A Mutation in PMP2 Causes Dominant Demyelinating Charcot-Marie-Tooth Neuropathy
Fig 3
Histology of the distal sural nerve.
(A) Transverse semi-thin section showed a normal range of myelinated fibers with loss of large myelinated fibers, onion bulbs (arrows), axonal degeneration with myelin abnormalities (arrow heads), and clusters of regenerative small myelinated fibers (curved arrow). (B) Histogram shows a unimodal distribution pattern. (C, D) Ultrastructural micrograph shows degenerating fibers with various abnormalities of myelin such as abnormal myelin compaction with adaxonal vacuoles, internal and external myelin folding, irregular myelin sheath, and onion bulbs or pseudo-onion bulbs surrounding the myelinated fibers. Magnifications: A, x400; C, x3000; D, x15000.