Table 2 Muscle pathology in patients with selectively high aldolase
Patient No 1Patient No 2Patient No 3Patient No 4Patient No 5Patient No 6Patient No 7Patient No 8Patient No 9Patient No 10Patient No 11Patient No 12% +ve
Perimysial pathology92
    Fragmentation+++++++++75
    Acid phosphatase + cells++++++++++83
    Alkaline phosphatase stain+++++++58
Inflammation
    Mononuclear cell foci50
        Endomysial+++25
        Perivascular+++25
Muscle fibre pathology
    Perifascicular58
        Atrophy+++++++58
        Necrosis and regeneration++++33
        COX stain reduced++17
        MHC-I staining++++++++++83
Small vessel pathology17
    Alkaline phosphatase stain0
    C5b-9 deposition++17
    Capillary number reduced+8
  • Muscle pathology findings: +, present; −, negative/absent; % +ve, per cent positive.

  • Alkaline phosphatase stain = increased number of endomysial capillaries with positive staining.

  • COX, cytochrome oxidase; MHC-I, major histocompatibility complex.