tibodies cross-reacting with pathogens expressed by carcinoma cells. Cancer-associated retinopathy with antirecoverin antibody generally shows rapidly progressive visual deterioration.1-3 Autoimmune retinopathy (AIR) is characterized by antiretinal antibody–positive serum in the absence of systemic carcinoma but with slowly progressive visual deterioration,1,3,4 although the pathogenic mechanism is uncertain. We describe a patient with CAR with antirecoverin antibody who had slowly progressive visual deterioration resembling AIR. Figure 3. At 3-month follow-up, coarse pigment clumping and atrophy at the macula and gross attenuation of the macular arterioles are evident. Note also the optic disc pallor. mg/wk) were started. Three months later, the visual acuity remained no light perception. Optic disc pallor and gross attenuation of perifoveal vessels were noted. The macula showed pigment clumping and atrophy (Figure 3). Comment. Snake venom is a complex heterogeneous composition of substances that predominantly affects the synapse (neurotoxic) or coagulation pathway(hemotoxic).Largedosescan resultindisseminatedintravascularcoagulopathy (DIC) and ischemic damage to vital organs. Toxic vasculitis has reportedly been caused by certain species of the Viperidae family.5 Hemorrhagins (complement-mediated toxic components of viperine venom) may induce severe vasospasm, endothelial damage, and increased vascular permeability.Vasospasmand/orDICmay result in vascular occlusion. Fibrin thrombi in the capillaries, perivascular hemorrhages, and necrosis, all observed in our patient, are features of DIC. The likely cause of visual loss could be (1) ophthalmic artery occlusion with subsequent dislodge of fibrin emboli into the end arterioles at the posterior pole; or (2) retinal necrosis and macular infarction secondary to an aborted DIC process associated with toxic optic neuropathy (venom or ASV serum toxicity). Ocular complications following a snake bite range from keratomalacia to vitreous hemorrhage,4 uveitis, optic neuritis,3 globe necrosis, and vi- sual loss due to cortical infarction.5 We are unaware of any previous report in the literature of macular infarction following a viperine snake bite. Visual prognosis is poor despite medical treatment. Jatinder Singh, MS, DNB Preetam Singh, DOMS Rajbir Singh, MS Vipin Kumar Vig, MS Correspondence: Dr Singh, Sardar Bahadur Dr Sohan Singh Eye Hospital, Katra Sher Singh, Chowk Farid, Amritsar-143006, Punjab, India ([email protected]). Financial Disclosure: None reported. 1. Campochiaro PA, Conway BP. Aminoglycoside toxicity—a survey of retinal specialists: implications for ocular use. Arch Ophthalmol. 1991; 109(7):946-950. 2. Bhalla A, Jain AP, Banait S, et al. Central retinal artery occlusion: an unusual complication of snake bite. J Venom Anim Toxins Incl Trop Dis. 2004;10(3):311-314. 3. Menon V, Tandon R, Sharma T, Gupta A. Optic neuritis following snake bite. Indian J Ophthalmol. 1997;45(4):236-237. 4. Rao BM. A case of bilateral vitreous haemorrhage following snake bite. Indian J Ophthalmol. 1977;25(2):1-2. 5. Merle H, Donnio A, Ayeboua L, et al. Occipital infarction revealed by quadranopsia following snake bite by Bothrops lanceolatus. Am J Trop Med Hyg. 2005;73(3):583-585. Slowly Progressive Cancer-Associated Retinopathy Cancer-associated retinopathy (CAR) is caused by antiretinal an- (REPRINTED) ARCH OPHTHALMOL / VOL 125 (NO. 10), OCT 2007 1431 Report of a Case. In 2004, we examined a 62-year-old woman with a 10-year history of progressive visual loss and night blindness. In 1994, Goldmann perimetry and electroretinography (ERG) at another hospital showed retinitis pigmentosa–like findings in the right eye and a normal appearance in the left. In 2000, ERG response showed further deterioration in the right eye and a normal response in the left. In 2002, the left eye also exhibited a visual field defect and ERG abnormality. The patient had no history of carcinoma or ocular trauma. When examined in 2004, our patient’s visual acuities were 20/30 OD and 20/20 OS. Slitlamp examination demonstrated mild iridocyclitis and nuclear cataract bilaterally. Funduscopic examination results demonstrated optic disc pallor, retinal artery whitening, and retinal pigment epithelial