Clinical and Electrophysiologic Characterization of Paraneoplastic and Autoimmune Retinopathies Associated With Antienolase Antibodies

Visual field response of antirecoverin CAR patient to corticosteroid therapy. Goldmann perimetric visual fields of patient before (top) and 6 weeks after (bottom) treatment with oral corticosteroids demonstrate significant reduction of the dense ring scotomas in each eye. The very small isopter present centrally for the right eye in top figure is using the I4e test target. Note that this isopter was much larger after treatment with corticosteroids.

Expanded International Society for Clinical Electrophysiology of Vision protocol full-field electroretinograms (ERGs) of patient with antirecoverin cancer-associated retinopathy (CAR) at time of recognition of her autoimmune retinopathy and 7 months after corticosteroid therapy compared with an age-matched normal subject. In this and subsequent full-field ERGs, the responses from the right and left eye are superimposed. Note that cone and rod ERG responses for this patient are equally subnormal when compared with a normal subject. Oscillatory potentials (OPs) are also severely abnormal. Comparison of patient’s ERG tracings before and 7 months after treatment with oral corticosteroids demonstrates a notable increase in amplitudes of rod responses, dark-adapted cone responses to red flash (arrows), light-adapted cone responses to single flash and 30-Hz flicker, and, to a lesser degree, scotopic bright white flash and the scotopic OPs. The rod and cone implicit times, including 30-Hz flicker (horizontal arrow designates peak timing), remained mildly prolonged.

Expanded International Society for Clinical Electrophysiology of Vision protocol full-field electroretinograms (ERGs) for cases 1, 2, and 3, compared with a similar-aged subject with normal ERG. The ERG responses are normal for all protocol steps for case 1. For case 2, rod responses are normal and responses to scotopic bright white flash and the oscillatory potentials (OPs) are mildly and moderately subnormal, respectively. For case 3, rod and scotopic bright flash responses are mildly subnormal, but OPs are markedly subnormal. Cone single flash and 30-Hz flicker responses are moderately subnormal and, at most, mildly prolonged for case 2, and severely subnormal and prolonged (horizontal arrow designates peak timing) for case 3.

The multifocal electroretinogram (mfERG) first-order tracing arrays (top) and ring averages (bottom) for the right eye for case 1, at age 74 years. The gray tracings represent those of a normal reference. For this and all subsequent mfERGs, the responses were averaged across 6 rings (ring 1 represents the central hexagon, 1 arc degree in diameter; ring 2, 1 to 6 arc degrees in diameter; ring 3, 6 to 12 arc degrees in diameter; ring 4, 12 to 21 arc degrees in diameter; ring 5, 21 to 31 arc degrees in diameter; and ring 6, 31 to 44 arc degrees in diameter). The peripheral mfERG responses are of normal amplitude, but the responses within the central 5 degrees from fixation are subnormal.

Multifocal electroretinogram first-order tracings, amplitude density plots, ring averages, and latencies for the left eye (top) and right eye (bottom) of case 2. Peripheral amplitude loss is evident for each eye, greater for the left eye, with prolonged implicit times peripherally for the left eye and pericentrally for the right eye.

Multifocal electroretinogram first-order tracings, amplitude density plots, ring averages, and latencies for the left eye (top) and right eye (bottom) of case 4. Central responses are severely subnormal bilaterally, more so for the left eye. For the left eye, the amplitude loss is more diffuse and extended peripherally, and the pericentral responses show prolonged latencies. For the right eye, peripheral responses are generally normal, but uneven, in amplitude. Note prolongation of latencies temporal to fixation for the right eye in the region associated with the relatively smaller amplitudes; this correlated well with the paracentral scotoma evident on Goldmann perimetry.