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.
Theroux
Mary C
MC
Department of Anesthesiology and Critical Care, Alfred I. duPont Hospital for Children, Wilmington, DE 19899, USA. mtheroux@nemours.org
Olivant
Alicia
A
Akins
Robert E
RE
D000110:Acetylcholinesterase / Q000032:analysis; D000293:Adolescent; D001706:Biopsy; D002648:Child; D006801:Humans; D020388:Muscular Dystrophy, Duchenne / Q000378:metabolism / Q000473:pathology*; D009469:Neuromuscular Junction / Q000737:chemistry / Q000473:pathology*; D011950:Receptors, Cholinergic / Q000032:analysis*; D013194:Staining and Labeling / Q000379:methods
D011950:Receptors, Cholinergic; D000110:Acetylcholinesterase