atrophy with partial pigmentation in the midperipheral area across 360° (Figure 1A). Fluorescein angiography demonstrated a window defect corresponding to the retinal degeneration (Figure 1B) and late leakage from retinal capillary vessels. Goldmann perimetry demonstrated central and temporal islands in the right eye and a ring scotoma in the left. Results of single bright-flash ERG were unrecordable bilaterally. Western blot analysis detected no serum autoantibodies. One year later, a visual field defect developed in the left eye. Western blot analysis detected serum autoantibody against a 23-kDa protein (recoverin) (Figure 2A). Although the slowly progressive vi- WWW.ARCHOPHTHALMOL.COM ©2007 American Medical Association. All rights reserved. Downloaded From: http://archpsyc.jamanetwork.com/ on 10/15/2014 was initiated and then tapered across 5 months. At the last follow-up examination, the patient’s visual acuity remained unchanged (20/30 OD and 20/20 OS), with no further progression of the visual field defect. A Comment. Patients with AIR exhibit slowly progressive visual deterioration mimicking retinitis pigmentosa, cystoid macular edema, and retinal vascular edema on fluorescein angiography.1,4 Some patients have systemic benign tumors.4 The patient we describe was diagnosed as having CAR with antirecoverin antibody 11 years after the initial visual symptoms appeared despite clinical features resembling AIR. Our group previously described a patient with CAR who had aberrant expression of recoverin in many carcinoma cells.2 In the current patient the causative tumor was quite small and only a few carcinoma cells expressed the recoverin antigen, suggesting that the slow clinical course correlated with the low number of recoverin-immunopositive tumor cells, unlike patients in previously reported cases.1,2 Based on the clinical course and immunohistochemical findings, we infer that lung adenocarcinoma or preneoplastic lesions, such as atypical adenomatous hyperplasia5 expressing recoverin, were present when the initial visual symptoms were noticed, suggesting that some patients with AIR have an occult preneoplastic or malignant tumor expressing recoverin. We conclude that regular screening for systemic cancer may be necessary to save the lives and vision of patients with presumed AIR. B Wataru Saito, MD Satoru Kase, MD Hiroshi Ohguro, MD Naoki Furudate, MD Shigeaki Ohno, MD Figure 1. Fundus photographs of the right eye on the initial visit in 2004. A, Fundus photograph demonstrates optic disc pallor, retinal artery whitening, and retinal pigment epithelial atrophy with partial pigmentation in the midperipheral area. B, Fluorescein angiography (42 seconds after dye injection) demonstrates a window defect corresponding to retinal degeneration. sual dysfunction suggested AIR, systemic screening detected a 2-mm bronchioloalveolar carcinoma without metastasis, and the patient was treated with lobectomy. Subsequently, CAR was diagnosed. A few carcinoma cells exhibited cytoplasmic immunoreactivity for recoverin (Figure 2C). One month after the lobectomy, the patient’s visual acuity decreased and late-phase fluorescein angiography demonstrated cystoid macular edema in the left eye and marked leakage from retinal capillary vessels bilaterally. Oral administration of prednisolone, 40 mg/d, (REPRINTED) ARCH OPHTHALMOL / VOL 125 (NO. 10), OCT 2007 1432 Correspondence: Dr Saito, Department of Ophthalmology and Visual Sciences, Hokkaido University Graduate School of Medicine, Nishi 7 chome, Kita 15 jou, Kita-ku, Sapporo 060-8638, Japan (wsaito @med.hokudai.ac.jp). Financial Disclosure: None reported. WWW.ARCHOPHTHALMOL.COM ©2007 American Medical Association. All rights reserved. Downloaded From: http://archpsyc.jamanetwork.com/ on 10/15/2014 A A B B C 150 kDa– 100 kDa– 75 kDa– 50 kDa– 37 kDa– 25 kDa– 15 kDa– Figure 2. Western blot analysis using bovine retinal soluble fractions in the patient’s serum (A) and immunohistochemistry performed on the resected lung sample (B and C) 1 year after the initial examination. A, Antibody to soluble 23-kDa (recoverin) protein was probed with the patient’s serum (lane A, 1:200 dilution). Lane B shows human recombinant recoverin. B and C, Hematoxylin-eosin staining (B) and immunodetection of recoverin (C) in bronchioloalveolar carcinoma. Cytoplasmic immunoreactivity for recoverin was noted in a few carcinoma cells (C) (original magnification ⫻200). 