Table e-2. Visual evoked potentials (VEP) elicited by light emitting diode (LED) goggles or checkerboard pattern reversal (PR) in seven patients with GJA12-related PMLD (cohort A) and seven boys with PMD (cohort B)with age-matched normal values [mean ± SD]11

PatientAge [years]StimEyeVEP wave latencies [ms]

N1Dev [SD]P2Dev [SD]

PMLD patients

A1 4LEDLn. r.

LEDRn. r.

11LEDL140+11220

LEDR144+12226

[74 ± 6][105 ± 8]

A29LEDL104+5130

LEDR108+6132

[74 ± 6][105 ± 8]

13LEDL142+11178

LED R140+11168

[74 ± 6][105 ± 8]

A3 6LEDL122+8170

LEDR152+13172

[74 ± 6][105 ± 8]

A4 9LEDLn. r.

LEDRn. r.

A5 8PRL119151+11

PRR121154+12

[ ± ][105 ± 4]

A6 14LEDLn.r.

Rn.r.

A9 14PRB58125+7

[105 ± 4]

______

PMD patients

B22LEDL134+10174

R128+9168

[74 ± 6][105 ± 8]

3LEDB130+9182

[74 ± 6][105 ± 8]

5LEDL134+10186

R125+9188

[74 ± 6][105 ± 8]

B34LEDL120+8152

R108+6158

[74 ± 6][105 ± 8]

B43LEDB156+14226

[74 ± 6][105 ± 8]

5LEDL135+10220

R140+11220

[74 ± 6][105 ± 8]

B51LEDB144+7190

[80 ± 9][115 ± 10]

5LEDB202+21254

[74 ± 6][105 ± 8]

B6 6LEDL118+7220

R124+8220

[74 ± 6][105 ± 8]

B74LEDL156+14216

R166+15236

[74 ± 6][105 ± 8]

B81LEDL98+2148

R100+2147

[80 ± 9][115 ± 10]

------

Stim = stimulation technique for acquisition of VEP; Dev = deviation from normal mean; L = left eye; R = right eye; B = both eyes; n. r. = not recordable

Normative data for LED stimulation as provided in reference #11; for PR stimulation from own data.