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Arquivos de Neuro-Psiquiatria

versión impresa ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.50 no.4 São Paulo dic. 1992

http://dx.doi.org/10.1590/S0004-282X1992000400003 

Idiopathic dystonia clinical, profile of 76 brazilian patients

 

Distonia idiopática: perfil clínico de 76 pacientes brasileiros

 

 

Luiz A. F. AndradeI; Henrique B. FerrazII

IAssociate Professor of Neurology. Sector for Investigation of Extrapyramidal Diseases, Department of Neurology and Neurosurgery, Escola Paulista de Medicina, São Paulo
IIPostgraduate in Neurology. Sector for Investigation of Extrapyramidal Diseases, Department of Neurology and Neurosurgery, Escola Paulista de Medicina, São Paulo

 

 


SUMMARY

Dystonia may be classified by age of onset (childhood, adolescence, adult onset), body distribution of the abnormal movements (focal, segmental, unilateral, multifocal and generalized) and etiology (idiopathic and symptomatic). We studied 76 patients with idiopathic dystonia among 122; cases of dystonic syndrome (62.3% of the total). There were 48 female and 28 male patients. Adult-onset focal dystonia was the most frequent feature (37 patients). The onset of generalized dystonia was more frequently seen under the age of 20, whereas focal and segmental dystonia usually started over this age. Postural tremor of the hands was observed in 19.7% of the patients. Spasmodic torticollis was the most prevalent form of dystonia overall. Except for writer's cramp, which occurred more frequently in males, and generalized dystonia, which was equally divided between sexes, all other forms were more frequent in females. Our data suggest that differences in racial origin, social and economical status and environmental factors do not account for a different manifestation in dystonia pattern.

Key words: dystonia, dystonia musculorum deformans, idiopathic dystonia.


RESUMO

A distonia pode ser classificada de acordo com a idade de início (infância, adolescência e idade adulta), distribuição corporal dos movimentos anormais (focal, segmentar, unilateral, multifocal e generalizada) e etiologia (idiopática e sintomática). Dentre 122 pacientes com o diagnóstico de síndrome distônica, estudamos 76 com quadros idiopáticos (62,3% do total). Havia 48 pacientes do sexo feminino e 28 do sexo masculino. O quadro mais frequentemente observado foi a da distonia focal iniciada na idade adulta. (37 pacientes). Havia 6 pacientes com distonia generalizada e o início desse quadro foi mais frequento abaixo dos 20 anos de idade. Quadros focais e segmentares predominaram e foram mais comumentemente iniciados na idade adulta. Tremor postural das mãos foi observado em 15 pacientes (19,7%). De todas as formas de distonia, o torcicolo espasmódico foi a que prevaleceu. Com a exceção da cãimbra do escrivão (com mais homens que mulheres acometidas) e da distonia generalizada (a mesma proporção entre os sexos), o sexo feminino predominou sobre o masculino. Nossos dados são semelhantes aos de outras séries que estudaram o quadro clínico da distonia idiopática. Assim, diferenças raciais, ambientais e sócio-econômicas não parecem ser determinantes: no padrão de manifestação da distonia idiopática.

Palavras-chave: distonia, dystonia musculorum deformans, distonia idiopática.


 

 

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

 

 

Acknowledgments — We thank to CNPq, and FINEP for partial financial support of this study.

 

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Dr. Luiz Augusto Franco de Andrade — Rua Borges Lagoa 1231, Conj. 44 - 04038 São Paulo SP - Brasil.