Generalized giant axonal neuropathy |
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| Authors: | Jürgen Peiffer Wolfgang Schlote Albert Bischoff Eugen Boltshauser Günter Müller |
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| Affiliation: | (1) Institute of Brain Research, University of Tübingen, Calwer Str. 3, D-7400 Tübingen, Germany;(2) Department of Submicroscopic Pathology and Neuropathology, University of Tübingen, Calwer Str. 3, D-7400 Tübingen, Germany;(3) Department of Neurology, University of CH-3000 Bern, Switzerland;(4) Department of Pediatrics, University of CH-8000 Zürich, Switzerland;(5) Department of Pathology, Kantonspital CH-7000 Chur, Switzerland |
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| Abstract: | 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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| Keywords: | Giant axonal neuropathy Neuroaxonal dystrophies Kinky hair Rosenthal fibres Neurofilaments |
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