Table 1

Characteristics of published familial cases of IBM

Reference	Clinical features				CK	EMG	Histology				MRI: atrophy +fatty degeneration	Treatment
Reference	Age at onset	Sex	Atrophy	Weakness	CK	EMG	Rimmed vacuoles	Mononuclear inflammation	Congo red staining	Tubulofilament 18–21 nm	MRI: atrophy +fatty degeneration	Treatment
*Hand and finger extensors more severely affected than flexors.
CK, creatine kinase; EMG, electromyography; MRI, magnetic resonance imaging; Q, quadriceps; TA, tibialis anterior; P, proximal; D, distal; d, dysphagia; My, myopathic; NA, not available; C, corticosteroids; VF, volar forearm; S, sensitive anomalies; TF, thigh flexors.
Nauman⁸	55	F	Q, TA	P D* d	6N	My	+	+	NA	+	Q, left TA	C
Nauman⁸	48	F	Q, TA	P D* d	4N	My	+	+	NA	+	NA	C
Sivakumar⁷	73	M	Q, VF	P D S	1N	NA	+	+	+	+	NA	NA
Family A	69	M	Q > VF	P D	1.5N	NA	+	+	+	+	NA	NA
Family B	55	M	Q < VF	P D	2N	NA	+	+	+	+	NA	NA
Family B	55	M	Q > VF	P D	3N	NA	+	+	+	+	NA	NA
Family C	60	M	Q > VF	P D d	1N	NA	+	+	+	+	Q > TF	NA
	60	M	Q > VF	P D	1.5N	NA	+	+	+	+	Q + TF	NA
	48	F	Q, VF	P D	1N	NA	NA	NA	NA	NA	Q > TF	NA
Amato⁶	62	M	Q, VF	P D d	2N	My	+	+	+	+	NA	NA
Amato⁶	60	M	Q, VF	P D S	NA	My	+	+	+	NA	NA	NA
Personal report	64	F	Q, VF	P D d	2N	My	+	+	+	NA	Q > TF	C + IgIV
Personal report	65	M	Q > VF	P D d	1N	My	+	+	+	NA	NA	C + IgIV