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Retinitis Pigmentosa — SCE Neurology MCQ

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EasyNeuro-ophthalmologyRetinitis PigmentosaSCE Neurology

A 60-year-old man presents with progressive bilateral visual field constriction over 3 years. Goldman perimetry shows bilateral ring scotomata. Fundoscopy shows bilateral optic disc pallor with retinal pigmentary changes (bone spicule pattern). ERG shows severely reduced rod and cone responses. What is the diagnosis?

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Correct answer: B — Retinitis pigmentosa

Retinitis pigmentosa (RP) is an inherited retinal dystrophy characterised by progressive rod photoreceptor degeneration followed by cone degeneration. Classic features include: nyctalopia (night blindness — earliest symptom), progressive peripheral visual field loss (ring scotoma), bone spicule retinal pigmentation, attenuated retinal arterioles, waxy disc pallor, and severely reduced ERG responses (both scotopic and photopic). RP can be inherited in autosomal dominant, autosomal recessive, or X-linked patterns. A: Glaucoma causes arcuate field defects, not ring scotomata, and has different fundoscopic findings. C: Vigabatrin causes concentric constriction without bone spicule pigmentation. D: Optic neuritis is acute with central scotoma. E: Papilloedema causes enlarged blind spots.

Reference: ABN Neuro-ophthalmology Guidelines