Table 1.

Clinical Characteristics of Affected Individuals of Pedigree PKRP130 Diagnosed with Autosomal Recessive CSNB

Individual ID	First Symptoms	Visual Acuity		Color Vision	Fundus Findings	ERG Characteristics
		OD	OS
10	Night blindness since early childhood	6/6	6/6	Normal	No macular atrophy, no pigment deposition, and no vascular attenuation.	The a- and b-waves are absent in the scotopic condition, whereas the cone responses are somewhat reduced in the photopic condition.
11	Night blindness since early childhood	6/6	6/6	Normal	ND	ND
12	Night blindness since early childhood	6/6	6/6	Normal	No macular atrophy, no pigment deposition, and no vascular attenuation.	The a- and b-waves are absent in the scotopic condition with normal cone responses in the photopic condition.
14	Night blindness since early childhood	6/6	6/6	Normal	No macular atrophy, no pigment deposition, and no vascular attenuation.	The a- and b-waves are absent in the scotopic condition with normal cone responses in the photopic condition.

ND, not determined.