A 60-year-old man presented with vitritis and optic neuropathy in the setting of headaches and behavioral changes. MRI brain revealed bilateral temporal lobe inflammation consistent with limbic encephalitis. He was subsequently diagnosed with small cell lung cancer with a paraneoplastic syndrome characterized by CRMP5 IgG as a cause of his symptoms. His visual symptoms improved markedly after anti-inflammatory therapy and his cognitive symptoms were mildly better following systemic chemotherapy. The clinical presentation, pathophysiology, and therapy of CRMP5 associated paraneoplastic syndromes are discussed.
1Division of Neuro-Ophthalmology, Departments of Neurology and Ophthalmology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
2Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
Reprint address: Heather E. Moss, MD, PhD, Department of Neurology, Hospital of the University of Pennsylvania, 3400 Spruce St., Philadelphia, PA 19104.
The authors wish to thank Dr. Darma Ie for performing the retina evaluation, biopsying the vitreous, and providing the fundus photos. The authors reported no proprietary or commercial interest in any product mentioned or concept discussed in this article.
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