Generalized giant axonal neuropathy: a filament-forming disease of neuronal, endothelial, glial, and schwann cells in a patient without kinky hair

J Peiffer, W Schlote, A Bischoff, E Boltshauser… - Acta …, 1977 - Springer
J Peiffer, W Schlote, A Bischoff, E Boltshauser, G Müller
Acta neuropathologica, 1977Springer
Summary The process of Giant Axonal Neuropathy (GAN) is not restricted to the peripheral
nerves, but also involves the central nervous system. In a 25 year old man with normal hair,
abundant axon swellings and spheroids were observed in the spinal cord, brain system, and
cerebral cortex. The findings in the sural nerve have already been published by Boltshauser
et al.(1977). Accumulations of filaments in the axons and in the perineural cells were
accompanied by Rosenthal fibres. The ultrastructural pattern of GAN differs clearly from that …
Summary
The process of Giant Axonal Neuropathy (GAN) is not restricted to the peripheral nerves, but also involves the central nervous system. In a 25 year old man with normal hair, abundant axon swellings and spheroids were observed in the spinal cord, brain system, and cerebral cortex. The findings in the sural nerve have already been published by Boltshauser et al. (1977). Accumulations of filaments in the axons and in the perineural cells were accompanied by Rosenthal fibres. The ultrastructural pattern of GAN differs clearly from that of Neuroaxonal Dystrophies.
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