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Hemeralopia in an older adult
SURVEY OF OPHTHALMOLOGY
VOLUME 37.
NUMBER 3. NOVEMBER-DECEMBER
1992
CLINICAL CHALLENGES RONALD BURDE AND PETER SAVINO, EDITORS
Hemeralopia in an Older Adult KAREN
GEHRS,
M.D., AND JAMES TIEDEMAN,
M.D., PH.D.
Duke University Eye Center, Durham, North Carolina
(In keeping with the CPC
f ormat,
the abstract and key words are Presented at the end of this Paper.)
Case Report. A 69-year-old white male first noted difhculty seeing in bright illumination in June of 1988. He was seen by an ophthalmologist who found the patient’s visual acuity to be 20/30 in both eyes in dim illumination and 20/70 in both eyes in bright illumination. Bilateral nuclear sclerotic cataracts were diagnosed, and it was felt that the patient’s decreased visual acuity in bright light was due to glare from his cataracts. Cataract extraction was recommended and the patient underwent an uncomplicated extracapsular cataract extraction with a posterior chamber lens implant in his right eye. His visual acuity improved to 20/20 with correction postoperatively. The patient continued to notice a gradual decrease in vision in both eyes, and by October of 1989 he had a corrected visual acuity of 20130 in the right eye. He underwent fluorescein angiography, which revealed macular pigmentary changes and drusen. The decrease in his vision was attributed to macular pathology, and he was started on multivitamins with selenium and zinc. In November of 1989, the patient, now 7 1 years old, returned complaining of a continued decrease in vision in the right eye. He was found to have a visual acuity of 20125 in the right eye, which decreased to 20/70 in bright light. He demonstrated a markedly prolonged macular photostress time, with visual acuity remaining at the 20/70 level for 30 minutes after indirect ophthalmoscopy. He was referred to the Duke
Eye Center for evaluation of hemeralopia. The patient’s primary complaints in December of 1989 were the same as those in June of 1988: difficulty seeing in bright daylight and difficulty adapting to dim illumination. His past medical history was remarkable for hypertension, myocardial infarction, adult onset diabetes mellitus, chronic obstructive pulmonary disease, cholelithiasis, and benign prostatic hypertrophy. His medications included Aldomet, Uniphyl, metaclopramide, and Tranxene. He had a 48pack-year smoking history and denied alcohol ingestion. His diet was said to be well balanced, and he denied a history of weight loss. He had no family history of ocular disorders suggestive of a cone dystrophy and had no prior history of color blindness. On examination, corrected visual acuity was 20!40 in both eyes. Intraocular pressures were 11 mm of mercury on the right, and 13 mm of mercury on the left. Slit-lamp examination of the right eye revealed a well-healed limbal wound superiorly, a clear cornea, deep and quiet anterior chamber and well-positioned posterior chamber intraocular lens. Slit-lamp examination of the left eye was unremarkable. Funduscopic examination of the right eye revealed a clear view to the fundus, marked disc pallor, marked attenuation of retinal vessels, and a coarse granular appearance of the pigment epithelium in the posterior pole (Fig. 1). Funduscopic examination of the left eye was similar to the right.
185
186
Surv Ophthalmol
37 (3) November-December
What is your differential diagnosis? What ophthalmologic studies would you perarm? f Goldmann
visual field testing
Fig. 2. Goldmann
revealed
normal
visual fields at the time of presentation
GEHRS, TIEDEMAN
1992 peripheral
fields with relative
central
scotomata
(Figs. 2 and 3). Fl uorescein angiography demonstrated a 20-second delay in retinal and choroida1 filling,
and diffuse
RPE mottling.
reveal central and paracentral
An electro-
scotomata.
Fig. 3. Goldmann visual fields eighteen months after diagnosis and treatment reveal increased central scotomata.
HEMERALOPIA IN AN OLDER ADULT
187
r;ig. 5. Light adapted electroretinograms at the time of presentation reveal extinguished cone ftmction. (top: 30 Hz flicker, bottom: light adapted white flash)
Fig. 4. Dark adapted electroretinograms at the time of presentation reveal diminished rod and cone function (top: dark adapted blue flash, middle: dark adapted red flash, bottom: dark adapted light flash)
revealed nonrecordable cone retinogram responses and grossly attenuated rod responses in both eyes (Figs. 4 and 5). Laboratory studies included a hemoglobin of 11.8 gms/dl, normal platelet count, white blood count, and serum chemistries, a nonreactive VDRL and a westergren sedimentation rate of 51 mm/hour.
