Schwann cell length in unmyelinated fibres of human sural nerve.
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Biomedical subjects
Publications and source records attributed to F Behse.
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For comparison with findings in neuropathy, sensory conduction was studied along distal and proximal segments of the superficial peroneal, sural, and posterior tibial nerves in 71 healthy subjects 15 to 72 years of age and normal values were established (Table 2). In the distal segments of the nerves of the leg the amplitudes of the sensory potentials were one tenth those in the nerves of the upper extremity; the potentials were split up into several components, and electronic averaging was used routinely to analyse the shape of the potentials. The maximum sensory conduction velocity was 56·5 m/sec, SD 3·4 m/sec, in proximal; and 46·1 m/sec, SD 3·7 m/sec, in distal segments of the nerves (subjects 15 to 30 years, 34 to 36°C). Slowing of conduction with increasing age was the same proximally and distally (subjects 40 to 65 years: proximally 53·1 m/sec, SD 4·6 m/sec; distally 42·5 m/sec, SD 5·5 m/sec, 34 to 36°C). The velocity in the slowest components of the sensory potentials averaged 20 m/sec. The sensory velocity was 3 to 6 m/sec faster than the motor. The error arising from measuring the conduction distance on the surface across the capitulum fibulae was evaluated.
Histologic findings are described in nerves from men exposed to lead, from patients with discrete clinical signs of peripheral neuropathy, and from controls. Every nerve from control subjects showed an abnormality (paranodal remyelination, segmental remyelination, or regeneration) in teased fibers. The only histologic alteration in eight lead-exposed males without signs or symptoms of neuropathy was a slightly increased incidence of paranodal remyelination. Sixteen patients with discrete neurologic symptoms and signs had a loss of large myelinated fibers and an increased incidence of regenerated fibers among teased fibers. Electron microscopy of unmyelinated fibers showed an increased occurrence of Schwann-cell processes, of fibers undergoing degeneration, and of Schwann-cell subunits with many profiles as the earliest signs of abnormality. Clinically mild neuropathies may exhibit advanced regeneration in the case of unmyelinated fibers. The earliest sign of degeneration in myelinated fibers was a diminution in the number of axonal organelles.
Reports on biopsy findings in multifascicular nerves in lepromatous leprosy (LL) are rare and detailed morphometrical data are not available. In a case of early LL with normal electrodiagnostic findings in sural nerve, the present study revealed marked segmental de- and remyelination concomitant with the sequelae of considerable Wallerian degeneration of preferentially small myelinated fibers (MF) in spite of a normal number/nerve and density/mm2. Segmental de- and remyelination of several consecutive internodes in teased fibers suggests continuous bacterial spread via Schwann cells. In 2 more advanced LL-cases, nervous parenchyme was severely reduced, in a borderline lepromatous (BL) case obviously in part caused by cell infiltrates and granulomata. Distinct fascicle differences in MF-involvement were demonstrated by coefficients of variation of MF/mm2 and teased fiber preparations in LL, consistent with the hypothesis of initial focal spread of bacteria. Numbers and densities of endoneurial vessels were increased only in the later stages of LL. Enlargement of endoneurial area, due to different factors, was encountered except for the most severe LL-case with extensive endoneurial collagenization. Morphometric results were compared with those of other neuropathies. Intact and degenerating bacteria mostly in phagosomes of Schwann cells associated with unmyelinated axons and in macrophages were seen only in the early LL- and the BL-case. Sparse mononuclear cell infiltrates and small focal necrosis, present even in LL-cases, underline the complex pathogenesis of nerve fiber involvement.
We present data of 2 patients suffering from chronic motor-sensory multifocal neuropathy with persistent conduction block. The first case concerns a 9-year follow-up of a female, aged 24 years at onset with persistent multiple conduction blocks and a tendency towards generalization of clinical deficits. Eight years after onset sural nerve biopsy revealed extreme interfascicular variations of de/remyelination, onion bulb formation, fiber loss, edema, and proliferation of basal lamina of endoneurial capillaries. Serum antibodies against GM1 gangliosides were not detected. The second case, a 29-year-old Yemenitic male with a 5-year history, exhibited conduction blocks in motor and sensory fibers, and a normal sural nerve biopsy. Our results are discussed with respect to those of some 30 cases individually reported in the literature.