Ultrastructural differentiation of the mitochondrial myopathies
E. KOUTSOURAKI, P. HAMLATZIS, P. ARMENTZOUDIS, D. XAFENIAS, K. SIAMOULIS.
A. KARLOVASITOU, P. PAPISTAS, A. ZAFIROPOULOS, V. COSTA, S.J. BALOYANIS

We have attempted to describe in this study the clinical phenomena as well as the histological and ultrastructural findings of five cases of mitochondrial myopathies who have been hospitalized in the 1st Department of Neurology of Aristotelian University, Thessaloniki, Greece in the last decade. From the clinical point of view the principal phenomena of the cases consisted of proximal muscular weakness, fatigue and marked limitation of the lateral gaze movements. Only one of the cases demonstrated a moderate pseudohypertrophy of the gastrocnemius muscle. The examination of the muscle biopsy on electron microscope revealed an impressive polymorphism and pleomorphism of the size and shape of the mitochondria in all of the cases. The majority of the abnormal mitochondria were accumulated under the sarcolemma and around the nucleus. A substantial number of mitochondria demonstrated derangement of the cristae, though some of them included crystall like bodies or osmiophilic granules. In some of cases the marked morphological variation of the mitochondria pleads in favour of a substantial ultrastructural polymorphism of mitochondrial myopathies.

Key words: Μyopathies, mitochondrial, electron microscope.