What is your diagnosis now? How would you proceed from here?
Discussion The patient’s primary complaints were of hemeralopia and prolonged dark adaptation. Early in the course of this condition his symptoms were attributed to glare from cataract. When cataract extraction did not alleviate his symptoms, he was referred for further evaluation. Clinically he was observed to have decreased vision, normal anterior segments, and
evidence of widespread retinal degeneration with vascular attenuation and disc pallor. At this point the differential diagnosis included cone or cone-rod dystrophies, toxic or metabolic retinopathies, and paraneoplastic retinopathy. There were no focal retinal, vascular, or choroidal lesions and no evidence for diffuse choroidal disease. Presentation of an inherited cone or cone-rod dystrophy in a 69-year-old patient would have been highly unlikely. The patient gave no history suggestive of cone dysfunction prior to 1988. There was no family history of cone dysfunction. A primary rod dystrophy was not considered, as the patient denied nyctalopia and actually saw better in dim illumination. Toxic and metabolic etiologies could be excluded by the patient’s history. There was no history of quinine, chloroquine, or methanol ingestion. Additionally, the patient demonstrated full peripheral visual fields and abnormal central fields unlike quinine and methanol toxicity which produce constricted fields. There was no postoperative history to suggest gentamicin toxicity due to inadvertent intraocular injection, and funduscopic changes were bilateral and symmetric. The patient stated that his diet was good, and he demonstrated no anterior segment signs of vitamin A deficiency. He also denied nyctalopia, which would have been suggestive of vitamin A deficiency.
188
Surv Ophthalmol
37 (3) November-December
Hemeralopia and prolonged photostress recovery time suggested cone dysfunction. Markedly prolonged dark adaptation indicated rod dysfunction as well. Funduscopic examination was consistent with widespread photoreceptor degeneration. ERG confirmed the absence of cone function and attenuated rod function. As cone and conerod dystrophies and toxic/metabolic etiologies could be excluded, the most likely diagnosis was thought to be a visual paraneoplastic syndrome. Visual paraneoplastic syndrome, paraneoplastic retinopathy, or carcinoma associated retinopathy (CAR) is a rare disorder in which widespread photoreceptor degeneration occurs in association with a remote, and frequently occult, malignancy. Since Sawyer’s original description of this condition in three patients with small cell carcinoma of the lung, an additional eighteen case reports have appeared in the literature. ‘-g,13-15Paraneoplastic retinopathy has been reported to occur with breast carcinoma, cervical carcinoma, endometrial carcinoma, undifferentiated lung carcinoma, and malignant melanoma. Patients with carcinoma associated retinopathy typically experience hemeralopia or nyctalopia, a progressive decrease in vision, and dyschromatopsia.‘-g~‘3-‘5 Some experience a flickering lights sensation.” Funduscopic examination may initially be normal, but later reveals optic disc pallor, retinal arterial attenuation, and pigmentary changes. Color vision is often abnormal and visual field testing may reveal central, or constricparacentral , or ring scotomata tion.2,4-6,8,g,‘1,13,14Electroretinograms generally demonstrate markedly diminished rod and cone studies have responses. ‘-‘J~J~ Histopathologic revealed marked degeneration or absence of photoreceptors with gliosis of outer retinal layers. Ultrastructural studies have demonstrated gliosis, macrophages containing melanin, and accumulation of premelanosomes and melanosomes in the RPE suggestive of abnormal melanogenesis.2~3~15*‘4On e author noted thickening of Bruch’s membrane and basal laminar deposits, although these findings could have been associated with age-related macular degeneration in a 66-year-old patient. ’ Other authors have noted no changes in Bruch’s membrane or the choriocapillaris. Carcinoma associated retinopathy ^ ,. (CAR), like other paraneoplastic syndromes, appears to be an autoimmune phenomenon. Some patients have been reported to respond, at least tran-
1992
GEHRS, TIEDEMAN
Fig. 6. Chest CT reveals parahilar mass in left lung (arrow).
