Neuromuscular

Cystinosis



H&E stain

H&E stain
Muscle fibers: Varied sizes
Muscle fibers: Vacuoles, irregular shaped; Large nuclei

VvG stain
Vacuoles in muscle fibers


VvG stain

NADH stain
Internal architecture: Mildly irregular

Vacuoles: Contain
  Basophilic granular debris
  Red-green birefringent material

Congo red stain

Congo red stain


Congo red stain

Vacuoles: Associated with AMPDA positive agggregates

AMPDA stain
Acid phosphatase positive material: Some associated with vacuoles

Acid phosphatase stain
Immature muscle fibers

ATPase pH 4.3 stain

Return to Cystinosis
Return to Muscle biopsies

8/11/2014