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Clinicopathologic Reports, Case Reports, and Small Case Series
June 2002

Unilateral Manifestation of Melanoma-Associated Retinopathy

Arch Ophthalmol. 2002;120(6):866-869. doi:

A rare complication of cutaneous melanoma is the development of melanoma-associated retinopathy (MAR), a condition characterized by the acute onset of night blindness, persistent pulsating photopsias, and constriction of the visual field.1 The pathogenetic mechanisms of the syndrome have been clarified by the detection of immunoglobulins directed against bipolar cells of the retina.2 The symptoms generally spread to the other eye within an interval of 4 days to 2 months.3,4 We report a case of MAR that remained unilateral during an observation period of 18 months.

Report of a Case

A cutaneous malignant melanoma was removed from the left leg of a 45-year-old woman in 1992. Because a systematic search revealed no metastasis, no further therapy was administered.

One evening in January 2000, she suddenly experienced difficulty seeing in dark conditions through her right eye, which greatly interfered with her stereovision. She also reported shimmering lights that fluctuated in brightness.

Because an ophthalmological examination demonstrated good visual acuity and no fundus alterations, the patient's complaints were judged to be of psychic origin. Four months later, a metastatic cutaneous melanoma was removed from her left leg. She underwent chemotherapy and was referred to our electrophysiological laboratory because of her persisting visual complaints.

She still had a best-corrected visual acuity of 20/20 OU. Although an ophthalmoscopy consistently showed no alterations in the right eye, a previously unknown uveal nevus was detected in the macular area of the left eye. Computerized perimetry (Octopus) revealed a marked visual field constriction and a general depression of light sensitivity in the right eye. The left eye exhibited no perimetric alterations (Figure 1).

Figure 1
 A, Visual field of the right eye
(left) and left eye (right) estimated using Octopus equipment. The x- and
y-axes represent dimensions of the visual field in degrees. B, Cumulative
defect curves (Bebie curve) from the 59 central points in rank order cumulative
sensitivity distribution.

A, Visual field of the right eye (left) and left eye (right) estimated using Octopus equipment. The x- and y-axes represent dimensions of the visual field in degrees. B, Cumulative defect curves (Bebie curve) from the 59 central points in rank order cumulative sensitivity distribution.

Full-field electroretinograms (ERGs) were recorded with gold-foil electrodes in both eyes after maximal pupil dilatation and a 30-minute period of dark adaptation. A stroboscopic lamp placed behind the patient provided flashing lights on a Ganzfeld screen. Scotopic, maximal, and photopic responses were recorded according to the International Society for Clinical Electrophysiology of Vision recommendations. The single-flash, rod-dominant ERG showed a markedly decreased scotopic response in the right eye, whereas maximal-intensity stimulation elicited a typical ERG that showed no abnormalities, a selective b-wave depression with a preserved a wave (Figure 2). Both the scotopic rod-isolated response and the maximal response in the left eye were in the normal range. Fifty individual ERG and oscillatory potential responses to blue light flashes were averaged. The averaged ERG and oscillatory potentials were greatly impaired in the right eye but normal in the left eye.

Figure 2
 Electrophysiological recordings
from the right eye (left) and left eye (right) of the patient. A, Scotopic,
rod-isolated response. B, Maximal, dark-adapted response. C, Electroretinographic
(ERG) response to red flash. D, Averaged ERG response to 50 blue flashes.
E, Averaged oscillatory potentials to 50 blue flashes. Calibrations: 50 µV;
100 milliseconds.

Electrophysiological recordings from the right eye (left) and left eye (right) of the patient. A, Scotopic, rod-isolated response. B, Maximal, dark-adapted response. C, Electroretinographic (ERG) response to red flash. D, Averaged ERG response to 50 blue flashes. E, Averaged oscillatory potentials to 50 blue flashes. Calibrations: 50 µV; 100 milliseconds.

In the immunocytochemistry test, the patient's serum displayed strong binding to retinal bipolar cells, similar to that of a known patient with MAR.

Five control electrophysiological examinations together with perimetry and ophthalmoscopy demonstrated no changes during the following 15-month observation period. Unfortunately, the patient developed agranulocytosis in July 2001 and died of pulmonary metastases and respiratory failure 1 month later. The family did not allow an autopsy.

Comment

In our case, the acute night blindness, sensations of shimmering lights, normal ERG results, and constricted visual field, together with the normal appearance of the retina and the preserved visual acuity, were diagnostic of MAR. The positive immunological reaction of the serum substantiated the diagnosis. The case is peculiar because the condition failed to spread to the other eye during an 18-month observation period. Until recently, all reported MAR cases were bilateral with some interocular latency difference in the appearance of symptoms.3,4 Although cases with unilateral normal-appearing ERGs have been reported, no evidence of melanoma-related immune activity was found.5 In our case, some damage to the other eye might have remained undetected by the electrophysiological tests, or the tragic outcome may have prevented a late manifestation of the disease in that eye. Nonetheless, the relatively long observation period raises the possibility that MAR manifests unilaterally in some patients.

This work was supported by ETT/Hungary grants 56411 and 57404.

We thank Ann Milam, PhD (University of Pennsylvania, Philadelphia), for performing the immunochemistry test.

Corresponding author and reprints: György Benedek, Department of Physiology, Faculty of Medicine, University of Szeged, H-6720 Szeged, POB 427, Hungary (e-mail: benedek@phys.szote.u-szeged.hu).

References
1.
Berson  ELLessell  S Paraneoplastic night blindness with malignant melanoma. Am J Ophthalmol. 1988;106307- 311Article
2.
Milam  AHSaari  JCJacobson  SG  et al.  Autoantibodies against retinal bipolar cells in cutaneous melanoma-associated retinopathy. Invest Ophthalmol Vis Sci. 1993;3491- 100
3.
Kellner  UBornfeld  NFoerster  MH Severe course of cutaneous melanoma associated paraneoplastic retinopathy. Br J Ophthalmol. 1995;79746- 752Article
4.
Boeck  KHofmann  SKlopfer  M  et al.  Melanoma-associated paraneoplastic retinopathy: case report and review of the literature. Br J Dermatol. 1997;137457- 460Article
5.
Fishman  GAAlexander  KRMilam  AH  et al.  Acquired unilateral night blindness associated with a negative electroretinogram waveform. Ophthalmology. 1996;10396- 104Article
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