siently, to systemic corticosteroid or other immunosuppressive therapy.‘-‘*14 Several authors report detection of antiretinal antibodies in patients with this syndrome.4-8,10*‘2*14*‘5Grunwald and coworkers demonstrated the presence of serum antiretinal antibodies in patients with small cell carcinoma of the lung and visual paraneoplastic syndrome.5 These authors subsequently demonstrated breakdown of the blood retinal barrier with antibodies against both tumor cells and retinal antigens deposited in the retina.‘j Kornguth and coworkers studied sera from patients with visual paraneoplastic syndrome, patients with cancer but no ocular disease, and normal controls. Using immunohistochemical techniques, they demonstrated that antisera from patients with carcinomas and visual paraneoplastic syndrome reacted with retinal antigens, but antisera from patients with carcinoma and no visual symptoms or from normal controls did not react with retinal antigens.” Polans and coworkers have isolated a 26 kD protein which they feel is the CAR antigen and which shares 90% homology with visinin, a cone photoreceptor protein similar to calmodulin.‘2 Thirkill and others have isolated a 23 kD protein that binds to retinal antigens in four patients with cancer of the cervix and lung. They were unable to detect this antigen in patients with lung cancer and no visual complaints or in normal controls.‘4”5 All of these authors postulate mechanisms by which antibodies against tumor antigens are able to cross the blood retinal barrier and crossreact with retinal antigens.
Case Report, Continued The clinical impression was that of an acquired photoreceptor degeneration secondary to occult malignancy (carcinoma associated retinopathy).
HEMERALOPIA
IN AN OLDER
189
ADULT
References
The patient was referred to an internest for systemic evaluation. Initial evaluation including chest X-ray, chest and abdominal CT, upper GI endoscopy, and barium enema were negative. A repeat chest CT one month later demonstrated a 4 x 2.5 cm mass in the left upper lung with associated adenopathy (Fig. 6). An incomplete resection revealed the mass to be squamous cell carcinoma of the lung with local nodal metastases. Further work-up staged the tumor as T2N2MO. The patient underwent a total of 6000 rads radiation therapy to the left lung. He has demonstrated no evidence of local recurrent disease or metastatic disease over the 18 months following tumor excision and radiation therapy, but his vision has continued to deteriorate. Corrected visual acuity on last examination, 18 months after radiation therapy, was 20/80 OD and 20/60 OS. A trial of systemic steroids did not improve his visual acuity, visual fields, or electroretinogram.
1. Berson E, Lessell S: Paraneoplastic night blindness associated with malignant melanoma. Am J 0$&&n& 106: 307-311, 1988 2. Buchanan TA, Gardener TA, Archer DB: An ultra-structural study of retinal photoreceptor degeneration associated with bronchial carcinoma. Am J O@halmol97:277287, 1984 3. Cogan DG, KuwabaraT, Curie J, Kattah J: Para-neoplastische Retinonathie unter dem klinischen Bild einer Zanfendystrophik mit Achromatopsie. Klin Monatsbl Augek 1990 heilld 197:15&158, 4. Crofts JW, et al: Visual paraneoplastic syndrome associated with undifferentiated endometrial carcinoma. Can J Ophthalmol23:128-133, 1988 5. Grunwald GB, et al: Autoimmune basis for visual paraneoplastic syndrome in patients with small cell lung car1987 cinoma. Cancer 60:780-786, 6. Grunwald GB, et al: Autoimmune basis for visual paraneoplastic syndrome in patients with small cell lung carcinoma. Lancet 1:658-66 1, 1985 7. Jacobson DM, Thirkill CE, Tipping SJ: A clinical triad to diagnose paraneoplastic retinopathy. Ann New01 28: 162-167, 1990 8. Keltner JL, Roth AM, Chang RS: Photoreceptor degeneration: possible autoimmune disorder. Arch Ophthulmol 101:564-569, 1983 9. Klingell TG, et al: Paraneoplastic retinopathy. J Clin Neuroo$hthalmol 4:239-245, 1984 10. Korngath SE, et al: Occurrence of antiretinal ganglion cell antibodies in patients with small cell carcinoma ofthe lung. Cancer 50:1289-1293, 1982 11. Korngath SE, et al: Anti-neurofilament antibodies in the sera of patients with small cell carcinoma of the lung and with visual paraneoplastic syndrome. Cancer Res 46: 2588-95, 1986 12. Polans AS, Buczylko J, Crabb J, Palczewski K: A photoreceptor calcium binding protein is recognized by autoantibodies obtained from patients with cancer associated retinopathy. J Cell Biol 112:981-989, 1991 13. Sawyer RA, et al: Blindness caused by photoreceptor degeneration as a remote effect of cancer. Am J Ofhthulmol81:606-613, 1976 14. Thirkill CE, et al: Cancer associated retinopathy. Arch Ophthalmol 105:372-375, 1987 15. Thirkill CE, et al: Cancer associated retinopathy (CAR syndrome) with antibodies reacting with retinal, optic nerve, and cancer cells. N Engl J Med 321:1589-1594, 1989
Summary Paraneoplastic retinopathy is a rare disorder involving widespread photoreceptor degeneration as a remote effect of cancer. It is probably an autoimmune phenomenon. Paraneoplastic retinopathy has been reported in association with small cell lung carcinoma, breast carcinoma, cervical carcinoma, endometrial carcinoma, and malignant melanoma. We report the first case associated with squamous cell carcinoma of the lung. We also emphasize the occult nature of the primary tumor in this patient who presented with ocular symptoms nearly two years before a diagnosis of lung cancer, and who underwent extensive systemic evaluation twice before the primary tumor was discovered. Early recognition of this syndrome with diagnosis of an occult, resectable primary tumor may be life-saving.
