Glazed (vision) and confused.
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.
Moss
Heather E
HE
Division of Neuro-Ophthalmology, Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA. Heather.Moss@uphs.upenn.edu
Liu
Grant T
GT
Dalmau
Josep
J
D000970:Antineoplastic Agents / Q000627:therapeutic use; D003072:Cognition Disorders / Q000175:diagnosis; D004569:Electroencephalography; D005128:Eye Diseases / Q000175:diagnosis*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006867:Hydrolases; D007074:Immunoglobulin G / Q000097:blood; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D008869:Microtubule-Associated Proteins; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000276:immunology; D009901:Optic Nerve Diseases / Q000175:diagnosis*; D010257:Paraneoplastic Syndromes / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D055752:Small Cell Lung Carcinoma / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D014057:Tomography, X-Ray Computed; D014822:Vitreous Body / Q000473:pathology*
D000970:Antineoplastic Agents; D005938:Glucocorticoids; D007074:Immunoglobulin G; D008869:Microtubule-Associated Proteins; D009419:Nerve Tissue Proteins; C415087:DPYSL5 protein, human; D006867:Hydrolases