- characterised by triad of
- night blindness (loss of rod function)
- tunnel vision (rod dysfunction)
- pigmented 'bony spicules'
Important systemic associations
- Abetalipoproteinemia (Bassen Kornzweig syndrome)
- Refsum's disease
- Usher syndrome
- Laurence Moon Biedl syndrome
- Kearns Sayre syndrome
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