| Identifier |
walsh_2018_s2_c6 |
| Title |
Triple Take - Video |
| Creator |
Johanna Beebe; Liang Cheng; Shira Simon; Michael Wall; Randy Kardon; Alkapalan Deema; Ian Han; Matthew Thurtell |
| Affiliation |
(JB) Park Nicollet, Department of Ophthalmology, St. Louis Park, Minnesota, USA; (LC) University of Iowa Carver College of Medicine, Iowa City, Iowa, USA; (SS) (MW) (RK) (IH) (MT) University of Iowa, Department of Ophthalmology, Iowa City, Iowa; (AD) University of Iowa, Department of Pathology, Iowa City, Iowa |
| Subject |
autoimmune reinopathy, Optic Neuropathy, Paraneoplastic, Special staining, Electroretinogram |
| Description |
On admission, his creatinine was noted to be elevated to 3.8 mg/dL. A subsequent renal ultrasound demonstrated adult polycystic kidney disease and a large mass arising from the right kidney, raising concern for neoplastic disease and a paraneoplastic syndrome as the cause for his vision loss. Biopsy of his renal mass showed a benign renal oncocytoma. A full-body PET-CT scan showed no other areas of hypermetabolic activity other than the known right renal mass. ERG demonstrated severe cone greater than rod dysfunction. Anti-retinal and optic nerve antibody testing showed auto-antibodies to multiple proteins, including alpha- and gamma-enolase. Immunohistochemical staining of the oncocytoma biopsy specimen showed positive alpha- and gamma-enolase staining throughout the sample. Ultimately, the unifying diagnosis was a paraneoplastic autoimmune retinopathy and optic neuropathy secondary to a renal oncocytoma. The patient's treatment involved IV corticosteroids, plasmapheresis, and complete excision of the renal mass with an associated partial nephrectomy. With treatment, he has gained a significant improvement in his vision. At his most recent follow-up visit, his visual acuities were 20/125 OD and 20/200 OS, with improved bilateral central scotomas. |
| History |
A 42 year-old male noticed difficulty reading labels while stocking shelves at work. Over the next three weeks, he developed a dark 'cloud' in his central vision in the left eye, and then the right eye. He did not have associated pain. When evaluated by the retina service, his visual acuities were 20/100 OD and 20/1250 OS without a relative afferent pupillary defect. Goldmann perimetry showed central scotomas OU. His anterior segment was unremarkable. Dilated fundus examination showed subtle telangiectatic vessels on the optic nerve head and in the macula OU. Optical coherence tomography (OCT) showed diffuse inner retinal thinning with focal irregularities in the outer plexiform layer in the macula OU, as well as ganglion cell layer thinning OU. He was evaluated; in neuro-ophthalmology for possible Leber's hereditary optic neuropathy. Repeat visual field, dilated fundus examination, and OCT findings were unchanged from previously. MRI brain and orbits with and without contrast showed subtle bilateral optic nerve enhancement. He was thought to have bilateral optic neuritis. He was admitted for further work-up and management. CSF evaluation showed a minimally elevated protein concentration of 48 mg/dL, with normal constituents otherwise. CSF cytology was negative. NMO antibodies were not detected and a paraneoplastic panel was negative. Further diagnostic testing was performed. |
| Disease/Diagnosis |
Paraneoplastic autoimmune retinopathy and optic neuropathy secondary to a renal oncocytoma |
| Presenting Symptom |
A 42 year-old male noticed difficulty reading labels while stocking shelves at work. Over the next three weeks, he developed a dark 'cloud' in his central vision in the left eye, and then the right eye. He did not have associated pain. When evaluated by the retina service, his visual acuities were 20/100 OD and 20/1250 OS without a relative afferent pupillary defect. Goldmann perimetry showed central scotomas OU. His anterior segment was unremarkable. Dilated fundus examination showed subtle telangiectatic vessels on the optic nerve head and in the macula OU. Optical coherence tomography (OCT) showed diffuse inner retinal thinning with focal irregularities in the outer plexiform layer in the macula OU, as well as ganglion cell layer thinning OU. He was evaluated; in neuro-ophthalmology for possible Leber's hereditary optic neuropathy. Repeat visual field, dilated fundus examination, and OCT findings were unchanged from previously. MRI brain and orbits with and without contrast showed subtle bilateral optic nerve enhancement. He was thought to have bilateral optic neuritis. He was admitted for further work-up and management. CSF evaluation showed a minimally elevated protein concentration of 48 mg/dL, with normal constituents otherwise. CSF cytology was negative. NMO antibodies were not detected and a paraneoplastic panel was negative. Further diagnostic testing was performed. |
| Date |
2018-03 |
| References |
1. Fox, A., H.N. Sen, and R. Nussenblatt, Autoimmune Retinopathies, in Ryan's Retina. 2018, Elsevier. p. 1562-1571. 2. Golovastova, M.O., et al., The cancer-retina antigen recoverin as a potential biomarker for renal tumors. Tumour Biol, 2016. 37(7): p. 9899-907. 3. Wu A. Oncocytic Renal Neoplasms on Resections and Core Biopsies: Our Approach to This Challenging Differential Diagnosis. Arch Pathol Lab Med 2017 Oct;141(10):1336-1341. doi: 10.5858/arpa.2017-0240-RA. |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Source |
2018 North American Neuro-Ophthalmology Society Annual Meeting |
| Relation is Part of |
NANOS Annual Meeting 2018 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2018. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6pw0nz3 |
| Setname |
ehsl_novel_fbw |
| ID |
1320237 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6pw0nz3 |
