Abstract

Treatments for paraneoplastic optic neuropathy (PON), a tumor-related autoimmune disease, include immunosuppression, plasma exchange, and immunoglobulin therapies, as well as treatment of the underlying disease. Herein, we describe the clinical course of an older adult patient with PON whose loss of vision improved after switching between epidermal growth factor receptor-tyrosine kinase inhibitor (EGFR-TKI) treatments for cancer. A 76-year-old woman, who had been treated with gefitinib for lung adenocarcinoma for two years, presented with acute bilateral visual disturbances. Her decimal best-corrected visual acuity (BCVA) was 0.3 in the right eye (RE) and 0.7 in the left eye (LE). Slit-lamp examination and funduscopy showed no abnormal findings. Two weeks later, her BCVA decreased to 0.2 in the RE and 0.01 in the LE. Goldman's perimetry showed a defect in the lower nasal RE and extensive visual-field loss in the LE. Single-flash electroretinograms showed normal amplitudes. Magnetic resonance imaging revealed left optic neuritis and showed neither metastatic cancer nor multiple sclerosis. Pattern-reversal visual evoked potentials showed decreased P100 amplitudes in both eyes (BE). Based on a diagnosis of PON from clinical findings, methylprednisolone pulse treatment was administered. However, her BCVA became no light perception in BE two months after the first visit. Because the tumor tissue was found to be positive for the EGFR T790M resistance mutation by bronchoscopy, the EGFR-TKI treatment was changed to osimertinib, decreasing the size of the lung cancer lesions. Her BCVA improved to hand motion in BE. Her final BCVA was 0.01 in the RE, counting fingers 10 cm in the LE. She died at the age of 79 years. To our knowledge, no reports have shown improvement in BCVA in patients with PON after changing EGFR-TKI treatments. This report indicates that some patients may develop severe visual dysfunction without early treatment for the primary tumor.

Highlights

  • Paraneoplastic optic neuropathy (PON) is one of several tumor-related autoimmune diseases, such as cancerassociated retinopathy (CAR) and melanoma-associated retinopathy (MAR) [1, 2]

  • We describe the clinical course of an older adult patient diagnosed with and treated for PON associated with lung adenocarcinoma

  • We describe a rare case of clinically diagnosed PON associated with lung adenocarcinoma in which visual acuity was initially improved after switching between EGFR-TKI treatments, but resulted in severe loss of visual acuity

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Summary

Introduction

Paraneoplastic optic neuropathy (PON) is one of several tumor-related autoimmune diseases, such as cancerassociated retinopathy (CAR) and melanoma-associated retinopathy (MAR) [1, 2]. Malignant tumors, such as small-cell lung cancer and malignant lymphoma, are known to cause PON [1, 2], but it can be caused by benign tumors, such as choroid meningioma [3]. In some patients with autoimmune optic neuropathies, visual function can be improved with corticosteroid treatment, and overall visual prognosis is better compared to those of CAR and MAR [1]. We describe the clinical course of an older adult patient diagnosed with and treated for PON associated with lung adenocarcinoma

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