Ординатура / Офтальмология / Английские материалы / Electrophysiology of Vision_Lam_2005
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Early in CAR, ERG is markedly impaired even when the retina appears normal and, therefore, is a key diagnostic test (Fig. 13.5). Virtually, all ERG parameters including scotopic and photopic a-wave and b-wave responses are severely diminished and prolonged early in the disease even when the retina appears normal (53,54). With further progression of the disease, the ERG responses become non-detectable. The EOG and VEP findings parallel ERG responses. For instance, pattern VEP is also severely impaired early in the disease secondary to the retinal dysfunction (53).
Recoverin-Associated Retinopathy and
Autoimmune Retinopathy without Cancer
Not all patients with retinopathy associated with autoantibodies to recoverin are found to have cancer and, therefore, do not have cancer-associated retinopathy (55). Whitcup et al.
Figure 13.5 Examples of full-field ERG responses in cancer-asso- ciated retinopathy (CAR) and melanoma-associated retinopathy (MAR). The ERG responses are impaired early in CAR even when the retina appears normal. The ERG responses in MAR are similar to those of congenital stationary night blindness.
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(56) reported a patient with recoverin autoantibodies with similar findings as cancer-associated retinopathy but no cancer was found after 3 years of investigation. Heckenlively et al. (57) documented antirecoverin immunoreactivity in 10 patients with retinitis pigmentosa and no systemic malignancy (57). This suggests that autoimmune retinopathy may have clinical features similar to retinitis pigmentosa or that in some retinitis pigmentosa cases, there may be an autoimmune component exacerbating the underlying disease. A diagnosis of autoimmune retinopathy without cancer should be made with caution as cancer-associated retinopathy may occur before the diagnosis of cancer, and occult malignancy may be present in patients with autoimmune retinopathy. Regardless, the ERG findings in recoverin-associated retinopathy are similar to those in cancer-associated retinopathy with early impairment of ERG components even when the retina appears normal.
Melanoma-Associated Retinopathy
Melanoma-associated retinopathy (MAR) is a paraneoplastic disorder that occurs usually in patients with an established diagnosis of cutaneous melanoma. Visual symptoms include flickering shimmering photopsias, nyctalopia, and diminished vision. The symptoms progress over months and are typically but not always associated with metastatic disease. As in cancer-associated retinopathy, the retina in MAR may have a normal appearance in the early stage of the disease.
In MAR, ERG is a key diagnostic test not only because it is impaired early in the disorder even when the retina appears normal but also because of its characteristic pattern of selective impaired b-wave (Fig. 13.5) (58–60). Patients with MAR are found to have the following on standardized full-field ERG: (1) scotopic rod flash response—reduced and prolonged, (2) scotopic combined rod–cone bright flash response—mildly reduced a-wave with a marked selective decrease in the b-wave amplitude resulting in a negative pattern (i.e., b-wave to a-wave amplitude ratio <1.0 with the peak of the b-wave not reaching baseline), (3) oscillatory
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potentials—reduced, (4) photopic cone flash response—nor- mal or mildly reduced, and (5) photopic cone flicker response—normal in amplitude with or without mildly prolonged. Focal foveal ERG may be reduced and prolonged depending on foveal function (60).
Taking together, the full-field ERG responses in MAR are similar to those of congenital stationary night blindness (58). Dysfunction of the on-response in MAR has been found with specialized ERG techniques, consistent with the finding on immunofluorescence microscopy that human retinal bipolar cells are bound by labeled antibodies from serum of MAR patients, thus implicating that bipolar cells may be the targets of the paraneoplastic antibody in MAR (60,61).
CRMP-5 Paraneoplastic Retinopathy and
Optic Neuropathy
Paraneoplastic autoantibody specific for type 5 collapsin response-mediator protein (CRMP-5), a neuronal cytoplasmic protein expressed in central and peripheral neurons, may be associated with bilateral vitritis, retinopathy, and optic neuropathy (62). Of the 116 CRMP-5-seropositive patients reported by Yu et al. (63), lung carcinoma, mostly small-cell, was found in 77% and thymoma in 6%. Among CRMP- 5-seropositive patients, multifocal neurologic signs are common, but bilateral retinopathy and optic neuropathy may occur as the predominant features of this paraneoplastic syndrome (62). Both the scotopic and the photopic full-field ERG responses may be impaired in this condition (62).
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14
Ocular Vascular Disorders
The electrophysiologic findings of ocular vascular disorders caused by vascular occlusion and neovascular proliferation are summarized in this chapter. The ERG is particularly useful in determining retinal damage from vascular occlusion when acute clinical signs are no longer present (Fig. 14.1). In addition, the ERG is helpful to assess the risk of neovascular development in central retinal vein occlusion. Retinal vasculitis disorders such as Behc¸et disease are discussed in Chapter 13. The outline of this chapter is as follows:
Vascular occlusions:
Ophthalmic artery occlusion
Central retinal artery occlusion
Branch retinal artery occlusion
Central retinal vein occlusion
Branch retinal vein occlusion
Other proliferative neovascular disorders:
Retinopathy of prematurity
Diabetes retinopathy
Sickle cell retinopathy
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