Neuromuscular

LIPID STORAGE

Differential Diagnosis
Types

Infant
Child
Child: Ketogenic diet
Leigh: SURF1
Adult: HIV
Adult: ETFDH
Ultrastructure

Lipid storage: Child

H & E stain
Droplets
  Multiple, Small & Clear
  Most & Largest in Type I fibers

Gomori Trichrome stain

H & E stain


Sudan black stain
Lipid droplets
  Large lipid droplets in type I muscle fibers.
  Smaller or less abundant lipid droplets in other fibers

Sudan black stain

Large lipid droplets are in type I muscle fibers.

ATPase pH 9.4 stain
Lipid droplets: Less staining than myelin lipid

VvG stain

Internal architecture: Punctate

NADH stain

Lipid storage: Child on ketogenic diet

  • H & E and GT stains: Multiple, Small, clear regions in muscle fibers
  • Sudan black: Stains many, moderate sized lipid droplets in all muscle fibers
  • AMPDA: Punctate cytoplasm

H & E stain

Gomori trichrome stain

Sudan stain

AMPDA stain

Lipid storage: Adult with HIV
  • Small, clear vacuoles: H & E, GT & ATPase stains
  • Abnormal internal architecture with a punctate pattern: NADH & SDH
  • Large lipid droplets in type I muscle fibers: Sudan black

H & E stain

 

Gomori Trichrome stain

ATPase pH 9.4 stain

NADH stain

SDH stain

Sudan black stain

 

Sudan black stain

Lipid storage: Infant

Lipid droplets
  Large
  Multiple in each muscle fiber
  Most in type I muscle fibers

Internal architecture: Punctate

Lipid: Ultrastructure


From: Cory Toth MD
Lipid droplets in muscle fibers: Ultrastructure

From: Oliver Ni



Lipid Disorders

Lipid storage in a muscle fiber

Neighboring muscle fibers with no lipid storage

Muscle fibers in Lipid-storage Myopathy
  Top fiber: Lipid is Abundant
  Bottom fiber: Minor increased lipid

Muscle fibers in Lipid-storage Myopathy
  Top fiber: Lipid is Abundant
  Bottom right fiber: Minor increased lipid

Lipid storage: Lipid droplets & closely neighboring Mitochondria

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Lipid myopathies: Classification

3/22/2021