Case Report

Clinical and Pathological Features of Childhood-Onset Nemaline Myopathy: A Report of Four Cases

Figure 1

(a) HE staining revealed inequality of size of muscle fibers and a lot of emarcid muscle fibers and compensatory hypertrophic muscle fibers. Some dark red uniform of eosinophils matters was observed among some of the muscle fibers. Magnification 400. (b) Modified Gomori trichrome (MGT) staining showed dark blue rod-like structures or nemaline rods, and abnormal red fiber was not found. Magnification 400. (c) Histochemistry of ATP enzyme staining demonstrated abnormal distribution of type I (predominated) and type II (rare) muscle fibers. Magnification 400. (d) Lead-uranium double staining demonstrated myofibril disorderly arrayed, and a large number of nemaline body under the sarcolemma and between the myofibril, and electron dense nemaline bodies of Z-band origin on ultrastructural examination. Magnification 12000. (e), (f) Immunohistochemical results of the two specimens with necrotic muscle fibers showed that CD4 positive cells and some other cells were gathered among the muscle fibers (e), but CD8 positive cells were not found (f). Magnification 400.
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