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Affected nerves shows myelin
sheath and axon degeneration. The large myelinated fibers are affected, demonstrating
segmental demyelination and macrovacuolization. Both the motor and sensory fibers of the
peripheral nerves demonstrate fatty degenerative changes and disintegration of the
medullary sheaths due to demyel-ination by toxin. The anterior horn cells and posterior
columns of the spinal canal can be involved, and the central nervous system may develop
signs of hemorrhage, meningitis and encephalitis. Nonetheless, the polyneuro-pathy is
reversable. At autopsy, the heart is pale brown,
soft and enlarged with a characteristic streaky appearance. Neutral fat accumulations are
observed in approximately 50% of patients, with extensive hyaline degeneration and
necrosis with inflammatory changes. EM demonstrates swollen disorganized mitochondria
containing dense osmophilic granules. |