Importance of juvenile parkinsonism in elucidating the pathogenesis of Parkinson’s disease

  • M. Yokochi
Conference paper
Part of the Key Topics in Brain Research book series (KEYTOPICS)


In recent studies of Parkinson’s disease, the importance of studying juvenile parkinsonism has been emphasized. The reason for this are as follows.
  1. (1)

    Juvenile parkinsonism provides a crucial clinical model as a prototype of dopamine deficiency syndrome caused by nigro-striatal dysfunction. It might eliminate the factors related to aging from clinical and pathobiochemical findings.

  2. (2)

    The case group of juvenile parkinsonism is situated between parkinson’s disease with onset after middle age and dopa-responsive dystonia with onset in childhood. Without study of juvenile parkinsonism, further development of nosological discussion of the dopamine deficiency syndrome will not be possible.

  3. (3)

    Interestingly, some cases have recently shown different pathological pictures in the substantia nigra from the traditional parkinsonian pathology of Lewy body pathology in autopsies. This evidence suggests that in juvenile parkinsonism, there is an underlying mechanism in the pathological process toward the lesion in the substantia nigra.


From these significant aspects, tracing of juvenile parkinsonism will contribute to finding the core of the pathophysiology and to determining the real pathogenesis of the dopamine deficiency syndrome.

In this paper, the clinical characteristics of epidemiology, symptomatology and prognosis, and pathological findings up to now, are presented. In addition, nosological discussion is developed on the basis of recent publications on juvenile parkinsonism.


