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Distonias: conceitos, classificação e fisiopatologia

Dystonias: concepts, classification and pathophysiology

Resumos

Pacientes portadores de movimentos distônicos têm sido relatados na literatura desde o fim do século passado. A conceituação clínica do movimento distônico tem sido debatida. Atualmente, é definida como uma síndrome de contrações musculares mantidas, frequentemente causando movimentos repetitivos ou de torção, ou posturas anormais. Distonias costumam ser classificadas segundo três critérios: distribuição, idade de início e etiologia. As formas generalizadas costumam iniciar-se na infância enquanto as formas focais quase sempre se iniciam na idade adulta. Movimentos e posturas distônicas podem ocorrer durante o repouso ou apenas durante o movimento voluntário. Geralmente pioram ou são desencadeados pela adoção de posturas específicas. Não se conhecem os mecanismos fisiopatológicos responsáveis pelo aparecimento da distonia. Evidências obtidas a partir do estudo das formas secundárias sugerem o envolvimento de algumas regiões dos núcleos da base, particularmente o putâmen e o globo pálido.

distonia; núcleos da base; síndromes distônicas


Since the last decades of the past century there have been several reports in the medical literature describing patients with dystonia. The clinical and phenomenological concepts of dystonic movements have been debated. Presently, dystonia is defined as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements, or abnormal postures. Dystonia is usually classified according to three criteria: distribution, age of onset and etiology. The generalized forms often have their onset at childhood whereas the focal forms are most commonly seen in adults. Dystonic movements and postures can occur during resting or they can be precipitated by voluntary movements or by the adoption of specific posture patterns.The pathophysiologic mechanisms related to the appearance of dystonia are presently unknown. However, there are compelling evidences suggesting the involvement of some basal ganglia nuclei (e.g.putamen and globus palidus) in the development of dystonia.

dystonia; basal ganglia; dystonic syndromes


Distonias: conceitos, classificação e fisiopatologia

Dystonias: concepts, classification and pathophysiology

João Carlos Papaterra Limongi

Doutor em Medicina, Médico Assistente da Divisão de Neurologia do Hospial da Clínicas da Faculdade de Medicina da Universidade de São Paulo

RESUMO

Pacientes portadores de movimentos distônicos têm sido relatados na literatura desde o fim do século passado. A conceituação clínica do movimento distônico tem sido debatida. Atualmente, é definida como uma síndrome de contrações musculares mantidas, frequentemente causando movimentos repetitivos ou de torção, ou posturas anormais. Distonias costumam ser classificadas segundo três critérios: distribuição, idade de início e etiologia. As formas generalizadas costumam iniciar-se na infância enquanto as formas focais quase sempre se iniciam na idade adulta. Movimentos e posturas distônicas podem ocorrer durante o repouso ou apenas durante o movimento voluntário. Geralmente pioram ou são desencadeados pela adoção de posturas específicas. Não se conhecem os mecanismos fisiopatológicos responsáveis pelo aparecimento da distonia. Evidências obtidas a partir do estudo das formas secundárias sugerem o envolvimento de algumas regiões dos núcleos da base, particularmente o putâmen e o globo pálido.

Palavras-chave: distonia, núcleos da base, síndromes distônicas.

ABSTRACT

Since the last decades of the past century there have been several reports in the medical literature describing patients with dystonia. The clinical and phenomenological concepts of dystonic movements have been debated. Presently, dystonia is defined as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements, or abnormal postures. Dystonia is usually classified according to three criteria: distribution, age of onset and etiology. The generalized forms often have their onset at childhood whereas the focal forms are most commonly seen in adults. Dystonic movements and postures can occur during resting or they can be precipitated by voluntary movements or by the adoption of specific posture patterns.The pathophysiologic mechanisms related to the appearance of dystonia are presently unknown. However, there are compelling evidences suggesting the involvement of some basal ganglia nuclei (e.g.putamen and globus palidus) in the development of dystonia.

Key-words: dystonia, basal ganglia, dystonic syndromes.

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Aceite: 28-setembro-1995.

Dr João Carlos Papaterra Limongi - Rua Atlântica 460 - 01440-000 São Paulo SP - Brasil.

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Datas de Publicação

  • Publicação nesta coleção
    07 Dez 2010
  • Data do Fascículo
    Mar 1996
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