Novel frameshift mutation in the SACS gene causing spastic ataxia of charlevoix-saguenay in a consanguineous family from the Arabian Peninsula: A case report and review of literature

Table 2 Needle electromyography of proband II-3

Items	Insertionalactivity	Spontaneous activity		Voluntary motor unit action potentials
		Fibrillation potentials	Fasciculation potentials	Amplitude	Duration	Polyphasia	Recruitment	Activation
LT MG	↑	+1	0	+2	+2	+2	↓↓	NL
LT TA	↑	+2	0	+2	+1	+2	↓↓	NL
LT VL	NL	0	0	NL	NL	NL	NL	NL
LT ADM	↑	+1	0	+1	+1	+2	↓↓↓	NL

↑ means increased; ↓↓ means moderately reduced; ↓↓↓ means severely reduced; NL: Normal; LT: Left; MG: Medial gastrocnemius; TA: Tibialis anterior; VL: Vastus lateralis; ADM: Abductor digiti minimi.