Table 1.

Characteristics of Achromatopsia and Blue Cone Monochromatism Patients

Patient No	Sex	Age at ERG (yr)	Spherical Equivalent (D)	Visual Acuity (most recent)	Photophobia	Nystagmus	PPR*
ACHR
1	F	1.0	+3.75‡	20/190 §	Yes	Yes	Yes
2	F	1.1	+5.50‡	20/960 §	No	Yes	Yes
3	F	1.2	+6.50‡	20/250	Yes	Yes	No
4	M	1.5†	+7.75‡	20/500	Yes	Yes	No
5	M	1.6	+0.25	20/125	Yes	Yes	Yes
6	F	2.3	+4.00‡	20/133§	Yes	Yes	Yes
7	M	2.3	+8.50‡	20/180§	Yes	Yes	No
8	M	2.7	+2.00	20/400	Yes	Yes	Yes
9	F	3.0	+4.25‡	20/250	Yes	Yes	Yes
10	F	3.9	+8.00‡	20/300	Yes	Yes	Yes
11	F	4.9	−3.00‡	20/320	Yes	Yes	Yes
12	M	6.2†	+3.75‡	20/200	No	Yes	No
13	M	7.0	+1.00	20/222	Yes	Yes	Yes
14	M	7.7†	−4.75‡	20/200	Yes	Yes	No
15	F	20.2†	−1.50	20/125	Yes	Yes	Yes
BCM
16	M	4.0	−2.88‡	20/160	Mild	Yes	Yes
17	M	6.8	−1.75‡	20/209	No	Yes	Yes
18	M	7.7	−6.13‡	20/400	Yes	Yes	Yes
19	M	8.3†	−1.63	20/160	Mild	Yes	No
20	M	9.4†	−0.50	20/80	Mild	Yes	Yes
21	M	22.1†	−10.25‡	20/130	Mild	Yes	No

^*PPR: paradoxical pupillary response

^†tested awake

^‡outside 99% prediction limit for normal⁴¹, ⁴²

^§grating acuity (Teller Acuity Cards)⁹⁷