C Histomorphology of neuromuscular junction in Duchenne muscular dystrophy

BACKGROUND: Our objective was to better understand neuromuscular junction (NMJ) morphology in children with Duchenne muscular dystrophy (DMD). METHODS: We examined the NMJs of four children, age 6-13 years, who were diagnosed with DMD. Using our previously established staining technique, we examined the gross appearance of the NMJs in patients with DMD and evaluated the spread of acetylcholine receptors (AChRs) in relationship to the NMJs. We used a computerized algorithm to measure the area of staining corresponding to AChRs and NMJ. RESULTS: Abnormal shape and morphological appearance of some of the NMJs was clearly evident. The spread of AChRs in DMD patients is comparable with the spread of AChRs in nonDMD patients. CONCLUSIONS: The distribution of AChRs in relationship to the boundaries of NMJs in DMD children is similar to the distribution of NMJs in the erector spinae muscles of idiopathic scoliosis patients.