Reprint address: Dr. Tiedeman, Univ. of Virginia, Dept. of Ophthalmology, PO Box 475, Charlottesville, VA 22908.
Abstract. An elderly male was referred for evaluation of hemeralopia when cataract extraction did not alleviate his symptom of difficulty seeing in bright illumination. Visual acuity examination revealed marked arteriolar attenuation, disc was 20/40 OU. Funduscopic pallor, central
and retinal pigment epithelial changes. Visual fields demonstrated scotomata. An ERG was consistent with widespread photoreceptor
relative
para-
degeneration. A diagnosis of visual paraneoplastic syndrome was made and an extensive systemic evaluation revealed a squamous cell carcinoma of the lung. The presentation and evaluation of acquired photoreceptor dysfunction in adulthood is discussed. (Surv Ophthalmol37: 185189, 1992)
Key words. cancer associated retinopathy paraneoplastic syndrome photoreceptor ??
hemeralopia degeneration ??
??
lung cancer
??
VOLUME 37.
NUMBER 3. NOVEMBER-DECEMBER
1992
CLINICAL CHALLENGES RONALD BURDE AND PETER SAVINO, EDITORS
Hemeralopia in an Older Adult KAREN
GEHRS,
M.D., AND JAMES TIEDEMAN,
M.D., PH.D.
Duke University Eye Center, Durham, North Carolina
(In keeping with the CPC
f ormat,
the abstract and key words are Presented at the end of this Paper.)
Case Report. A 69-year-old white male first noted difhculty seeing in bright illumination in June of 1988. He was seen by an ophthalmologist who found the patient’s visual acuity to be 20/30 in both eyes in dim illumination and 20/70 in both eyes in bright illumination. Bilateral nuclear sclerotic cataracts were diagnosed, and it was felt that the patient’s decreased visual acuity in bright light was due to glare from his cataracts. Cataract extraction was recommended and the patient underwent an uncomplicated extracapsular cataract extraction with a posterior chamber lens implant in his right eye. His visual acuity improved to 20/20 with correction postoperatively. The patient continued to notice a gradual decrease in vision in both eyes, and by October of 1989 he had a corrected visual acuity of 20130 in the right eye. He underwent fluorescein angiography, which revealed macular pigmentary changes and drusen. The decrease in his vision was attributed to macular pathology, and he was started on multivitamins with selenium and zinc. In November of 1989, the patient, now 7 1 years old, returned complaining of a continued decrease in vision in the right eye. He was found to have a visual acuity of 20125 in the right eye, which decreased to 20/70 in bright light. He demonstrated a markedly prolonged macular photostress time, with visual acuity remaining at the 20/70 level for 30 minutes after indirect ophthalmoscopy. He was referred to the Duke
Eye Center for evaluation of hemeralopia. The patient’s primary complaints in December of 1989 were the same as those in June of 1988: difficulty seeing in bright daylight and difficulty adapting to dim illumination. His past medical history was remarkable for hypertension, myocardial infarction, adult onset diabetes mellitus, chronic obstructive pulmonary disease, cholelithiasis, and benign prostatic hypertrophy. His medications included Aldomet, Uniphyl, metaclopramide, and Tranxene. He had a 48pack-year smoking history and denied alcohol ingestion. His diet was said to be well balanced, and he denied a history of weight loss. He had no family history of ocular disorders suggestive of a cone dystrophy and had no prior history of color blindness. On examination, corrected visual acuity was 20!40 in both eyes. Intraocular pressures were 11 mm of mercury on the right, and 13 mm of mercury on the left. Slit-lamp examination of the right eye revealed a well-healed limbal wound superiorly, a clear cornea, deep and quiet anterior chamber and well-positioned posterior chamber intraocular lens. Slit-lamp examination of the left eye was unremarkable. Funduscopic examination of the right eye revealed a clear view to the fundus, marked disc pallor, marked attenuation of retinal vessels, and a coarse granular appearance of the pigment epithelium in the posterior pole (Fig. 1). Funduscopic examination of the left eye was similar to the right.