1. Chan JW. Paraneoplastic retinopathies and optic neuropathies. Surv Ophthalmol. 2003;48(1): 12-38. 2. Saito W, Kase S, Yoshida K, et al. Bilateral diffuse uveal melanocytic proliferation in a patient with cancer-associated retinopathy. Am J Ophthalmol. 2005;140(5):942-945. 3. Weleber RG, Watzke RC, Shults WT, et al. Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies. Am J Ophthalmol. 2005;139(5):780-794. 4. Heckenlively JR, Fawzi AA, Oversier J, Jordan BL, Aptsiauri N. Autoimmune retinopathy: patients with antirecoverin immunoreactivity and panretinal degeneration. Arch Ophthalmol. 2000; 118(11):1525-1533. 5. Greenberg AK, Yee H, Rom WN. Preneoplastic lesions of the lung. Respir Res. 2002;3:20-29. Cascade-like Oculomotor Misinnervation Report of a Case. A 6-month-old boy with a history of microcephaly and a type II atrioseptal defect was investigated for oculomotor abnormalities. There was no family history of oculomotor abnormalities. Examination showed an absence of adduction of the left eye on attempted right gaze and during attempted convergence. Instead, simultaneous abduction of both eyes was observed (Figure 1A). However, rhythmic adductive movements with an amplitude of approximately 10° were elicited upon sucking (Figure 1D). We observed both a slight narrowing of the left palpebral fissure and adductive movements simulta- neously. Exotropia was observed in the primary position and abduction of the left eye was limited on left gaze (Figure 1B and C). Upgaze and downgaze were normal. No ptosis or enophthalmos were correlated with these findings. Pupillary function was normal, with no unusual changes in eye movements. Right-eye motility was unremarkable. Comment. Congenital ocular misinnervation can occur in a variety of forms. It typically involves the sixth cranial nerve. Most common is the Duane, or congenital retraction, syndrome, which consists of hypoplasia of the sixth nerve (nucleus) and innervation of the lateral rectus muscle by a branch of the oculomotor nerve.1 Depending on the relative contribution of third nerve fibers to the medial and lateral rectus, the patient is first with either predominant limitation of abduction or adduction. Some of our patient’s clinical features can be explained by an extreme form of the Duane syndrome, where most, if not all, oculomotor nerve branch fibers originally directed to the medial rectus innervate the lateral rectus, thus leading to simultaneous abduction on attempted adduction (also referred to as synergistic divergence).2 Interestingly, although no lefteye adduction could be elicited on (REPRINTED) ARCH OPHTHALMOL / VOL 125 (NO. 10), OCT 2007 1433 lateral gaze or convergence, we observed adductive movements during sucking. Missing innervation of the medial rectus by oculomotor nerve fibers was replaced by fibers, most likely originating from a motor branch of the trigeminal nerve. Thus, the lack of innervation of the lateral rectus (Figure 2A) appears to have triggered a sequence of aberrant nerve sprouting, resulting, initially, in a shift of fibers originally meant for the medial rectus toward the lateral rectus (Figure 2B). Second, and possibly as a consequence of lack of innervation of the medial rectus muscle, a shift of trigeminal nerve motor fibers to the medial rectus took place (Figure 2C), leading to a trigemino-oculomotor synkinesis between the lateral pterygoid or one of the suprahyoid muscles and the medial rectus. The slight narrowing of the palpebral fissure observed during sucking can be explained by synkinetic contraction of the medial muscle against a tight lateral rectus muscle in that specific situation, which led to discrete retraction of the globe. The combination of the Duane and Marcus Gunn syndromes or other misinnervation syndromes involving the sixth, third, and fifth cranial nerves3,4 recurs. Other cases with synergistic divergence and a trigemino-oculomotor synkinesis have been reported4 (also WWW.ARCHOPHTHALMOL.COM ©2007 American Medical Association. All rights reserved. Downloaded From: http://archpsyc.jamanetwork.com/ on 10/15/2014