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Juvenile Parkinson's disease and its response to L-dopa therapy

  • Original Papers
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Journal of Neural Transmission - Parkinson's Disease and Dementia Section

Summary

Four youngsters, between the ages of 13–27 years, presented dystonic foot. Two to three years following the appearance of the dystonic foot, cogwheel rigidity and tremor appeared on the dystonic foot side. Treatment with low doses of levodopa/carbidopa consistently reversed the symptoms.

“On-off” phenomena appeared in the first years of treatment and persisted for the entire period of 5 to 15 years of illness. No parkinsonian signs were present when the dystonic foot appeared. Based on our observations and on a review of the literature, we conclude that responsiveness to low doses of L-dopa is the major maker of juvenile Parkinson's disease.

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References

  • Alajouanine TH, Castaigne P, Held JP, Cambier J, Pierrot-Deseilligny E (1968) Flexion tonique des orteils au cours de la marche chez l'hemiplegique. Essai d'analyse semeiologique: possibilités therapeutiques. Rev Neurol 118: 343–354

    PubMed  Google Scholar 

  • Allen N, Knopp W (1976) Hereditary parkinsonism-dystonia with sustained control by L-dopa and anticholinergic medication. Adv Neurol 14: 201–213

    PubMed  Google Scholar 

  • Askenasy JJM, Mendelson L, Katz A, Costeff H (1975) Basal ganglia disorders with marked circadian fluctuations. Sleep Res 14: 142

    Google Scholar 

  • Askenasy JJM, Streifler M, Felner S (1978) The synaptic significance of metoclopramide induced dyskinetic-dystonic head and neck movements in pregnancy. J Neural Transm 42: 73–84

    Article  PubMed  Google Scholar 

  • Askenasy JJM, Oberman TJ, Herzberg M, Casasso R, Streifler M (1980) Serum dopamine beta hydroxylase activity and metoclopramide provocative test in torsion dystonia. J Neural Transm 47: 69–77

    Article  PubMed  Google Scholar 

  • Barbeau A, Pourcher E (1982) New data on the genetics of Parkinson's disease. Can J Neurol Sci 53–60

  • Dejerine J (1914) Semeiologie des affections du systeme nerveux. Masson, Paris

    Google Scholar 

  • Denny-Brown D (1978) Clinical symptomatology of diseases of basal ganglia. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology, vol 6. Diseases of the basal ganglia. North Holland, Amsterdam, pp 133–172

    Google Scholar 

  • Duvoisin R (1984) Is Parkinson's disease acquired or inherited? Can J Neurol Sci 11: 151–155

    PubMed  Google Scholar 

  • Goldstein K (1938) The tonic foot response to stimulation of the sole: its physiological significance and diagnostic value. Brain 61: 269–283

    Google Scholar 

  • Hall AJ (1923) Encephalitis lethargica (epidemic encephalitis). Lancet i: 731–740

    Google Scholar 

  • Hoehn M, Yahr MD (1967) Parkinsonism: onset, progression and mortality. Neurology 17: 427–442

    PubMed  Google Scholar 

  • Hornykiewicz O (1979) Compensatory biochemical changes at the striatal dopamine synapse in Parkinson's disease—limitations of L-dopa therapy. Adv Neurol 24: 275–28116

    Google Scholar 

  • Hunt JR (1917) Progressive atrophy of the globus pallidus. Primary atrophy of the pallidal system. Brain 40: 138–148

    Google Scholar 

  • Klawans HL (1981) Hemiparkinsonism as a late complication of hemiatrophy: a new syndrome. Neurology 31: 625–628

    PubMed  Google Scholar 

  • Kurland LT (1958) The pathology and treatment of parkinsonism. Fields WS (ed), Spring-field, Ill, p 5

    Google Scholar 

  • Manfredi M, Sacco G, Sideri G (1975) The tonic ambulatory foot response. Brain 98: 167–180

    PubMed  Google Scholar 

  • Marinesco G, Draganesco S, Stoicesco G (1925) Sur une variété speciale de paraplegie spasmodique familiale caráctérisée par des crises paroxystiques d'hypertone, probablement d'origine extra-pyramidal et par des troubles végétatifs. Encéphale 20: 645–654

