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Congenital Stationary Night Blindness
Main Features
- Night Blindness, variable vision loss, largely normal fundus
- May have nystagmus and strabismus
Distinguishing Features
- Night blindness
- Normal Appearing Fundus
- High Myopia
- may have tilted discs and nerve pallor
- ERG
- “Negative ERG”
- Photoreceptor derived a-wave with a bright flash is normal
- Reduction of the inner nuclear derived b-wave from inner retinal dysfunction
- No detectable Rod specific ERG- dark adapted dim flash
Differential Dx
- Idiopathic Nystagmus Syndrome
Pathogenesis
- Autosomal Dominant
- Normal or mildly reduced visual acuity
- Autosomal recessive
- Mild to moderate central vision loss
- Nystagmus and strabismus
- X-Linked
- Mild to moderate central vision loss
- Nystagmus , strabismus
Treatment
- Unordered List Item