Substantia Nigra Levodopa Treatment Juvenile Onset Lewy Body Pathology Familial Incidence 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Barbeau A, Pourcher E (1981) Studies on the etiology of Parkinson’s disease. In: Abstracts, 12th World Congress of Neurology. International Congress Series: p 548. Excerpta Medica, p 132Google Scholar
  2. Barbeau A, Pourcher E (1982) New data on genetics of Parkinson’s disease. Can J Neurol Sci 9: 53–60PubMedGoogle Scholar
  3. Barbeau A (1986) Parkinson’s disease: clinical features and etiopathology. In: Vinken PJ, Bruyn GW, Klawans HL (eds) Handbook of clinical neurology, vol 5(49). Elsevier Science Publisher, pp 87–152Google Scholar
  4. Calne S, Schoenberg B, Martin W, Uitti RJ, Spencer P, Calne DB (1987) Familial Parkinson’s disease: possible role of environmental factors. Can J Neurol Sci 14: 303–305PubMedGoogle Scholar
  5. Diamond SG, Markham Ch H (1976) Present mortality in Parkinson’s disease: the ratio of observed to expected deaths with a method to calculate expected deaths. J Neural Transm 38: 259–269PubMedCrossRefGoogle Scholar
  6. Gershanik OS, Leist A (1986) Juvenile onset Parkinson’s disease. Adv Neurol 45:213 –216Google Scholar
  7. Gibb WR, Lees AJ (1987) The progress of idiopathic Parkinson’s disease is not explained by age-related changes. Clinical and pathological comparison with post-encephalitic parkinsonian syndrome. Acta Neuropathol (Berl) 73:195 –201Google Scholar
  8. Gibb WRG, Lees AJ (1988) A comparison of clinical and pathological features of young-and old-onset Parkinson’s disease. Neurology 38: 1402–1406PubMedGoogle Scholar
  9. Harada H, Nishikawa S, Takahashi K (1983) Epidemiology of Parkinson’s disease in a Japanese City. Arch Neurol 40: 151–154PubMedCrossRefGoogle Scholar
  10. Hoehn MM, Yahr MD (1967) Parkinsonism: onset, progression, and mortality. Neurology 17: 427–442PubMedGoogle Scholar
  11. Hunt JR (1917) Progressive atrophy of the globus pallidus. Brain 40: 58–148CrossRefGoogle Scholar
  12. Ishikawa A, Miyatake T (1985) Pathophysiology of juvenile parkinsonism-regarding juvenile parkinsonism with autosomal recessive trait. Jpn J Neuropsychopharmacol (Jpn) 7: 809–815Google Scholar
  13. Ishikawa A, Miyatake T (1988) Juvenile parkinsonism. In: Miyatake T (ed) Niigata Symposium Series on Neurology, No5. Parkinsonism (Jpn). Kagaku Hyoronsha, Tokyo, pp 22–49Google Scholar
  14. Javoy-Agid F, Ruberg M, Taquet H, Bokobza B, Agid Y, Gaspar P, Berjer P, N’Guyen-Legros J, Albarez C, Gray F, Esconelle R, Scatton B, Roquier L (1984) Biochemical neuropathology of Parkinson’s disease. Adv Neurol 40: 189–198PubMedGoogle Scholar
  15. Kondo T, Narabayashi H, Nagatsu T, Yamaguchi T, Sawada M (1986) Effects of tyrosine administration on plasma biopterin in patients with juvenile parkinsonism and their relatives. Adv Neurol 45: 217–222Google Scholar
  16. Lieberman A, Dziatolowski M, Kupersmith M, Serby M, Goodgold A, Korein J,Goldstein M (1979) Dementia in Parkinson’s disease. Ann Neurol 6: 355–359Google Scholar
  17. Lima B, Neves G, Nora M (1987) Juvenile parkinsonism: clinical and metabolic characteristics. J Neurol Neurosurg Psychiatry 50: 345–348PubMedCrossRefGoogle Scholar
  18. Ludin SM, Ludin HP (1989) Is Parkinson’s disease of early onset a separate disease entity? J Neurol 236: 203–207PubMedCrossRefGoogle Scholar
  19. Miyazawa Y, Abe N, Ohtoh T (1987) An autopsy case of juvenile parkinsonism. Neurol Med (Jpn) 26: 578–583Google Scholar
  20. Mizutani Y, Sugita Y, Kosaka K, Yokochi M, Satoh T, Narabayashi H (1988) Juvenile parkinsonism: neuropathological findings in 7 cases. In: Abstract for 29th General Meeting of Societa Neurologica Japonica (Jpn), p 271Google Scholar
  21. Mizutani Y, Yokochi M, Oyanagi S (1991) Juvenile parkinsonism: a case with first clinical manifestation at the age of six years and with neuropathological findings suggesting a new pathogenesis. Clin Neuropathol (Berl) (in press)Google Scholar
