Muscle Histopathologic Scoring

Background: The following is a simplified pathologic grading score of FSHD muscle biopsies collected for research studies. The main objective of this scoring system is not a detailed assessment of the pathologic changes seen in FSHD but an overall pathological severity scoring to be used to correlate with clinical features (muscle strength or mass) or results of molecular studies derived from that same muscle. The scoring is based on H&E and Trichrome staining and scores the muscle based on 4 features: 1. variability in fiber size, 2. extent of central nucleation, 3. necrosis and regeneration, 4. interstitial fibrosis. For each feature, the severity of the abnormality is grade from 0-3 (0 normal, 1 mild, 2 moderate, 3 severe). The combined score range is from 0 (normal muscle) to 12 (end stage muscle).

Muscle biopsy histopathologic scoring (Download PDF file)

 

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