9.11  Electrophysiology

The rationale of electrophysiology testing is to provide objective evidence of function at the different levels of the visual system, thus enabling accurate localisation and characterisation of dysfunction in the vast majority of patients. The three main arms of electrophysiological testing are the:

1. Electroretinogram (ERG)
   
   1. Full field ERG (mass response from photoreceptors and INL)
   2. Pattern ERG (central retinal ganglion cells (RGC))
   3. Multifocal ERG (macular cones)
2. Electro-oculogram (EOG): RPE
3. Visual evoked potential (VEP): Cortical Standardisation of testing is provided by The International Society for Clinical Electrophysiology of Vision (ISCEV).

There are four types of waveforms:

1. a-wave - negative wave which represents photoreceptor activity (rods > cones)
2. b-wave - positive wave which is produced by inner retinal activity (ON-bipolar cells, Muller cells)
3. oscillatory potentials – are superimposed on the b wave. They are produced by inner plexiform layer activity (amacrine cells)
4. c-wave - slow response that originates from the RPE

• Stimulation of the entire retina with a source of light under varying conditions of retinal adaptation
• Provides a mass response of the retina and is normal in localised macular disease
• No significant retinal ganglion cell (RGC) contribution
• Amplitude reflects quantity of functioning retina
• Implicit time (latency) reflects quality of functioning retina
• General photoreceptor dysfunction will cause decreased amplitude and increased implicit time.
• Focal photoreceptor dysfunction will cause a decrease in amplitude but normal implicit time.
• The full field ERG is performed in scotopic and photopic conditions
  • Scotopic (dark adapted) ERG
    • Rod driven response (DA 0.01) - Low intensity stimulus
    • Maximal combined response (DA 10.0) - High intensity stimulus
  • Photopic (light adapted) ERG
    • Single flash (LA 3.0) – cone location sensitive
    • Flicker ERG (LA 30Hz) – cone specific (but generated at the inner retinal level). Delayed in birdshot chorioretinopathy
• The main disadvantage of the full field ERG is that it does not localise the sites of disease activity and provide information on the retinotopic distribution of disease loss

• a-wave spared but selective b-wave reduction
• Due to dysfunction post-phototransduction

1. CRAO
2. CSNB
3. X-linked retinochisis
4. Quinine toxicity
5. Melanoma associated retinopathy
6. Batten disease
7. Birdshot chorioretinopathy
8. Cone-rod dystrophy (occasionally)

• Provides a measure of central retinal ganglion cell function by using a reverse flickering checkboard stimulus under photopic conditions
• Helps differentiate between macular and optic nerve dysfunction
• The waveforms assessed are:
  • N35 – assess photoreceptor function
  • P50 – reflects activity distal to the ganglion cells. Typically normal in optic neuropathies
  • N95 – generated by ganglion cells. Typically abnormal in optic neuropathies
• An abnormal PERG is seen in cone dystrophies/Stargardts disease. A normal PER suggests that there is central sparing

• A method of recording macula response spatially by comparing local cone function from different areas of central retina in photopic conditions
• Largely still research based but has clinical value in conditions such as the cone dystrophies, Stargardt disease, retinitis pigmentosa and acute zonal occult outer retinopathy (AZOOR)