In Reply.Ìý —I would like to thank Nelson and Davis for their bringing to our attention a recently published article regarding low carnitine palmityltransferase enzyme activity and proximal muscle weakness.¹ In that case, the muscle biopsy specimen showed ragged red fibers and paracrystalline inclusions by electron microscopy. In both patients in our article,² ragged red fibers were not seen on light microscopy, and electron microscopy studies did not show paracrystalline inclusions in the mitochondria.