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Electron-microscopic heterogeneity of onion-bulb neuropathies of the Déjerine-Sottas type

Two patients in one family with the variant described by Lyon (1969)

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Summary

The microscopic and electron microscopic findings in sural nerve biopsies of a brother and sister with chronic polyneuropathy of the Dejerine-Sottas type resemble those reported by Lyon (1969). A genetically determined variant appears to be involved.

The relationship between this syndrome and onion-bulb neuropathies is discussed. It seems unlikely that only segmental demyelination and remyelination is responsible for onionbulb formation. The relationship between axons in the layers of onion-bulbs and the central fibre is discussed, and between basement membranes and Schwann cell processes and the central fibre. Significant quantitative evidence of axon degeneration and regeneration after primary axon degeneration is pointed out. Earlier probable cases in the literature are reviewed.

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Joosten, E., Gabreëls, F., Gabrééls-Festen, A. et al. Electron-microscopic heterogeneity of onion-bulb neuropathies of the Déjerine-Sottas type. Acta Neuropathol 27, 105–118 (1974). https://doi.org/10.1007/BF00687161

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  • DOI: https://doi.org/10.1007/BF00687161

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