluctuation: clinico-pathophysiological identification in reference to juvenile Parkinson's disease. Proceedings of the VIII International Symposium on Parkinson's Disease, New York: Raven Press 1985:61.         [ Links ]

59. Stern M, Dulaney E, Gruber SB, Golbe L, Bergen M, Hurtig H, Gollomp S, Stolley P. The epidemiology of Parkinson's disease: a case-control study of young-onset and old-onset patients. Arch Neurol 1991 ;48:903-907.         [ Links ]

60. Tanner CM, Chen B, Wang WZ, Peng ML, Liu ZL, Liang XL, Kao LC, Gilley DW. Schoenberg, B.S. Environmental factors in the etiology of Parkinson's disease. Can J Neurol Sci 1987;14:419-423.         [ Links ]

61. Tanner CM, Grabler P, Goetz CG. Occupation and the risk of Parkinson's disease: a case-control study in young-onset patients. Neurology 1990; 40 (Suppl 1):422.         [ Links ]

62. Tanner CM, Langston JW. Do environmental toxins cause Parkinson's disease? A critical review. Neurology 1990; 40 (Suppl 3): 17-30.         [ Links ]

63. Teravainen H, Forgath L, Hietanen M, Schulzer M, Schoenberg B, Calne D.B. The age on onset of Parkinson's disease: etiological implications. Can J Neurol Sci, 1986;13:317-319.         [ Links ]

64. Van Bogaert L. Contribution clinique et anatomique a 1'étude de la paralysie agitante juvenile primitive. Rev Neurol (Paris) 1930;11:315-326.         [ Links ]

65. Willige H. Über Paralysis Agitans in Jugendlichem Alter. Z Gesamte Neurol Psychiatr 1911 ;4:520-587.         [ Links ]

66. Yamamura Y, Sobue I, Ando K, Dda M, Yanagi T, Kono C. Paralysis agitans of early onset with marked fluctuation of symptoms. Neurology 1973;23:239-244.         [ Links ]

67. Yokochi M. Juvenile Parkinson's disease: 1. Clinical aspects. Adv Neurol Sci (Tokyo) 1979;23:1048-1059.         [ Links ]

68. Yokochi M. Juvenile Parkinson's disease: Pharmacokinetic study. Adv Neurol Sci (Tokyo) 1979;23:1060-1073.         [ Links ]

69. Yokochi M, Narabayashi H. Clinical characteristics of juvenile Parkinsonism. In Rose FCR, Capildeo R. Research progress in Parkinson's disease. Tunbridge Wells, Kent, R. Pitman Medical, 1981:35-39.         [ Links ]

70. Yokochi M, Narabayashi H, Iizuka R, Nagatsu T. Juvenile parkinsonism some clinical, pharmacological, and neuropathological aspects. In Hassler RG, Christ JF. Adv Neurol, Vol 40. New York: Raven Press, 1984;407-413.         [ Links ]

71. Yoshimura N, Hayashi S, Fukushima Y. Diffuse Mallory bodies in the liver, diffuse Lewy bodies in the brain and diffuse fat replacement (lipomatous pseudohypertrophy) of the pancreas in a patient with juvenile Parkinson's disease. Acta Pathol Japonica 1992;42:826-831.         [ Links ]

 

 

Aceite: 6-agosto-1996.

 

 

Dr. Luiz Augusto Franco de Andrade - Rua Borges Lagoa 1231 conjunto 44 - 04038-033 São Paulo SP - Brasil.

Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License