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Neurons of origin of the rat sural nerve were labelled with horseradish peroxidase. Dorsal root ganglionic cells were located in the L4 and L5 ganglia, and occasionally at the L6 level. Most of these sensory neurons measured under 35 microns in diameter. In keeping with previous electrophysiological studies suggesting the presence of motor fibers to plantar muscles in the rat sural nerve, motoneurons were identified at the caudal end of the L5 spinal segment, intermingled in the posterior aspect of the ventral horn with posterior tibial motor cells supplying the foot muscles. A quantitative analysis of HRP-labelled motoneurons revealed no difference between normal (average 67) and deafferented animals (average 70), the values being only marginally lower than counts of motor axons in deafferented sural nerves (average 80).

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It has been stated that peripheral neuropathy can be a feature of so-called ophthalmoplegia-plus syndrome, but to date only one case of hypertrophic neuropathy has been reported. This study is concerned with the clinical, electrophysiological, and pathological observations in a 37-year-old man with progressive external ophthalmoplegia and a ragged-red fiber myopathy associated with severe sensorimotor neuropathy. Histological and morphometric studies 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 relation to axon calibers. In contrast to the muscle biopsy findings, no mitochondrial paracrystalline inclusions were observed in the nerve. However, the number of mitochondria per square micron of Schwann cell cytoplasm was elevated 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 cells which, in nerves, could lead to axon loss and abnormality of myelination.

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SummaryTwo female patients aged 30 and 40 years with the Charlevoix-Saguenay ataxia were studied. Both had absent sensory action potentials in upper and lower extremities but, unlike typical cases of Friedreich's ataxia, they displayed a marked slowing of motor conduction velocities. Sural nerve biopsies taken from calf and ankle revealed a severe loss of large my elina ted axons contrasting with a normal myelinated fiber density. Evidence for active axonal degeneration was scarce, with no indication of axonal regeneration.Teased myelinated fibers revealed an increased variability of internodal length but no evidence for myelin breakdown. These findings support, as a primary defect, a developmental abnormality of peripheral nerve, namely a lack of maturation of large myelinated axons and possibly a faulty myelination of nerve fibers. We think it is unlikely to represent a progressive axonal atrophie or dystrophic process, as suggested in Friedreich's ataxia.