Neuromuscular

SPINAL MUSCULAR ATROPHY
Scapuloperoneal neuronopathy: TRPV4 mutations



H & E stain
Morphology
Muscle fibers
  Small & Intermediate-sized
    Angular & Polygonal
  Hypertrophic
  Internal nuclei: Often multiple
Grouped muscle fiber atrophy

H & E stain
Internal nuclei

Congo red stain
Pyknotic nuclear clumps

Congo red stain

Internal architecture: Irregular in larger muscle fibers


NADH stain

NADH stain


VvG stain
Chronic changes
  Internal nuclei
  Partial fusion of muscle fibers
  Capillary inside muscle fiber
  Pyknotic nuclear clumps

Gomori trichrome stain

Cytoplasmic bodies in a small muscle fiber

Gomori trichrome stain

Muscle fiber types: Type 1 predominance; No fiber type grouping

ATPase, pH 9.4

Vacuoles in Muscle fibers




H & E stain

H & E stain

Gomori trichrome stain

Congo red stain
Vacuoles
  Contain basophilic granular debris
  May be near eosinophilic cytoplasmic bodies

Congo red stain

Congo red stain

Congo red stain

Aggregates
  Irregular; Dark; Cytoplasmic
  May be near or around internal nuclei

Caveolin-3 stain

Caveolin-3 stain

Caveolin-3 may also stain rim of vacuoles

Caveolin-3 stain
Caveolin-3 staining around internalized capillary

Caveolin-3 stain

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2/17/2016