Table 2.

Nerve conduction studies (NCS) and electromyography (EMG) in the proband from family 1 (II:1) and family 1A (II:2) reported here as compared with other probands (F2, F4-6, F8, F16 and F26) from different SPG11 families (Table 1)

Case	Motor nerve conduction studies									Sensory nerve conduction studies						EMG		Summary findings of NCS and EMG
	Median nerve			Ulnar nerve			Tibial nerve			Median nerve		Ulnar nerve		Sural nerve		Limbs. Upper: lower
	DML (msec)	CMAP amp (uV)	MCV (m/s)	Distal lat (msec)	CMAP amp (uV)	MCV (m/s)	Distal lat (msec)	CMAP amp (uV)	MCV (m/s)	SNAP amp (uV)	SCV (m/s)	SNAP amp (uV)	SCV (m/s)	SNAP amp (uV)	SCV (m/s)	Chronic denervation
SPG 11 patients
F1 II:1	3.3	2.7	48	2.8	2.4	63	5.1	0.3	N/A	12	68	6	60	NR	NR	Yes	Yes	Severe sensory-motor axonal neuropathy
F1A II:2	4.4	0.4	41	N/A	NR	N/A	4.7	0.1	36	17	50	6	51	NR	NR	Yes	Yes	Severe sensory-motor axonal neuropathy
F2	3.3	10.7	55	2.4	11.9	58	4	3.9	53	25	62.5	23	59	14	41.5	No	No	Normal NCS/EMG
F4	3.7	0.9	40	4.1	3.7	42	5.5	3.6	43	N/A	N/A	N/A	N/A	11.6	35	Mod	Mod	Mild axonal neuropathy
F5	4	4.9	51	2.4	10	61	3.4	5.2	48	28	61	13	67	11	55	No	No	Normal NCS/EMG
F6	3.2	8.3	56	2.3	8.9	62	2.8	3.7	45	23	65	15	64	12	48	N/A	No	Normal NCS/EMG
F8	3.9	7.3	64	N/A	N/A	N/A	4.8	5.3	45	19	61	9	50	6	55	No	Mild	Mild axonal neuropathy
F16	4.1	6.6	52	N/A	N/A	N/A	5	3.4	42.5	15	57.5	14	53.5	14	41.5	No	No	Normal NCS/EMG
F26	3.6	6.1	53	3.8	3.8	43	5.9	3.3	35	28	54	19	56	9	40	Mod	Mod	Mild axonal neuropathy

NR no response, Amp amplitude, lat latency, DML distal motor latency, CMAP compound muscle action potential, mod moderate, μV microvolts, m/s meters per second, msec milliseconds