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Consideration on two cases of dystonia-parkinsonism

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Abstract

We describe two sporadic cases of dystonia-parkinsonism at different stages of disease progression. The two girls, first seen at the ages of 10 and 12 years, have been followed for 9 and 2 years respectively. In both patients L-dopa 60 mg+carbidopa 6 mg brought about a swift remission of symptoms, which persists to date. All examinations, including CT and MR brainscans, were normal. The CSF and urine levels of HVA and 5 HIAA were low in one case and normalized with treatment. This finding might provide a fairly valid predictive index of responsiveness to L-dopa.

Sommario

Gli autori descrivono due casi sporadici di Distonia-parkinsonismo in differenti stadi di progressione della malattia.

Si tratta di due ragazze viste per la prima volta rispettivamente a 10 e 12 anni e seguite per 9 e 2 anni. Dosi di 60mg di L-dopa+6mg di Carbidopa hanno determinato in entrambe una regressione pronta e completa dei sintomi che persiste a tutt'oggi.

I livelli di HVA e 5HIAA sono risultati abbassati sia a livello liquorale che urinario in un caso e si sononormalizzati con la terapia.

Questo dato potrebbe costituire un indice sufficientemente valido di predizione dell'efficacia della cura con L-dopa.

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References

  1. Balottin U., Lanzi G., Zambrino C.A.:Illustrazione di un caso di Dystonia Musculorum Deformans trattato con L-Dopa. Rivista di Neurobiologia XVII-3–XVII-4:584–590, 1981.

    Google Scholar 

  2. Bedard P., Languellier P., Villeneuve A.:Estrogens and extrapyramydal system. Lancet 2:1367–1368, 1977.

    CAS  PubMed  Google Scholar 

  3. De Jong A.P.J.M., Haan E.A., Manson J.I. et al.:Kinetic study of catecholamine metabolism in hereditary progressive dystonia. Neuropediatrics 20:3–11, 1989.

    PubMed  Google Scholar 

  4. Deonna T.:DOPA-sensitive progressive dystonia of childhood with fluctuation of symptoms — Segawa's Syndrome and possible variants. Neuropediatrics 17:81–85, 1986.

    CAS  PubMed  Google Scholar 

  5. Garg B.P.:Dystonia musculorum deformans: implications of therapeutic response to levodopa and carbamazepine, Arch. Neurol. 39:376–377, 1982.

    CAS  PubMed  Google Scholar 

  6. Halliday G.M., Li Y.W., Blumbergs P.C. et al.:Neuropathology of immunohistochemically identified brainstem neurons in Parkinson's disease. Ann. Neurol. 27,4:373–385, 1990.

    Article  CAS  PubMed  Google Scholar 

  7. Nygaard T.G., Duvoisin R.C.:Hereditary dystonia-parkinsonism syndrome of juvenile onset. Neurology 36:1424–1428, 1986.

    CAS  PubMed  Google Scholar 

  8. Obeso J.A., Luguin M.R., Artieda J.:Motor manifestations of basal ganglia disease. Pharmacological studies in humans. In M. Sandler et al. (Eds.) Neurotransmitters Interactions in the Basal Ganglia, Raven Press, New York, 205–217, 1987.

    Google Scholar 

  9. Ouvrier R.A.:Progressive dystonia with marked diurnal fluctuation. Ann. Neurol. 4:412–417, 1978.

    Article  CAS  PubMed  Google Scholar 

  10. Robinson R.O., Hicks B., Stuchfield P., Mardsen C.D.:A case of oestrogen sensitive dystonia. Congress of the European Federation of Child Neurology Societies, Nordwijkerhuit, Holland, may 1983.

  11. Rolando S., Cremonte M.:Distonia progressiva con marcata fluttuazione mattino-sera. Istituto Gaslini 14:176–179, 1982.

    Google Scholar 

  12. Segawa M., Hosaka A., Miyagawa F., Nomura Y., Imai H.:Hereditary progressive dystonia with marked diurnal fluctuation. In Fahn S., Eldridge R., Adv. Neurol. 14:215–233, 1976.

    CAS  PubMed  Google Scholar 

  13. Sunohara N., Mano Y., Ando K., Satoyoshi E.:Idiopathic dystonia parkinsonism with marked diurnal fluctuations. Ann. Neurol. 17:39–45, 1985.

    Article  CAS  PubMed  Google Scholar 

  14. Willemse J., van Nieuwenhuizen O., Gooskens R.H., Westernberg H.G.M.:Treatment of non-fluctuating progressive dystonia: a neuropharmacological approach. Neuropediatrics 15:208–210, 1984.

    CAS  PubMed  Google Scholar 

  15. Yokochi M., Narabayashi H., Iizuka R., Nagatsu T.:Juvenile parkinsonism — some clinical, pharmacological and neuropathological aspects. Ann. Neurol. 40:407–413, 1984.

    CAS  Google Scholar 

  16. Zeman W., Dyken P.:Dystonia musculorum deforman. In Vinken P.J., Bruyn G.W. (Eds.), Handbook of Clinical Neurology. Vol. 6, Amsterdam-North Holland 517–543, 1978.

    Google Scholar 

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Authors and Affiliations

  1. Divisione di Neuropsichiatria Infantile, Fondazione “Istituto Neurologico C. Mondino”, IRCCS, Università di Pavia, Via Palestro, 3-27100, Pavia

    Zambrino C. A., Balottin U., Borgatti R. & Lanzi G.

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  1. Zambrino C. A.
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  2. Balottin U.
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  3. Borgatti R.
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  4. Lanzi G.
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Zambrino, C.A., Balottin, U., Borgatti, R. et al. Consideration on two cases of dystonia-parkinsonism. Ital J Neuro Sci 12, 475–478 (1991). https://doi.org/10.1007/BF02335509

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  • DOI: https://doi.org/10.1007/BF02335509

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