Electrophysiology reports look like a page of squiggles and numbers. The trick is to remember that each test listens to one level of the visual pathway. Ask which level is abnormal and the diagnosis usually follows.
Four rules
1. One test, one levelEOG = RPE. Full-field ERG = the whole retina (rods and cones). Pattern ERG = macula (P50) and ganglion cells (N95). Multifocal ERG = a map of central cone function. VEP = the whole path to the visual cortex, dominated by the macula.
2. a-wave = photoreceptors, b-wave = the next cellThe negative a-wave comes from photoreceptors; the positive b-wave mainly from ON-bipolar and Müller cells. If the a-wave is normal but the b-wave small (negative ERG), the problem is after the photoreceptors.
3. Separate rods and cones with light and speedIn the dark with a dim flash only rods respond. Background light saturates rods, so light-adapted responses are cones. Rods cannot follow a 30 Hz flicker, so flicker is pure cone.
4. The full-field ERG misses small lesionsA normal full-field ERG does not rule out macular disease: the macula is only a small part of the retina. Use pattern or multifocal ERG for the centre.
Which test listens to which level
The ISCEV full-field ERG, step by step
Step
Condition
What responds
What it tells you
DA 0.01
Dark-adapted, dim flash
Rods only (b-wave, no a-wave)
Rod pathway
DA 3.0 (and DA 10)
Dark-adapted, bright flash
Rods + cones
a-wave = photoreceptors; b-wave = inner retina. Look at b/a ratio
DA oscillatory potentials
Filtered from the bright flash
Amacrine cells (inner retina)
Reduced in diabetic retinopathy, ischaemia
LA 3.0
Light-adapted, flash
Cones
Cone system
LA 30 Hz flicker
Light-adapted, flicker
Cones only
Most sensitive cone test; delayed in early disease
A negative ERG keeps the a-wave but loses most of the b-wave.
Causes of a negative ERG
X-linked retinoschisis (with foveal schisis in a boy)
Congenital stationary night blindness: complete (ON-bipolar defect, e.g. NYX) and incomplete (CACNA1F)
Central retinal artery occlusion or ischaemic CRVO: inner retina dies, photoreceptors (choroid-fed) survive
Melanoma-associated retinopathy (antibodies against ON-bipolar cells)
Quinine toxicity, juvenile Batten disease, some birdshot chorioretinopathy