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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

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

http://dx.doi.org/10.1590/S0004-282X1975000200003 

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
II
Municipalidad de la Ciudad de Buenos Aires Medical Research Council Scholar

 

 


SUMMARY

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.


RESUMEN

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.


 

 

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

 

 

REFERENCES

1. BETHLEM, J.; VAN WIJNGAARDEN, G. M.; MUMENTHALER, M. & MEIJER, H. — Centronuclear myopathy with type I fiber atrophy and myotubes. Arch. Neurol. (Chicago) 23:70, 1970.         [ Links ]

2. CAMPBELL M. J.; REBEIZ, J. & WALTON J. — Myotubular, centronuclear or pericentronuclear myopathy. J. neurol. Sci. 8:425, 1969.         [ Links ]

3. ENGEL, W. K.; GOLD, G. M. & KARPATI, G. — Type I fiber hypotrophy and central nuclei. Arch. Neurol. (Chicago) 18:435, 1968.         [ Links ]

4. KINOSHITA, M. & CADMAN, T. E. — Myotubular myopathy. Arch. Neurol. (Chicago) 18:265, 1968.         [ Links ]

5. McCOMAS, A. J.; FAWCETT, P.; CAMPBELL, M. J. & SICA, R. E. P. — Eletrophysiological estimation of the number of motor units within a human muscle. J. Neurol. Neurosurg. Psychiat. 34:121, 1971.         [ Links ]

6. McCOMAS, A. J.; SICA, R. E. P. & CAMPBELL, M. J. — "Sick" motoneurones, a unifying concept of muscle disease. Lancet 1:321, 1971.         [ Links ]

7. ORTIZ DE ZARATE, J. C. & MARUFFO, A. — The descending ocular myopathy of early childhood. Myotubular or centronuclear myopathy. Europ. Neurol. 3: 1, 1970.         [ Links ]

8. SCHOCHET, S. S.; ZELLWEGER, H.; IONASESCU, V. & McCORMICK, W. F. — Centronuclear myopathy: disease entity or a syndrome? Light and electronmicroscopy study of two cases and review of the literature. J. neurol. Sci. 16: 215, 1973.         [ Links ]

9. SHER, J. H.; RIMALOVSKI, A. B.; ATHANASSIADES, T. J. & ARONSON, S. M. — Familiar myotubular myopathy: a clinical, pathological, histochemical and ultrastructural study. J. Neurop. Exp. Neurol. 26: 132, 1967.         [ Links ]

10. SICA, R. E. P. & McCOMAS, A. J. — An electrophysiological investigation of limb-girdle and facioscapulohumeral dystrophy. J. Neurol. Neurosurg. Psychiat. 34:469, 1971.         [ Links ]

11. SICA, R. E. P.; McCOMAS, A. J.; UPTON, A. R. M. & LONGMIRE, D. — Motor unit estimations in small muscles of the hand. J. Neurol. Neurosurg. Psychiat. 37:55, 1974.         [ Links ]

12. SPIRO, A. J.é SHY, G. M. & GONATAS, N. K. — Myotubular myopathy: persistance of fetal muscle in a adolescent boy. Arch. Neurol. (Chicago) 14:1, 1966.         [ Links ]

 

 

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

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