mortality/aging
• deaths occurred at 332 and 354 days of age due to severe paralysis of all four limbs
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nervous system
• at 350 days of age
• atrophy of the anterior horn due to loss of neurons
• filamentous inclusions of varying size and shape in neurons and axons
• mild rarefaction of the neuropil, mild increase in astrocytic nuclei
• rare examples of vacuolar changes, sometimes in surviving neurons, sometimes in dendrites or axons
• only minimal involvement of the posterior columns
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• the largest fibers in brachial and lumbo-sacral plexuses are completely replaced by strings of large lipid laden macrophages
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• the largest fibers in lumbo-sacral plexuses are completely replaced by strings of large lipid laden macrophages
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• loss of axons, increased perineurial space and, frequently, numerous myelin debris-laden macrophages
• no involvement of the posterior roots.
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