Table 1. Clinical characteristics of the patients.

Case number	Sex/age	Age at onset						Serum CK level (25–170 IU/l)	nEMG
		Toe dorsiflexion difficulty, foot drop	Leg weakness	Limb-girdle weakness	Finger extensor weakness	Neck flexion weakness	Scoliosis
II-2	F/80y, died	NA	NA	NA	NA	NA	NA	NA	NA
III-2	F/68y, died	NA	NA	NA	NA	NA	NA	NA	NA
III-5	F/64y, died	Before 10y	12y	40y	45y	50y	14y	ND	ND
III-7	F/57y	7y	12y	36y	45y	52y	14y	ND	ND
III-9	F/53y	8y	11y	30y	45y	40y	NM	ND	ND
IV-3	M/46y	8y	12y	32y	NM	42y	20y	ND	ND
IV-5	M/40y	6y	14y	30y	NM	NM	NM	ND	ND
IV-12	F/40y	7y	11y	30y	NM	35y	NM	171	Myogenic change
IV-14	M/37y	6y	10y	35y	25y	30y	14y	ND	ND
IV-15	M/35y	6y	7y	35y	NM	NM	NM	ND	ND
IV-18	M/30y	7y	11y	25y	NM	NM	NM	ND	ND
IV-19	F/28y	6y	12y	24y	8y	24y	12y	ND	ND
V-4	M/14y	3y	5y	14y	NM	NM	11y	134	Myogenic change
V-6	F/12y	7y	11y	NM	NM	NM	NM	86	ND

Abbreviations: CK, creatine kinase; F, female; M, male; NA, not available; ND, not done; nEMG, needle electromyogram; NM, not manifested; y, year.