Neuromuscular

AXON SWELLINGS

General
Giant Axonal Neuropathy
Polyglucosan bodies
Swellings
  Sprouts with membrane profiles


Axon Swellings


Giant Axonal Neuropathy


From Pr P Landrieu
Many enlarged thinly myelinated, or unmyelinated, axons.
Reduced number of myelinated axons.


From Pr P Landrieu
Enlarged unmyelinated, or thinly myelinated, axons.


From Pr P Landrieu
Teased axons with focal swellings (Arrows).

From T Mozaffar
Long curly eyelashes; Curled scalp hair

Polyglucosan Bodies



H&E stain

 



VvG stain
Intramuscular axons: Focal swellings

VvG stain
 


VvG stain
Intramuscular axons: Focal swellings

PAS stain
Intramuscular nerve: Polyglucosan bodies


PAS stain

 

 


Polyglucosan Bodies: Swellings in Peripheral nerve axons

Toluidine blue stain

 


Ultrastructure: From Robert Schmidt
Polyglucosan Bodies


Polyglucosan Body Disease



Axon Swellings: Enlarged Unmyelinated axons, or Axon sprouts


Enlarged axon: Contains tubulovesicular profiles

Enlarged axon: Contains organelles & tubulovesicular profiles

Tubulovesicular profiles

Tubulovesicular profiles & Peripheral organelles
2/13/2018
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