Sempere et al. Journal of Medical Case Reports 2011, 5:319 http://www.jmedicalcasereports.com/content/5/1/319 C A S E R E P O R T JOURNAL OF MEDICAL CASE REPORTS Open Access Bilateral optic neuritis in a 26-year-old man with common variable immunodeficiency: a case report Angel P Sempere1*, ML Tahoces2, Susana Palao-Duarte1 and Alfonso Garcia-Perez2 Abstract Introduction: Common variable immunodeficiency encompasses a group of heterogeneous conditions linked by a lack of immunoglobulin production and primary antibody failure. Although primary immunodeficiencies are typically characterized by recurrent infections, autoimmune manifestations have increasingly been recognized. Neurological complications are extremely rare and to the best of our knowledge optic neuritis has not been described previously. We report the case of a patient with common variable immunodeficiency who developed loss of vision secondary to bilateral optic neuritis. Case presentation: A 26-year-old Caucasian man with a diagnosis of common variable immunodeficiency presented to our facility with loss of vision secondary to bilateral optic neuritis. Results of a thorough study for infectious, neoplastic and autoimmune diseases were negative. Our patient was treated with intravenous methylprednisolone with almost complete improvement and he remained asymptomatic at a 12-month follow-up. Conclusions: Bilateral optic neuritis should be added to the list of autoimmune disorders related to common variable immunodeficiency. If a patient with common variable immunodeficiency experiences loss of vision, the possibility of bilateral optic neuritis should be considered as rapid initiation of high-dose corticosteroids may improve visual recovery. Introduction Common variable immunodeficiency (CVID) encom- passes a group of heterogeneous conditions linked by a lack of immunoglobulin production and primary anti- body failure. Although primary immunodeficiencies are typically characterized by recurrent infections, autoim- mune manifestations have increasingly been recognized [1]. The commonest autoimmune conditions in CVID are hematologic cytopenias. Neurological complications are extremely rare [2] and to the best of our knowledge optic neuritis has not been described previously. We report the case of a patient with CVID who developed loss of vision secondary to bilateral optic neuritis. Case presentation A 26-year-old man with a diagnosis of CVID presented to our facility with loss of vision in both eyes. The first manifestation of CVID was hemolytic anemia at the age * Correspondence: aperezs@mac.com 1Neurology Department, Hospital General Universitario de Alicante, Alicante, Spain Full list of author information is available at the end of the article of 14, when the diagnosis was made. Later, he experi- enced several episodes of autoimmune thrombocytope- nia and neutropenia. Since diagnosis, his only serious infectious complication had been an episode of salmo- nella sepsis at the age of 23. Actual treatment included intravenous immunoglobulin (0.4 g/kg every three weeks) and prednisone (10 mg daily). On presentation, our patient reported progressive loss of vision that had begun five days earlier. He also had mild pain with eye movement. His visual acuity was 0.1 with reduced color perception, and both pupils showed a poor response to light stimulation. A confrontational visual field examination showed a diffusely decreased visual field in both eyes. Results of a slit-lamp examina- tion were negative for uveitis. On funduscopic examina- tion, his optic disk appeared normal. He was afebrile, and the results of general physical and neurological examina- tions were normal. There was no family history of eye disorders. An MRI of the brain, orbit and spine revealed a mild enhancement of both optic nerves without other abnormalities. Pattern-shift visual evoked potentials © 2011 Sempere et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.