A 71-year-old woman had development of generalized fatigue over 1 week, along with low-grade fever. The fever resolved, but the fatigue persisted. Subsequently, retro-orbital and head discomfort developed. One month later, she had blurred vision. An ophthalmic examination revealed mild dyschromatopsia, bilateral visual field constriction, bilateral marked optic disc edema, and vitreous cells graded as vitreous haze score 2.0.
Magnetic resonance imaging of the brain showed confluent abnormal areas of T2 hyperintensity without mass effect or enhancement involving the subcortical and periventricular white matter in the cerebral hemispheres bilaterally, basal ganglia, pons, and left thalamus. She had 2 lumbar punctures, which showed normal opening pressures. Cerebrospinal fluid analysis showed an increased white blood cell count cytologically consistent with reactive pleocytosis with a predominance of lymphocytes. The cerebrospinal fluid protein level was increased and cultures were negative for organisms. Immunostain confirmed polyclonal plasma cells and a possible T-cell proliferative disorder. The cerebrospinal fluid and serum were positive for collapsin-response mediator protein 5-immunoglobulin G and microtubule-associated protein 1B- immunoglobulin G antibodies at high titers. Computed tomography of the chest, abdomen, and pelvis showed an indeterminate pulmonary nodule in the upper lobe. Bronchoscopy identified thickened mucosa in the right lower lung consistent with small cell carcinoma. Positron emission tomography showed abnormal hypermetabolic areas of the ascending colon. Biopsy revealed tubulovillous adenoma of the ascending colon, and the patient underwent a right-sided colon resection and anastomosis.
The patient was diagnosed with paraneoplastic optic neuropathy (collapsin-response mediator protein 5-immunoglobulin G–associated optic neuropathy and vitritis).
Intravenous methylprednisolone was given for 5 days, followed by a prolonged course of oral prednisone, with slight visual improvement. The patient underwent a right-sided thoracotomy with biopsy of the right lower lobe, the results of which were consistent with small cell undifferentiated carcinoma.
Paraneoplastic neurologic syndromes are a heterogeneous group of disorders associated with various systemic cancers and with other mechanisms believed to be immune mediated. Paraneoplastic visual syndromes can precede or follow a diagnosis of cancer.
A case of paraneoplastic optic neuropathy and outer retinitis positive for autoantibodies against collapsin response mediator protein-5, recoverin, and α-enolase
