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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.33 no.2 São Paulo June 1975 

Neuropathy in myotubular or centronuclear myopathy


Neuropatia na miopatia miotubular ou centronuclear



Roberto E. P. SicaI; Olga P. SanzII

ISección de Electroneurofisiologia Clínica, Servicio de Neurología, Hospital Ramos Mejía, Buenos Aires
Municipalidad de la Ciudad de Buenos Aires Medical Research Council Scholar




A detailed electrophysiological study has been made of the extensor digitorum brevis, thenar, hypothenar and soleus muscles in one patient with myotubular or centronuclear myopathy. The main finding was a noticeable reduction in the population of active motor units in all the investigated muscles. The remainer units showed normal sizes. The experimental observations have been interpreted in terms of a neuropathic process.


Un estudio electrofisiológico detallado fué hecho en los músculos extensor corto de los dedos, de la eminencia tenar, de la eminencia hipotenar y soleo en un paciente con el diagnóstico de miopatía miotubular o centronuclear. El hallazgo principal fué una notoria reducción en el número de unidades motoras activas en todos los músculos investigados, en tanto que las unidades remanentes mostraron tamaño conservado. Las observaciones hechas se han interpretado como favoreciéndo la génesis neurógena en el desarrollo de este proceso.



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Sección de Eletroneurofisiología — Servicio de Neurología — Hospital Ramos Mejía — Urquiza 609 — Buenos Aires, Argentina.
— The authors are indebted to Dr. Ortiz de Zárate for permission to study his patient.

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