Neuromuscular

AMYOTROPHIC LATERAL SCLEROSIS

Common patterns of denervation in muscle


 

H & E stain
 
Numerous small regions of grouped angular atrophic muscle fibers.

ATPase stain. pH 9.4

ATPase stain. pH 4.3
ATPase stain. pH 9.4
Atrophic muscle fibers in one group are often of varied types.
Little type grouping except in chronic, slowly progressive cases

NADH stain
 
Atrophic muscle fibers stain darkly
Some small muscle fibers have targets or targetoid changes


Motor axon in ALS: Collateral sprouting of terminal axon



From Bramwell: Atlas of Clinical Medicine

Spinal cord: Reduced number of motor neurons in anterior horn

Hyaline conglomerate inclusions (HCI)
    in ALS/SOD motor neurons

From A Hays MD

H & E stain
Neurofilament stain

Peripherin stain

Ubiquitin stain
Large HCI inclusions in motor neurons (Arrows)
  • May occupy most of the neuronal cytoplasm
  • Contain: Neurofilaments & peripherin but little ubiquitin
  • May be more prominent with some SOD mutations
  • Not present in sporadic ALS

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2/19/2006