Abstract
PurposeTo report the clinical, electrophysiologic and immunopatholologic findings in a patient with progressive visual loss due to retinal and optic nerve degeneration associated with a Waldenström's macroglobulinemia (WM).MethodsA 77‐year old man with mild stage WM, complained of progressive bilateral visual loss, with photopsia, intense photophobia and loss of color perception. Patient developed severe legs pain and unsteadiness.Complete ophthalmological, neurological and hematological examinations were realized, and patient followed over a period of 2 years. Immunohistochemical studies to determine the presence of serum antiretinal antibodies were performed.ResultsVisual acuity was gradually reduced to 1/10 RE and 2/10 LE, with a severe loss of color perception. The visual field showed a deep central and peripheral visual loss in both eyes. Optic nerves were pale and excavated, macular remained normal as peripheral retina. Cerebral CT scan did not show anomalies in the brain as in the optic nerves, lumbar puncture was unremarkable. Neurological revealed a rest less leg syndrome. Electroretinogram showed a reduce rod response and a delayed combined and cone response. Hematologic evaluations confirmed the WM and immunohistochemistry showed reactivity of the patient's serum against the photoreceptors.The patient was treated with 4 intravenous infusions of Rituximab, followed 1 month later by 1gr of Methylprednisolone for 3 days. Improvement of central vision (4/10 RE, 6/10 LE) and peripheral visual fields was reported.ConclusionsThe visual loss in this patient is due to a combined dysfunction in the retina and the optic nerve and presumed to result from antibodies IgM‐subtype reacting to proteins in different retinal antigens.
Published Version
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