Details of Retinal Tests Available

These test permit discovery of retinal function, dissecting the retina layer by layer, cell by Cell

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Examples of Disorders of Pigment epithelium

 

Example of Disorders of Pigment Epithelium

A number of conditions affect Receptor cells as well as the Pigment Epithelium.Included amongst these are:

  • Retinitis Pigmentosa
  • Cone dystrophy
  • Choroideraemia 

These will be discussed in disorders of the Receptor cells.

  • Vitelliform Macular Dystrophy (Best's Disease) affects Pigment Epithelium specifically without involving other retinal layers.

Retinal Pigment Epithelium Disorder

Mother and son

Right Egg yolk -vitelliform Left "Scrambled"
Multifocal ERG

Reduced central responses normal peripheral amplitudes and waveforms

Summary of Best's Dystrophy

 

 The most common disorder primarily of the Rods and Pigment Epithelium is Retinitis Pigmentosa

 

Examples of Disorders of Receptor Cells       (A) Rods

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Disorders of Receptor Cells (A) Rods

 

Examples of Disorders of Receptor Cells       (B) Cones

Specific conditions affecting Cone cells are Cone Dystrophy and Congenital Achromatopsia (Rod monochromatism)

Achromatopsia (Rod monochromatism)

Absence of cone cells. Causes nystagmus and reduced acuity. Day blindness and a preference for dim lights. ERG confirms -Normal Scotopic, absent photopic.

Cone dystrophies    A recessive condition, prefer dim lights and causes day blindness (opposite of RP), onset at 10 –15 years and worsens with aging can only be diagnosed by ERG.

Cone /rod dystrophies    Variable reduction of scotopic together with loss of Photopic responses.

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Disorders of Receptor Cells (B) Cones

1. Cone Dystrophy 2. Achromatopsia


Examples of Disorders of Mid retinal layers  ::

Disorders of Mid Retinal Layers

Result in loss of B wave (origin Muller Cells). This produces a negative ERG where A wave>B wave

A negative ERG

A normal ER ompared with a negative ERG

Congenital Stationary Night Blindness

May b associated with Myopia, Nystagmus and reduced vision

Melanoma Associated Retinopathy

Note a typical history and a negative ERG

Auto Immune Retinopathy

Normal examination but reduced Acuity

Auto Immune Retinopathy

Normal Fundi and Fluorescein Angiography

Auto Immune Retinopathy

Normal OCT

Auto Immune Retinopathy

A Negative ERG

Multifocal ERG

Poor waveform and reduced amplitude all sectors. Response poor to therapy

Examples of Disorders of Inner Retinal Layers  ::    

Examples of Disorders of Visual Pathwys ::

Disorders of Optic Nerve Function

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Invesigation of Congenital Nystagmus  3 boys Visual Electrophysiology::