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Table 3 Ultrastructural features of myopathology of the four cerebrotendinous xanthomatosis patients

From: Neuromuscular abnormality and autonomic dysfunction in patients with cerebrotendinous xanthomatosis

  I-1 I-2 II-1 II-2
Changes of mitochondria     
   subsacrolemmal accumulation +++ +++ ++ +
   change in size and shape +++ ++ ++ +
   increase in amount +++ ++ ++ +
Changes of membranous system     
triads proliferation, swollen - ++ + -
increased amount of sacroplasmic reticulum + ++++ - +++
laminated bodies - - - ++
membranous debris +++ ++ ++ ++
Increased amount of lipid droplets + ++ +++ ++++
Increased amount of lipofuscin + + + ++
Increased amount of glycogen ++ ++ + ++
  1. + = positive; - = negative; the number of "+" = different amounts