Table 4 Characteristics of paraneoplastic syndromes
| Â | MAR | CAR | pAEPVM |
|---|---|---|---|
Autoantibodies against | Bipolar cells | Photoreceptors | RPE cells |
Associated tumor | Melanoma | SCLC | Mucosal melanoma |
Antibody testing | TRPM1, recoverin, α-enolase, CA II | Recoverin, α -enolase, CA II | Bestrophin, usually not performed |
Symptoms | Very symptomatic: progressive painless visual loss, photopsias, nyctalopia, shimmering, visual field defects, light sensitivity in case of inflammation | Few symptoms: blurred vision, metamorphopsia, nyctalopia, and photopsias | |
Retinal signs | Initially normal or subtle: retinal vessel attenuation, optic disc pallor, vitreous cells, pigmentary changes | More prominent: multifocal serous retinal detachments and subretinal deposits with a vitelliform appearance in the posterior pole | |
OCT | Non-specific | Loss of the outer retinal layers with foveal sparing | Subretinal fluid and deposits of hyperreflective material |
FA | Non-specific | Non-specific, sometimes retinal vasculitis | Blockage at the vitelliform lesion without retinal or papillary leakage |
FAF | Non-specific | Hyperautofluorescence around a hypoautofluorescent zone | Hyperautofluorescence corresponding to the deposits |
VF | (para)central scotoma, peripheral constriction | Non-specific | |
ERG | Electronegative | Extinguished rod and cone responses, mainly affecting the rods | Normal |
Treatment | - Corticosteroids in case of inflammation - Interruption of ICI? - Tumor control - Rituximab/IVIG/plasmapheresis/efgartigimod? | - Corticosteroids in case of inflammation - Tumor control - Wait and see | |
Prognosis | Poor | Poor | Usually good |