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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.47 no.4 São Paulo Dec. 1989 

Miopatia por deficiência de succinato-citocromo-C-redutase: possível defeito no complexo II da cadeia respiratória


Myopathy due to succinate-cytoehrome-C-reductase deficiency: possible deficiency in the complex II of the respiratory chain



Lineu Cesar WerneckI; Salvatore DiMauroII

IServiço de Doenças Neuromusculares do Hospital de Clínicas, Especialidade de Neurologia do Departamento de Clínica Médica, Universidade Federal do Paraná: Professor Adjunto de Neurologia
IIH. Houston Merritt Research Center for Muscular Dystrophy and Related Diseases, Columbia University College of Physicians and Surgeons, New York: Professor de Neurologia




Relato do caso de mulher de 24 anos de idade que apresentava astenia desde a puberdade, com agravamento nos últimos anos, cuja biópsia muscular revelou grande acúmulo de mitocôndrias. As dosagens dos enzimas mitocondriais mostrou importante redução da succinato-citocromo-C-redutase, sugerindo defeito na cadeia respiratória a nível do complexo II. Medicada com altas doses de vitamina C e K, melhorou da força muscular. São feitas considerações a respeito das principais síndromes com miopatias mitocondriais, bem como a respeito dos métodos de investigação em defeitos da cadeia respiratória.


The case of a 24 years-old -woman with weakness since the teens and progressive loss of muscle strenght is reported. The muscle biopsy showed increased number of mitochondria. In two occasions the respiratory chain enzymes showed important reduction of the succinate-cytochrome-C-reductase, suggesting a possible defect in the complex II of the respiratory chain. Large doses of vitamins C and K were prescribed. There was improvement of muscle strenght. A discussion about the most common syndromes marked by mitochondrial abnormalities in muscle is made, as well as about the type of work-up that should be done in suspect cases of respiratory chain defects.



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Serviço de Doenças Neuromusculares, Especialidade de Neurologia, Departamento de Clínica Médica, Hospital de Clínicas, Universidade Federal do Paraná - Rua General Carneiro 181, 30 andar - 80060 Curitiba PR - Brasil.

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