Summary
There have been several recent descriptions of myopathies associated with disordered oxidative metabolism in muscle fibres and particularly with accumulation of fat within the fibres. Some of these have been associated with abnormal muscle mitochondria but in others there may be an extramitochondrial defect of lipid metabolism. In this paper we describe a 38 year old man suffering from a progressive myopathy which clinically resembled polymyositis and which responded to treatment with steroids. Pathologically the condition was characterised by an apparent increase in the amount of lipid within the muscle fibres, an observation subsequently confirmed by quantitative chemical analysis, although the muscle mitochondria appeared to be normal. This case closely resembles others recently described in this department and elsewhere and it is suggested that they may be examples of a previously unrecognized clinico-pathological entity.
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Johnson, M.A., Fulthorpe, J.J. & Hudgson, P. Lipid storage myopathy: A recognizable clinicopathological entity?. Acta Neuropathol 24, 97–106 (1973). https://doi.org/10.1007/BF00684832
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DOI: https://doi.org/10.1007/BF00684832