Table 2. Clinical features of patients with the SNRNP200 mutation.

	II:1		III:3		III:9		IV:6		IV:7
	OD	OS	OD	OS	OD	OS	OD	OS	OD	OS
Age (years)/Sex	83/F		49/M		49/F		27/F		19/M
Age of onset of night blindness (years)	10		12		10		14		12
BCVA (logMAR)	NL	NL	0.1	0.2	0.2	NL	1.0	1.0	1.0	1.0
IOP(mmHg)	58	60	13	15	34	35	14	15	14	15
PSC	Yes	Yes	Yes	Yes	Yes	Yes	No	No	No	No
Optic disc	NA	NA	Pale	Pale	Pale	Pale	Cerinus	Cerinus	Cerinus	Cerinus
Artery attenuation	NA	NA	Yes	Yes	Yes	Yes	Yes	Yes	No	No
Pigment deposits	NA	NA	Int	Int	Int	Int	Diff	Diff	Diff	Diff
Visual field	NA	NA	30°	30°	10°	NA	60°	60°	30°	30°
ERG	NA	NA	NR	NR	NR	NR	Both remarkably reduced response		Both remarkably reduced response

F: female; M: male; BCVA: best corrected visual acuity; logMAR: logarithm of the Minimum Angle of Resolution; NL: no light perception; IOP: intraocular pressure; PSC: posterior subcapsular cataract; ERG: electroretinography; NR: not recordable; Int: intensive Diff: diffuse; NA: not available.