Neuropathy and mitochondrial myopathy

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Abstract
It has been stated that peripheral neuropathy can be a feature of so‐called ophthalmoplegia‐ plus syndrome, but to date one case of hypertrophic neuropathy has been reported. This study is concerned with the clinical, electrophysiology, and pathological observations made in a 37‐year‐old man with progressive extyernal ophthalmcmetric studi9ees of the sural nerve revealed a marked loss of large myelinated fibers and an occasional degenerating axon. Residual fibers had disproportionately thin myelin sheaths in reelation to axon calibedrs. In contrast to the muscle biopsy findings, no mitochondrial paracrystaline inclusions were observed in the nerve. However, the number of mitochondria per square micron of Schwann cell cytoplasm was wlevated when compared with values obtained from normal subjects and a patient with a chronic neuropathy. These findings may indicate an alteration of mitochondrial function common to muscle fibers and Schwann cels which, in nerves, could lead to axon loss and abnormality of myelination.