Onion bulb neuropathy

Compared with the normal ultrastructure of axons in a nerve (A), an “onion bulb” (B) is composed of a thinly myelinated axon (arrow) surrounded by multiple concentrically arranged Schwann cells.

■ Inset, Light-microscopic appearance of an onion bulb neuropathy, characterized by “onion bulbs” surrounding axons

● It is due to SEGMENTAL MYELINATION AND DEMYELINATION…

● It is SEEN in–

  1. CIDP(Chronic Inflammatory Demyelinating polyneuropathy)
  2. CHARCOT MARIE TOOTH DISEASE
  3. refsums disease and dejerine sottas syndrome, adreno leukodystrophy