Neuromuscular

Batten Disease: Muscle & Nerve Pathology


Muscle Pathology

Gomori trichrome stain
Muscle Fiber Morphology
  Mildly irregular internal architecture, especially in darker stained fibers

VvG stain

Muscle Fiber Morphology
  Mildly irregular internal architecture, with linearization in paler stained fibers

NADH stain


Congo red stain
Myonuclei
  Size: Often large
  Shapes: Irregular

Congo red stain


Acid phosphatase stain
Acid phosphatase + Granules
  Several large, dark granules present in most muscle fibers

Acid phosphatase stain


Acid phosphatase stain
Acid phosphatase + Granules
  Granules present in vessel walls

Acid phosphatase stain

Acid phosphatase stain
Acid phosphatase + Granules
  Granules present in vessel walls

Acid phosphatase stain

Acid phosphatase stain


LAMP2 stain
Batten disease muscle
  LAMP2 granules are prominent in muscle fiber cytoplasm

LAMP2 stain

Control muscle
  LAMP2 granules are not prominent in muscle fiber cytoplasm

LAMP2 stain


COX stain
Mitochondrial stains
  No mitochondrial pathology

SDH stain

Batten disease: Nerve pathology

H&E stain
Nerve morphology
  Normal endoneurium, perineurium & epineurium


Gomori trichrome stain
Myelinated axons
  Mild loss

VvG stain
Small unmyelinated axons
  Normal numbers

Neurofilament stain


Acid phosphatase stain
Acid phosphatase + Granules
  Scattered in endoneurium
  Present in walls of epineurial vessels

Acid phosphatase stain

Acid phosphatase stain
Acid phosphatase + Granules
  Present in walls of epineurial vessels

Acid phosphatase stain

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4/8/2023