Table 3.

Myopathological findings

		Muscle	Neurogenic fiber type grouping	Fiber type predominance	Fiber size variation	Fibrosis and fatty degeneration	Increase of internal nuclei	Fiber splitting	Necrosis	Lymphocyte infiltrates	Fiber regeneration	Rimmed vacuoles	Cytoplasmic myofibrillar inclusions	Core-like lesions	Rubbed-out fibers	Moth-eaten	Lobulated fibers	Ragged red fibers	COX– fibers
FF1
	III-6	VL	–	+		++	+	+	–	–	–	+	+	+	–	++	+	–	–
	III-8	S	(+)	–	++	++	++	++		–	–	+	++	++	–	–	–	–	–
	III-12	VL	–	+	(+)	+	–	–	–	–	+	(+)	+	–	–	–		–	–
		GLU	–	+	(+)	+	–	+	–	+	+	+	++	+	–	–		–	–
		VL	–		(+)	+	+	–	+	+	+	+	++	–	–	+	–	–	–
	IV-7	GM	–	–	+	+	+	+	+	+	–	+	+	+++	–	–	–	–	–
		GM	–	–	++	++	++	++	–	+	+	++	++	+	–	+	–	–	–
	IV-8	GM	–	–	+	–	+	+	+	+	+	+	+	+	–	+	+	–	–
	IV-9	VL	–	+	+	–	+	+	+	+	+	+	+	–	–	+	–	–	–
		VL	+	–	++	+	+	–	–	–	–	+	+	–	–	–	(+)	–	(+)
	IV-10	VL	–	–	–	–	–	–	–	–	–	+	(+)	+	–	–	–	–	–
		VL	–	+	+	–	+	–	–	–	–	+	+	+	–	–	–	–	–
FF2
	III-4	VL	–		+	–	+	–	–	–	–	+	+	–	–	–	–	–	(+)
	III-5	VL	–		+	–	+	–	–	–	–	+	++	–	–	–	–	–	(+)
	III-7	VL	–		++	++	+++	+	+	+	+	+	+	+	–	+	–	–	–
FF3
	IV-1	VL	+	–	++	+	+++	+	+	+	–	+	++	+	–	+	–	–	–
		GL	–	–	++	+	++	+	++	+	–	+	++	++	–	+	+	–	(+)
FF4
	III-2	VL	–	+	++	++	++	+	+	–	–	+	+	+	–	+	–	–	–
	IV-1	VL	–	–	(+)	–	+	–	+	–	+	+	+	++	–	+	–	–	–
FF5
	IV-1	VL	–	+	++	++	++	+	+	+	–	++	+	+	–	–	+++	–	–
FF6
	IV-1	VL	+	–	++	++	+	–	–	–	–	+	+	–	–	+	+	–	(+)

Internal nuclei amount assessed as: –, absent; (+), existent in < 1 %; +, existent up to 5 %; ++, existent in 6–20 %; +++, existent in over 20 % of the fibers. Fibrosis and fatty degeneration: –, no fibrosis, fatty degeneration; +, mildly increased; ++, moderately increased