185
186
Surv Ophthalmol
37 (3) November-December
What is your differential diagnosis? What ophthalmologic studies would you perarm? f Goldmann
visual field testing
Fig. 2. Goldmann
revealed
normal
visual fields at the time of presentation
GEHRS, TIEDEMAN
1992 peripheral
fields with relative
central
scotomata
(Figs. 2 and 3). Fl uorescein angiography demonstrated a 20-second delay in retinal and choroida1 filling,
and diffuse
RPE mottling.
reveal central and paracentral
An electro-
scotomata.
Fig. 3. Goldmann visual fields eighteen months after diagnosis and treatment reveal increased central scotomata.
HEMERALOPIA IN AN OLDER ADULT
187
r;ig. 5. Light adapted electroretinograms at the time of presentation reveal extinguished cone ftmction. (top: 30 Hz flicker, bottom: light adapted white flash)
Fig. 4. Dark adapted electroretinograms at the time of presentation reveal diminished rod and cone function (top: dark adapted blue flash, middle: dark adapted red flash, bottom: dark adapted light flash)
revealed nonrecordable cone retinogram responses and grossly attenuated rod responses in both eyes (Figs. 4 and 5). Laboratory studies included a hemoglobin of 11.8 gms/dl, normal platelet count, white blood count, and serum chemistries, a nonreactive VDRL and a westergren sedimentation rate of 51 mm/hour.
What is your diagnosis now? How would you proceed from here?
Discussion The patient’s primary complaints were of hemeralopia and prolonged dark adaptation. Early in the course of this condition his symptoms were attributed to glare from cataract. When cataract extraction did not alleviate his symptoms, he was referred for further evaluation. Clinically he was observed to have decreased vision, normal anterior segments, and
evidence of widespread retinal degeneration with vascular attenuation and disc pallor. At this point the differential diagnosis included cone or cone-rod dystrophies, toxic or metabolic retinopathies, and paraneoplastic retinopathy. There were no focal retinal, vascular, or choroidal lesions and no evidence for diffuse choroidal disease. Presentation of an inherited cone or cone-rod dystrophy in a 69-year-old patient would have been highly unlikely. The patient gave no history suggestive of cone dysfunction prior to 1988. There was no family history of cone dysfunction. A primary rod dystrophy was not considered, as the patient denied nyctalopia and actually saw better in dim illumination. Toxic and metabolic etiologies could be excluded by the patient’s history. There was no history of quinine, chloroquine, or methanol ingestion. Additionally, the patient demonstrated full peripheral visual fields and abnormal central fields unlike quinine and methanol toxicity which produce constricted fields. There was no postoperative history to suggest gentamicin toxicity due to inadvertent intraocular injection, and funduscopic changes were bilateral and symmetric. The patient stated that his diet was good, and he demonstrated no anterior segment signs of vitamin A deficiency. He also denied nyctalopia, which would have been suggestive of vitamin A deficiency.
