Neuromuscular

Myopathy with NMO & Aquaporin-4 (AQP4) Antibodies

Capillaries
Histiocytes
Muscle fibers
  Aggregates & Vacuoles
  Cytoplasmic bodies
  C5b-9 deposition
  MHC Class I
Pathology patterns
Perimysium

Muscle Fibers


H& E stain
Pattern
  Atrophy & Immaturity
  More pathology at edges of fascicles

H & E stain

ATPase pH 4.3 stain

Perimysial Connective Tissue


Alkaline phosphatase stain
Alkaline phospatase: Strong staining of perimysium & some neighboring endomysium
Acid phosphatase: Stains cells in perimysium & endomysium

Acid phosphatase stain

Acid phosphatase stain
Histiocytes
  Acid phosphatase cells scattered in endomysium & perimysium

Acid phosphatase stain

Muscle Fiber Pathology


H & E stain
Small muscle fibers
  Often immature (Basophilic)
  Internal nuclei
  Internal architecture
    Sworled
    Vacuoles & Aggregates

VvG stain

NADH stain

H & E stain

H & E stain

H& E stain

NADH stain

AMPDA blue stain


VvG stain

VvG stain

Cytoplasmic bodies

Gomori trichrome blue stain

Capillary pathology
  Some capillaries are large & have thickened basal lamina (Arrow)
  There is a regional loss of endomysial capillaries in some areas

Alcian blue stain


UEA1 stain

UEA1 stain

C5b-9 deposition
  Endomysial
  On surfaces of muscle fibers

C5b-9 stain

MHC-I upregulation by muscle fibers
  In regions with muscle fiber atrophy

MHC1 stain

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7/17/2019