    Google Scholar 

  • Marsden CD (1976) Dystonia: the spectrum of the disease. In: Yahr MD (ed) The basal ganglia. Raven Press, New York, pp 351–367

    Google Scholar 

  • Marsden CD, Parkes JD (1976) “On-off” effects in patients with Parkinson's disease on chronic levodopa therapy. Lancet i: 292–295

    Article  Google Scholar 

  • Martin WE, Resch JA, Baker AB (1971) Juvenile parkinsonism. Arch Neurol 25: 494–500

    PubMed  Google Scholar 

  • Martin WE, Young WI, Anderson VE (1973) Parkinson's disease. A genetic study. Brain 96: 495–506.76

    PubMed  Google Scholar 

  • Miyoshi K, Matsuoka T, Mizushima S (1969) Familial holotopistic striatal necrosis. Acta Neuropathol (Berlin) 13: 240–249

    Article  Google Scholar 

  • Muenter MD, Sharpless NS, Tyce GM, Darley FL (1977) Patterns of dystonia (IDI and DID) in response to L-dopa therapy for Parkinson's disease. Mayo Clin Proc 2: 1034

    Google Scholar 

  • Naidu S, Wolfson LI, Sharpless NS (1978) Juvenile parkinsonism. A patient with possible primary striatal dysfunction. Ann Neurol 3: 453–455

    Article  PubMed  Google Scholar 

  • Nausidea PA, William JW, Klawans HL (1980) Dystonic foot response of parkinsonism. Arch Neurol 37: 132–136

    PubMed  Google Scholar 

  • Nygaard TG, Duvoisin RC (1986) Hereditary dystonia-parkinsonism syndrome of juvenile onset. Neurology 36: 1424–1428

    PubMed  Google Scholar 

  • Rinne UK, Koskinen V, Lonnberg P (1980) Neurotransmitter receptors in the parkinsonian brain. In: Rinne UK, Klinger M, Stamm G (eds) Parkinson's disease: current progress, problems and management. Elsevier, Amsterdam, North Holland Biomedical Press, pp 93–119

    Google Scholar 

  • Rondot P, Ziegler M (1983) Dystonia: l-dopa responsive or juvenile parkinsonism. J Neural Transm [Suppl 19]: 273–281

    Google Scholar 

  • Schwab RS, England AC (1958) Parkinson's disease. J Chronic Dis 8: 488

    Article  PubMed  Google Scholar 

  • Segawa M, Hosaka A, Miyagawa F, Nomura Y, Imai H (1976) Hereditary progressive dystonia with marked diurnal fluctuations. Adv Neurol 14: 215–223

    PubMed  Google Scholar 

  • Tower SS (1940) Pyramidal lesion in the monkey. Brain 63: 36–90

    Google Scholar 

  • Van Bogaert L (1930) Contribution clinique et anatomique a l'étude de la paralysie agitante juvenile primitive. Rev Neurol 2: 315–326

    Google Scholar 

  • Ward CD, Duvoisin RC, Ince SE, Nutt SD, Eldridge R, Calne DB (1983) Parkinson's disease in 65 pairs of twins and in a set of quadruplets. Neurology 33: 815–824

    PubMed  Google Scholar 

  • Yamamura Y, Sabue I, Ando K (1973) Paralysis agitants of early onset with marked diurnal fluctuations. Neurology 23: 239–244

    PubMed  Google Scholar 

  • Yokochi M, Narabayashi H, Iizuka R, Nagatsu T (1984) Juvenile parkinsonism: some clinical, pharmacological and neuropathological aspects. Adv Neurol 40: 407–413

    PubMed  Google Scholar 

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Askenasy, J.J.M., Mendelson, L., Keren, O. et al. Juvenile Parkinson's disease and its response to L-dopa therapy. J Neural Transm Gen Sect 2, 23–30 (1990). https://doi.org/10.1007/BF02251243

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  • DOI: https://doi.org/10.1007/BF02251243

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