gms | German Medical Science

CAR is a rare paraneoplastic syndrome. It is most commonly associated with the small-cell carcinoma of the lung. Specific autoantibodies exist against the retina but also against other ocular structures. These are antibodies against the photoreceptor protein recoverin or against alpha-enolase, but other unknown autoantibodies play a role in the pathogenesis, too. They induce apoptotic death of retinal photoreceptor cells and lead to a visual loss or blindness.

We report about a 68-year-old patient, who presented in February 2003 with progressive visual dysfunction including visual acuity loss and visual field defects of unknown origin, which first manifested itself in the year 1998.

We found a visual acuity of 0.1 in both eyes, a concentric visual field defect and a central scotoma in the right eye. The scotopic and photopic electroretinogram was markedly reduced. Retinal vasculitis in both eyes, narrowing of the vessels, especially of the arterioles, optic atrophy and window defects in the retinal pigment epithelium were observed on fluorescein angiography. Due to the patients report about a resection of an adenocarcinoma of the cervix in June 2002, she was diagnosed as having CAR-syndrome. Serologic screening by Western blot analysis revealed different specific autoantibodies against retinal proteins (60 kDa, 30 kDa, 23 kDa). The patient was treated with corticosteroid pulse therapy starting with 150 mg and was maintained on 6 mg. The follow-up showed stable findings.

In patients with progressive visual loss, concentric visual field defects and pathologic electroretinogram as well as evidence of retinal vasculitis, a CAR-syndrome has to be ruled out. One has to take into consideration, that visual dysfunction can appear before the primary cancer is diagnosed.