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M.J. Leys, S. Tate, B. Ellis, J.V. Odom; Occult Macular Dystrophy in Patients with Serum Immunogammopathy . Invest. Ophthalmol. Vis. Sci. 2003;44(13):523.
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Purpose: To describe results in two patients with binocular unexplained central visual loss who after multiple investigations were diagnosed with monoclonal gammopathy. Methods: Electroculography, electroretinography, multifocal electroretinography and 10 Hz VEP were performed in two patients with bilateral paracentral scotomas. Additionally, neuroimaging studies, fluorescein angiography,extensive labs on serum and spinal fluid were performed. Results: Visual acuity was 20/200 OU in a patient with multiple myeloma. Visual acuity was 20/20 OD, 20/30 OS in a patient with benign monoclonal gammopathy. The EOG Light/Dark ratios were normal. In the first patient, serial ganzfeld ERGs were decreased in dark and light and prolonged in the dark. ERGs became more abnormal with time in the first patient. In the second patient, light adapted ERGs were delayed. Multifocal ERGs were very abnormal in both eyes of both patients. Ten Hz flash VEPs were normal. The visual function test results in both patients were consistent with outer retinal dysfunction as in "occult macular dystrophy". Neuroimaging tests were normal and fundus fluorescein angiograms nearly normal. Labs of serum and spinal fluid were normal, including serum viscosity. No CAR antibodies or Leber's Hereditary Optic Neuropathy mutations were detected. Monoclonal IgG kappa was found in the first patient who consequently was diagnosed with multiple myeloma. Monoclonal IgG lambda was detected in the second patient. Conclusion: Our two patients with monoclonal gammopathy could easily be assumed to have occult macular dystrophy. Occult macular dystrophy is a diagnosis of exclusion. Therefore, when patients present without a family history of vision loss they should have an extensive evaluation including protein electrophoresis.
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