Abstract
Cancer-associated retinopathy (CAR) should be suspected in patients who present with visual symptoms such as rapid unexplained visual loss and seeing shimmering lights, with an abnormal ERG. Electronegative ERG responses are not exclusive to melanoma-associated retinopathy (MAR) but may be seen in CAR as well. We describe a patient with CAR who presented with an electronegative ERG. A 67-year old woman, who presented with complaints of seeing shimmering lights, underwent an extensive ophthalmological and electrophysiological examination. Best-corrected visual acuity was 7/10 in the right and 9/10 in the left eye. Goldmann visual fields showed relative central scotomata and concentric narrowing. Slit-lamp and fundus examination were normal as was fluorescein angiography. Rod-specific ERG responses were severely reduced, with electronegative maximal combined rod-cone responses and delayed cone-responses with normal amplitudes. Melanoma-associated retinopathy was suspected. Extensive dermatological and internal evaluation eventually revealed an oat-cell carcinoma in the right lung. The patient died of pneumonia 2 years after presentation.
Abbreviations
- CAR:
-
Cancer-associated retinopathy
- DBCs:
-
Depolarizing bipolar cells
- ERG:
-
Electroretinogram
- HBCs:
-
Hyperpolarizing bipolar cells
- MAR:
-
Melanoma-associated retinopathy
- PNS:
-
Paraneoplastic syndromes
References
Bataller L, Dalmau J (2004) Neuro-ophthalmology and paraneoplastic syndromes. Curr Opin Neurol 17:3–8
Ling CP, Pavesio C (2003) Paraneoplastic syndromes associated with visual loss. Curr Opin Ophthalmol 14:426–32
Chan JW (2003) Paraneoplastic retinopathies and optic neuropathies. Surv Ophthalmol 48:12–38
Robertson DM (2002) Non-cancerous ophthalmic clues to non-ocular cancer. Surv Ophthalmol 47:397–430
Sawyer RA, Selhorst JB, Zimmerman LE, Hoyt WF (1976) Blindness caused by photoreceptor degeneration as a remote effect of cancer. Am J Ophthalmol 81:606–613
Berson EL, Lessell S (1988) Paraneoplastic night blindness with malignant melanoma. Am J Ophthalmol 106:307–311
Hooks JJ, Tso MO, Detrick B (2001) Retinopathies associated with antiretinal antibodies. Clin Diagn Lab Immunol 8:853–858
Cohen RG, Rizzo J III, Lou P (1997) New developments in cancer-associated retinopathy. Int Ophthalmol Clin 37:233–250
De Potter P (1998) Ocular manifestations of cancer. Curr Opin Ophthalmol 9:100–104
Keltner JL, Thirkill CE, Yip PT (2001) Clinical and immunologic characteristics of melanoma-associated retinopathy syndrome: eleven new cases and a review of 51 previously published cases. J Neuroophthalmol 21:173–187
Alexander KR, Fishman GA, Peachey NS, Marchese AL, Tso MO (1992) ‘On’ response defect in paraneoplastic night blindness with cutaneous malignant melanoma. Invest Ophthalmol Vis Sci 33:477–483
Boeck K, Hofmann S, Klopfer M, Ian U, Schmidt T, Engst R, Thirkill CE, Ring J (1997) Melanoma-associated paraneoplastic retinopathy: case report and review of the literature. Br J Dermatol 137:457–460
Chan C, O’Day J (2001) Melanoma-associated retinopathy: does autoimmunity prolong survival? Clin Exp Ophthalmol 29:235–238
Milam AH, Saari JC, Jacobson SG, Lubinski WP, Feun LG, Alexander KR (1993) Autoantibodies against retinal bipolar cells in cutaneous melanoma-associated retinopathy. Invest Ophthalmol Vis Sci 34:91–100
Potter MJ, Adamus G, Szabo SM, Lee R, Mohaseb K, Behn D (2002) Autoantibodies to transducin in a patient with melanoma-associated retinopathy. Am J Ophthalmol 134:128–130
Solomon SD, Smith JH, O’Brien J (1999) Ocular manifestations of systemic malignancies. Curr Opin Ophthalmol 10:447–451
Scholl HP, Zrenner E (2000) Electrophysiology in the investigation of acquired retinal disorders. Surv Ophthalmol 45:29–47
Koh AH, Hogg CR, Holder GE (2001) The incidence of negative ERG in clinical practice. Doc Ophthalmol 102:19–30
Alexander KR, Barnes CS, Fishman GA, Milam AH (2002) Nature of the cone ON-pathway dysfunction in melanoma-associated retinopathy. Invest Ophthalmol Vis Sci 43:1189–1197
Lei B, Bush RA, Milam AH, Sieving PA (2000) Human melanoma-associated retinopathy (MAR) antibodies alter the retinal ON-response of the monkey ERG in vivo. Invest Ophthalmol Vis Sci 41:262–266
Ladewig G, Reinhold U, Thirkill CE, Kerber A, Tilgen W, Pfohler C (2005) Incidence of antiretinal antibodies in melanoma: screening of 77 serum samples from 51 patients with American Joint Committee on Cancer stage I-IV. Br J Dermatol 152:931–938
Jacobson DM, Adamus G (2001) Retinal anti-bipolar cell antibodies in a patient with paraneoplastic retinopathy and colon carcinoma. Am J Ophthalmol 131:806–808
Masaoka N, Emoto Y, Sasaoka A, Fukushima A, Ueno H, Ohguro H (1999) Fluorescein angiographic findings in a case of cancer-associated retinopathy. Retina 19:462–464
Ohguro H, Yokoi Y, Ohguro I, Mamiya K, Ishikawa F, Yamazaki H, Metoki T, Takano Y, Ito T, Nakazawa M (2004) Clinical and immunologic aspects of cancer-associated retinopathy. Am J Ophthalmol 137:1117–1119
Dot C, Guigay J, Adamus G (2005) Anti-alpha-enolase antibodies in cancer-associated retinopathy with small cell carcinoma of the lung. Am J Ophthalmol 139:746–747
Weleber RG, Watzke RC, Shults WT, Trzupek KM, Heckenlively JR, Egan RA, Adamus G (2005) Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies. Am J Ophthalmol 139:780–794
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Goetgebuer, G., Kestelyn-Stevens, AM., De Laey, JJ. et al. Cancer-associated retinopathy (CAR) with electronegative ERG: a case report. Doc Ophthalmol 116, 49–55 (2008). https://doi.org/10.1007/s10633-007-9074-9
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DOI: https://doi.org/10.1007/s10633-007-9074-9