Neuromuscular

AMYOTROPHIC LATERAL SCLEROSIS

Muscle
Spinal cord: Historical images
ALS-SOD1 inclusions


ALS: 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

ALS: Bramwell & Charcot spinal cord pathology images


From Bramwell: Atlas of Clinical Medicine

Spinal cord: Reduced number of motor neurons in anterior horn

ALS Spinal cord pathology: Original patients of JM Charcot

Case reports:
  English translation from Google books
  Illustrations from original article

Case 1

From: R Baloh

Legend: "Section of the cord of Catherine A. (Case 1), taken from the superior cervical region.
  In a, one can see the rare cells and cell debris in the anterior horns.
  In b, sclerosis of the lateral columns, determined by the atrophy of a large number of axons."


From: R Baloh 		Legend: "Symmetric sclerosis of the posterior part of the
  lateral columns in the dorsal region (Case 2)"

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

Return to Neuromuscular Home Page
Return to Pathology index
Return to ALS


7/31/2009