Table 1.

Clinical Characteristics of Affected Individuals of Family PKRP070 Diagnosed with Autosomal-Recessive CSNB

		Visual Acuity
Individual ID	First Symptoms	OD	OS	Disease Progression	Fundus Findings	Electroretinographic Characteristics
14	night blindness since early childhood	6/6	6/6	stationary	no macular atrophy, no pigment deposition, and no vascular attenuation	a- and b-waves are absent under the scotopic condition, whereas the cone responses are somewhat reduced under the photopic condition
18	night blindness since early childhood	6/6	6/24	stationary	no macular atrophy, no pigment deposition, and no vascular attenuation	a- and b-waves are absent under the scotopic condition, whereas the cone responses are somewhat reduced under the photopic condition
28	night blindness since early childhood	6/18	6/9	stationary	no macular atrophy, no pigment deposition, and no vascular attenuation	a- and b-waves are absent under the scotopic condition with normal cone responses under the photopic condition
34	night blindness since early childhood	6/6	6/6	stationary	no macular atrophy, no pigment deposition, and no vascular attenuation	a- and b-waves are absent under the scotopic condition with normal cone responses under the photopic condition

OD, oculus dexter (right eye); OS, oculus sinister (left eye).