[CAR syndrome (carcinoma-associated retinopathy syndrome) associated with an adenocarcinoma of the cervix].
BACKGROUND
CAR is a rare paraneoplastic syndrome. It is most commonly associated with small-cell carcinoma of the lung. Specific autoantibodies exist not only against the retina but also against other ocular structures. They induce apoptotic death of retinal photoreceptor cells.


METHODS
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 in the year 1998.


RESULTS
We found a visual acuity of 0.1 in both eyes and concentric visual field defects. The scotopic and photopic electroretinogram was markedly reduced. Uveitis intermedia 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 patient's 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. The patient was treated with corticosteroid pulse therapy. The follow-up showed stable findings.


CONCLUSIONS
In patients with progressive visual loss, concentric visual field defects and pathological electroretinogram as well as evidence of uveitis, 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.
Seles
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Augenklinik der Universität Ulm. sseles@web.de
Lang
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D000230:Adenocarcinoma / Q000175:diagnosis*; D000368:Aged; D005260:Female; D006801:Humans; D020364:Paraneoplastic Polyneuropathy / Q000175:diagnosis*; D012174:Retinitis Pigmentosa / Q000175:diagnosis*; D013577:Syndrome; D002583:Uterine Cervical Neoplasms / Q000175:diagnosis*; D015867:Uveitis, Intermediate / Q000175:diagnosis*; D014786:Vision Disorders / Q000175:diagnosis*