188
Surv Ophthalmol
37 (3) November-December
Hemeralopia and prolonged photostress recovery time suggested cone dysfunction. Markedly prolonged dark adaptation indicated rod dysfunction as well. Funduscopic examination was consistent with widespread photoreceptor degeneration. ERG confirmed the absence of cone function and attenuated rod function. As cone and conerod dystrophies and toxic/metabolic etiologies could be excluded, the most likely diagnosis was thought to be a visual paraneoplastic syndrome. Visual paraneoplastic syndrome, paraneoplastic retinopathy, or carcinoma associated retinopathy (CAR) is a rare disorder in which widespread photoreceptor degeneration occurs in association with a remote, and frequently occult, malignancy. Since Sawyer’s original description of this condition in three patients with small cell carcinoma of the lung, an additional eighteen case reports have appeared in the literature. ‘-g,13-15Paraneoplastic retinopathy has been reported to occur with breast carcinoma, cervical carcinoma, endometrial carcinoma, undifferentiated lung carcinoma, and malignant melanoma. Patients with carcinoma associated retinopathy typically experience hemeralopia or nyctalopia, a progressive decrease in vision, and dyschromatopsia.‘-g~‘3-‘5 Some experience a flickering lights sensation.” Funduscopic examination may initially be normal, but later reveals optic disc pallor, retinal arterial attenuation, and pigmentary changes. Color vision is often abnormal and visual field testing may reveal central, or constricparacentral , or ring scotomata tion.2,4-6,8,g,‘1,13,14Electroretinograms generally demonstrate markedly diminished rod and cone studies have responses. ‘-‘J~J~ Histopathologic revealed marked degeneration or absence of photoreceptors with gliosis of outer retinal layers. Ultrastructural studies have demonstrated gliosis, macrophages containing melanin, and accumulation of premelanosomes and melanosomes in the RPE suggestive of abnormal melanogenesis.2~3~15*‘4On e author noted thickening of Bruch’s membrane and basal laminar deposits, although these findings could have been associated with age-related macular degeneration in a 66-year-old patient. ’ Other authors have noted no changes in Bruch’s membrane or the choriocapillaris. Carcinoma associated retinopathy ^ ,. (CAR), like other paraneoplastic syndromes, appears to be an autoimmune phenomenon. Some patients have been reported to respond, at least tran-
1992
GEHRS, TIEDEMAN
Fig. 6. Chest CT reveals parahilar mass in left lung (arrow).
siently, to systemic corticosteroid or other immunosuppressive therapy.‘-‘*14 Several authors report detection of antiretinal antibodies in patients with this syndrome.4-8,10*‘2*14*‘5Grunwald and coworkers demonstrated the presence of serum antiretinal antibodies in patients with small cell carcinoma of the lung and visual paraneoplastic syndrome.5 These authors subsequently demonstrated breakdown of the blood retinal barrier with antibodies against both tumor cells and retinal antigens deposited in the retina.‘j Kornguth and coworkers studied sera from patients with visual paraneoplastic syndrome, patients with cancer but no ocular disease, and normal controls. Using immunohistochemical techniques, they demonstrated that antisera from patients with carcinomas and visual paraneoplastic syndrome reacted with retinal antigens, but antisera from patients with carcinoma and no visual symptoms or from normal controls did not react with retinal antigens.” Polans and coworkers have isolated a 26 kD protein which they feel is the CAR antigen and which shares 90% homology with visinin, a cone photoreceptor protein similar to calmodulin.‘2 Thirkill and others have isolated a 23 kD protein that binds to retinal antigens in four patients with cancer of the cervix and lung. They were unable to detect this antigen in patients with lung cancer and no visual complaints or in normal controls.‘4”5 All of these authors postulate mechanisms by which antibodies against tumor antigens are able to cross the blood retinal barrier and crossreact with retinal antigens.
Case Report, Continued The clinical impression was that of an acquired photoreceptor degeneration secondary to occult malignancy (carcinoma associated retinopathy).
HEMERALOPIA
IN AN OLDER
189
ADULT
References
The patient was referred to an internest for systemic evaluation. Initial evaluation including chest X-ray, chest and abdominal CT, upper GI endoscopy, and barium enema were negative. A repeat chest CT one month later demonstrated a 4 x 2.5 cm mass in the left upper lung with associated adenopathy (Fig. 6). An incomplete resection revealed the mass to be squamous cell carcinoma of the lung with local nodal metastases. Further work-up staged the tumor as T2N2MO. The patient underwent a total of 6000 rads radiation therapy to the left lung. He has demonstrated no evidence of local recurrent disease or metastatic disease over the 18 months following tumor excision and radiation therapy, but his vision has continued to deteriorate. Corrected visual acuity on last examination, 18 months after radiation therapy, was 20/80 OD and 20/60 OS. A trial of systemic steroids did not improve his visual acuity, visual fields, or electroretinogram.