  22. Narabayashi H, Yokochi M, Iizuka R, Nagatsu T (1986) Juvenile parkinsonism. In: Vinken PJ, Bruyn GW, Klawans HL (eds) Handbook of clinical neurology 5 (49). Elsevier Science Publishers, pp 153–165Google Scholar
  23. Nygaard TG, Duvoisin RC (1986) Hereditary dystonia-parkinsonism syndrome of juvenile onset. Neurology 36: 1424–1428PubMedGoogle Scholar
  24. Nygaard TG, Marsden CD, Duvoisin RC (1988) Dopa-responsive dystonia. Adv Neurol 50: 377–384PubMedGoogle Scholar
  25. Ota U, Miyoshi S (1958) Familial paralysis agitans juveniles: a clinical, anatomical and genetic study. Folia Psychiat Neurol Jpn 12: 112–121PubMedGoogle Scholar
  26. Ouvrier RA (1978) Progressive dystonia with marked diurnal fluctuation. Ann Neurol 4: 412–417PubMedCrossRefGoogle Scholar
  27. Quinn N, Critchley P, Marsden CD (1987) Young onset Parkinson’s disease. Movement Disorders 2: 73–91PubMedCrossRefGoogle Scholar
  28. Rajput AH, Uitti RJ, Stern W, Laverty W (1986) Early onset Parkinson’s disease and childhood environment. Adv Neurol 45: 295–297Google Scholar
  29. Rogers D, Lees AJ, Smith E, Trimble M, Stern CM (1987) Bradyphrenia in Parkinson’s disease and psychomotor retardation in depressive illness. An experimental study. Brain 110: 761–776PubMedCrossRefGoogle Scholar
  30. Scott RM, Brody JA (1971) Benign early onset Parkinson’s disease: a syndrome distinct from classic postencephalitic parkinsonism. Neurology 21: 366–368PubMedGoogle Scholar
  31. Segawa M, Hosaka A, Miyagawa F, Nomura Y, Imai H (1976) Hereditary progressive dystonia with marked diurnal fluctuation. Adv Neurol 14:215 –223Google Scholar
  32. Segawa M, Nomura Y, Tanaka S, Hakamada S, Nagata E, Soda M, Kase M (1988) Hereditary progressive dystonia with marked diurnal fluctuation — consideration on its pathophysiology based on the characteristics of clinical and polysomnographical findings. Adv Neurol 50: 367–367PubMedGoogle Scholar
  33. Sunohara N, Mano Y, Toyoshima E, Ando K, Satoyoshi E (1982) Juvenile parkinsonism with marked diurnal fluctuation-about new findings. Clin Neurol (Jpn) 22:101 –105Google Scholar
  34. Sunohara N, Mano Y, Ando K, Satoyoshi E (1985) Idiopathic dystonia — parkinsonism with marked diurnal fluctuation of symptoms. Ann Neurol 17: 39–45PubMedCrossRefGoogle Scholar
  35. Wakabayashi K, Takahashi H, Takeda S, Ohama E, Ikuta F (1988) Parkinson’s disease: the presence of Lewy bodies in Auerbach’s and Meissner’s plexuses. Acta Neuropathol 76: 217–221PubMedCrossRefGoogle Scholar
  36. Yamamura Y, Iida M, Ando K, Sobue I (1968) A juvenile familial disorder with rigido spasticity, bradykinesia and minor dystonia alleviated after sleep. Clin Neurol (Jpn) 8: 233–243Google Scholar
  37. Yamamura U, Sobue I, Ando K, Iida M, Yanagi T, Kono C (1973) Paralysis agitans of early onset with marked diurnal fluctuation of symptoms. Neurology 23: 239–244PubMedGoogle Scholar
  38. Yokochi M (1979a) Juvenile Parkinson’s disease, part I. Clinical aspects. Adv Neurol Sci (Jpn) 23: 1048–1059Google Scholar
  39. Yokochi M (1979b) Juvenile Parkinson’s disease, part II. Pharmacokinetic study. Adv Neurol Sci (Jpn) 23: 1060–1073Google Scholar
  40. Yokochi M, Narabayashi H, Iizuka R, Nagatsu T (1984) Juvenile parkinsonism-some clinical, pharmacological and neuropathological aspects. Adv Neurol 40: 407–413PubMedGoogle Scholar
  41. Yokochi M, Nishimiya J (1985) DOPA induced dyskinesia. Adv Neurol Sci (Jpn) 29: 276–286Google Scholar
  42. Yokochi M (1988a) Long-term follow-up study in patients with juvenile parkinsonism. In: Nakanishi T (ed) Annual report of the Research Committee of CNS degenerative disease. The Ministry of Health and Welfare of Japan, pp 268–272Google Scholar
  43. Yokochi M, Mizutani Y, Narabayashi H, Tsuboi H (1988b) Long-term follow-up study in patient with juvenile parkinsonism. In: Book of Abstracts for 9th International Symposium on Parkinson’s disease (Jerusalem), p 70Google Scholar

Copyright information

© Springer-Verlag/Wien 1991

Authors and Affiliations

  • M. Yokochi
    • 1
  1. 1.Department of NeurologyTokyo Metropolitan Institute for NeurosciencesFuchu-si, Tokyo-to 183Japan

Personalised recommendations