Rod-sparing paraneoplastic retinopathy, opsoclonus, and peripheral neuropathy due to small cell lung carcinoma

Abstract

A 45-year-old female developed a sensorimotor polyneuropathy. Later, visual acuity, color vision, and day vision deteriorated progressively. Macular pigment epithelial mottling led to an electroretinogram, which revealed a relatively rod-sparing photoreceptor degeneration. A paraneoplastic syndrome was suspected and confirmed by bronchoscopy, disclosing a small cell lung carcinoma, and by positive CAR antibody titers. Subsequently, opsoclonus appeared. Although frequently patients with neurological paraneoplastic syndromes may have mixtures of the different syndromes, this is, to our knowledge, the first report of an association of CAR with paraneoplastic opsoclonus and peripheral neuropathy. We confirm a previous report by Cogan that paraneoplastic retinopathy may spare the rods and clinically present with central scotoma, dyschromaropsia, and hemeralo-pia.

Key Words:

• Paraneoplasia
• cancer-associated retinopathy
• cone dystrophy
• opsoclonus
• peripheral neuropathy
• small cell lung carcinoma