1. Berson E, Lessell S: Paraneoplastic night blindness associated with malignant melanoma. Am J 0$&&n& 106: 307-311, 1988 2. Buchanan TA, Gardener TA, Archer DB: An ultra-structural study of retinal photoreceptor degeneration associated with bronchial carcinoma. Am J O@halmol97:277287, 1984 3. Cogan DG, KuwabaraT, Curie J, Kattah J: Para-neoplastische Retinonathie unter dem klinischen Bild einer Zanfendystrophik mit Achromatopsie. Klin Monatsbl Augek 1990 heilld 197:15&158, 4. Crofts JW, et al: Visual paraneoplastic syndrome associated with undifferentiated endometrial carcinoma. Can J Ophthalmol23:128-133, 1988 5. Grunwald GB, et al: Autoimmune basis for visual paraneoplastic syndrome in patients with small cell lung car1987 cinoma. Cancer 60:780-786, 6. Grunwald GB, et al: Autoimmune basis for visual paraneoplastic syndrome in patients with small cell lung carcinoma. Lancet 1:658-66 1, 1985 7. Jacobson DM, Thirkill CE, Tipping SJ: A clinical triad to diagnose paraneoplastic retinopathy. Ann New01 28: 162-167, 1990 8. Keltner JL, Roth AM, Chang RS: Photoreceptor degeneration: possible autoimmune disorder. Arch Ophthulmol 101:564-569, 1983 9. Klingell TG, et al: Paraneoplastic retinopathy. J Clin Neuroo$hthalmol 4:239-245, 1984 10. Korngath SE, et al: Occurrence of antiretinal ganglion cell antibodies in patients with small cell carcinoma ofthe lung. Cancer 50:1289-1293, 1982 11. Korngath SE, et al: Anti-neurofilament antibodies in the sera of patients with small cell carcinoma of the lung and with visual paraneoplastic syndrome. Cancer Res 46: 2588-95, 1986 12. Polans AS, Buczylko J, Crabb J, Palczewski K: A photoreceptor calcium binding protein is recognized by autoantibodies obtained from patients with cancer associated retinopathy. J Cell Biol 112:981-989, 1991 13. Sawyer RA, et al: Blindness caused by photoreceptor degeneration as a remote effect of cancer. Am J Ofhthulmol81:606-613, 1976 14. Thirkill CE, et al: Cancer associated retinopathy. Arch Ophthalmol 105:372-375, 1987 15. Thirkill CE, et al: Cancer associated retinopathy (CAR syndrome) with antibodies reacting with retinal, optic nerve, and cancer cells. N Engl J Med 321:1589-1594, 1989
Summary Paraneoplastic retinopathy is a rare disorder involving widespread photoreceptor degeneration as a remote effect of cancer. It is probably an autoimmune phenomenon. Paraneoplastic retinopathy has been reported in association with small cell lung carcinoma, breast carcinoma, cervical carcinoma, endometrial carcinoma, and malignant melanoma. We report the first case associated with squamous cell carcinoma of the lung. We also emphasize the occult nature of the primary tumor in this patient who presented with ocular symptoms nearly two years before a diagnosis of lung cancer, and who underwent extensive systemic evaluation twice before the primary tumor was discovered. Early recognition of this syndrome with diagnosis of an occult, resectable primary tumor may be life-saving.
Reprint address: Dr. Tiedeman, Univ. of Virginia, Dept. of Ophthalmology, PO Box 475, Charlottesville, VA 22908.
Abstract. An elderly male was referred for evaluation of hemeralopia when cataract extraction did not alleviate his symptom of difficulty seeing in bright illumination. Visual acuity examination revealed marked arteriolar attenuation, disc was 20/40 OU. Funduscopic pallor, central
and retinal pigment epithelial changes. Visual fields demonstrated scotomata. An ERG was consistent with widespread photoreceptor
relative
para-
degeneration. A diagnosis of visual paraneoplastic syndrome was made and an extensive systemic evaluation revealed a squamous cell carcinoma of the lung. The presentation and evaluation of acquired photoreceptor dysfunction in adulthood is discussed. (Surv Ophthalmol37: 185189, 1992)
Key words. cancer associated retinopathy paraneoplastic syndrome photoreceptor ??
hemeralopia degeneration ??
??
lung cancer
??