Paraneoplastic autoimmune optic neuritis with retinitis defined by CRMP-5-IgG

被引:177
作者
Cross, SA
Salomao, DR
Parisi, JE
Kryzer, TJ
Bradley, EA
Mines, JA
Lam, BL
Lennon, VA
机构
[1] Mayo Clin & Mayo Grad Sch Med, Dept Neurol, Rochester, MN 55905 USA
[2] Mayo Clin & Mayo Grad Sch Med, Dept Lab Med & Pathol, Rochester, MN 55901 USA
[3] Mayo Clin & Mayo Grad Sch Med, Dept Immunol, Rochester, MN 55901 USA
[4] Mayo Clin & Mayo Grad Sch Med, Dept Ophthalmol, Rochester, MN 55901 USA
[5] Bay Area Retinal Consultants, Clearwater, FL USA
[6] Bascom Palmer Eye Inst, Miami, FL 33136 USA
关键词
D O I
10.1002/ana.10587
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Autoantibodies have defined two paraneoplastic visual disorders related to small-cell. lung carcinoma: retinopathy ("CAR"-IgG [23kDa, recoverin]) and optic neuritis (collapsin response-mediator protein-5 [CRMP]-IgG [62kDa]). Among 16 patients with CRMP-5-IgG and optic neuritis (aged 52-74 years; all smokers, 9 women), we documented coexisting retinitis in 5. None had CAR-IgG. Fifteen had subacute vision loss, swollen optic discs, and field defects. Vascular leakage was evident at and remote from the disc; 5/5 tested had abnormal electroretinograms. Nine had striking vitreous cells. Vitrectomy showed reactive lymphocytosis (4/4), predominantly CD4(+) (1/1). Most patients had multifocal neurological accompaniments. Cerebrospinal fluid contained lymphocytes (7-32), elevated protein, multiple oligoclonal immunoglobulin bands, and CRMP-5-IgG. Three patients superficially resembled Devic's disease at presentation. One autopsied patient had predominantly CD8(+) T lymphocytes infiltrating optic nerve and spinal cord. Eleven patients had confirmed small-cell. carcinoma; I had imaging evidence of lung cancer, 3 had renal or thyroid carcinoma. Full-length CRMP-5 protein was identified in normal retina and optic nerve by Western blot analyses. Photoreceptor cells, retinal ganglion cells, and nerve fibers exhibited CRMP-5-specific immunoreactivity. In summary, CRMP-5-IgG defines a paraneoplastic ophthalmological entity of combined optic neuritis and retinitis with vitreous inflammatory cells. Positive serology obviates the need for vitreous biopsy and expedites the search for cancer.
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页码:38 / 50
页数:13
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