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Clinical challenges

Second chances Lina Nagia, DOa,*, Jennifer I. Doyle, MDa, James R. Hackney, MDb, Michael S. Vaphiades, DOa, Lanning B. Kline, MDa, Marc H. Levin, MD, PhDc a

Department of Ophthalmology, School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA b Department of Pathology, School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA c Department of Ophthalmology, School of Medicine, University of California, San Francisco, San Francisco, California, USA

article info

(In keeping with the format of a clinical pathological conference, the abstract and key words appear at the end of the article.)

Article history: Received 8 June 2015 Accepted 8 June 2015 Peter Savino and Helen DaneshMeyer, Editors

1.

Case report

An 81-year-old woman had a 1-month history of blurred vision in the left eye that had acutely worsened 5 days before presentation. She experienced dull pain with left gaze, leftsided forehead tenderness, and mild weight loss. Her medical history was significant for hypertension, diet-controlled diabetes mellitus, a cerebrovascular accident, and basal cell carcinoma of the face. Medications included rivaroxaban, metoprolol, atorvastatin, and omeprazole. Visual acuity was 20/20 in the right eye, and she had only light perception in the left eye. There was a left relative afferent pupillary defect, and color vision and visual field testing were normal in the right eye. Eye movements were full, and the anterior segment examination was unremarkable. The right fundus was normal, whereas the left showed pallid optic disk edema with associated flame-shaped hemorrhages (Fig. 1). What would you do next?

2.

Comments

2.1.

Comments by Marc Levin, MD, PhD

In this elderly woman presenting with profound vision loss, headache, and pallid disk edema with nerve fiber layer hemorrhages, the suspicion is highest for anterior ischemic optic neuropathy from giant cell arteritis (GCA). The previously mentioned constellation places nonarteritic anterior ischemic optic neuropathy much lower on the differential. Despite the pain on eye movements, papillitis from demyelinating optic neuritis is unlikely given her advanced age and the presence of hemorrhages. Yet, the spectrum of neuromyelitis optica continues to broaden, and neuromyelitis optica can at least be considered here. Given the high suspicion for GCA, I would immediately initiate high-dose systemic corticosteroids, either orally or intravenously, and arrange for ipsilateral superficial temporal artery biopsy to take place within 1 week. This plan would not

The authors report no proprietary or commercial interests or conflicts. * Corresponding author: Lina Nagia, DO, Callahan Eye Hospital, 700 18th Street South, Suite 601, Birmingham, AL 35233, USA. E-mail address: [email protected] (L. Nagia). 0039-6257/$ e see front matter ª 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.survophthal.2015.06.002

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s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 5 ) 1 e4

the initial workup for GCA, optic nerve sheath and adjacent fat enhancement are observed with increasing frequency in cases of biopsy-proven GCA. In parallel, I would obtain a lumbar puncture. Cerebrospinal fluid cytology and flow cytometry should be performed in search of clonal malignant cells. A lymphocytic pleocytosis would be in itself nonspecific and consistent with GCA, as well as sarcoidosis, syphilis, Lyme disease, and tuberculosis. Although of relatively low yield in an elderly patient, I would investigate possible causes of atypical autoimmune optic neuropathy by sending serum ACE, ANA, SS-A/SS-B, ANCA, and neuromyelitis optica-IgG.

Fig. 1 e Left fundus shows pallid disk edema with peripapillary hemorrhages.

be influenced by the precise serum inflammatory marker values, although markedly elevated erythrocyte sedimentation rate or C-reactive protein level might lead one to consider biopsy of the contralateral side if the initial biopsy were negative.

3.

Case report (continued)

Erythrocyte sedimentation rate was 36 mm/hr and C-reactive protein level was 5.4 mg/L (normal,

Second chances.

An 81-year-old woman presented with light perception vision in her left eye and had pallid swelling of the left optic disk. Temporal artery biopsy was...
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