Papillitis, a Rare Cytomegalovirus Manifestation in an Immunocompetent Host

Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Papillitis, a Rare Cytomegalovirus Manifestation in an Immunocompetent Host Timothy N. O’Brien, BPharm, MPharm, MB, BCh, BAO, MRCPI, Gerard M. O’Connor, FRCS, FRCOphth, Stela Lefter, MD, MRCPI Downloaded from http://journals.lww.com/jneuro-ophthalmology by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC1y0abggQZXdgGj2MwlZLeI= on 05/04/2022 V ision-threatening cytomegalovirus (CMV) infections are most often diagnosed in immunocompromised patients, such as those with AIDS, but rarely it can cause an optic neuropathy in immunocompetent individuals. A 43-year-old Italian man presented with painless blurred vision and flashing lights in the periphery of his right eye progressively deteriorating over 10 days. This complaint arose in the context of a flu-like illness featuring a nonproductive cough, rhinorrhea, and fevers. Past medical history included hospitalization for pneumonia 6 months previously. Otherwise, there was a remote history of migraines with visual aura and corrective surgery for strabismus in the right eye as a child. He worked in an office for an IT company and was married, with a 2-year-old daughter who was also suffering from similar viral symptoms. He smoked 5 cigarettes per day and drank 6 units of alcohol per week. He denied any illicit drug use or sexual infidelity. There was no family history of ophthalmological or neurological disorders. On examination he was systemically well with an elevated BMI. His temperature was 38.4 °C and he had a resting sinus tachycardia of 120 bpm. Cardiac, respiratory, and gastrointestinal examinations were unremarkable. General inspection of both eyes was normal. There was a deficit in the inferior temporal quadrant and acuity was reduced to 6/9. A mild right-sided relative afferent pupillary defect was detected. Eye movements were normal. Retinal photography revealed optic disc edema and disc hemorrhages in the right eye (Fig. 1). Formal visual field assessment revealed a right eye inferior quadrant deficit (See Supplemental Digital Content 1, Figure E1, http://links.lww.com/ WNO/A393). Examination of the left eye and remainder of the neurological examination were normal. All laboratory results are presented in Supplemental Digital Content 2 (See Table E1, http://links.lww.com/WNO/ A394). Initial blood results demonstrated an elevated white cell count with lymphocytosis and raised C-reactive protein. Liver function tests were mildly deranged. Viral serology was positive for CMV immunoglobulin M (IgM) and immunoglobulin G (IgG), whereas polymerase chain reaction (PCR) confirmed active CMV infection (2,334 copies/mL). Other infectious etiologies including HIV were ruled out. Cerebrospinal fluid analysis revealed a mildly elevated protein level, but was negative for CMV DNA and oligoclonal bands. MRI of the brain and orbits was normal with no white matter inflammatory lesions. A chest X-ray was normal. Because of the persistent sinus tachycardia, a computed tomography pulmonary angiogram was performed and ruled out a pulmonary embolism. Once acute CMV infection was confirmed, the patient was discharged on oral valganciclovir 900 mg twice daily for 3 weeks and prednisolone eye drops for 7 days after consultation with Infectious Disease and Ophthalmology specialists respectively. Steroids were not administered systemically. Four weeks postdischarge at outpatient follow-up, the patient reported a resolution of visual Departments of Neurology (TNO, SL), and Ophthalmology (GMO), Cork University Hospital, Wilton, Cork, Ireland. The authors report no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Web site (www. jneuro-ophthalmology.com). Address correspondence to Timothy N. O’Brien, BPharm, MPharm, MB, BCh, BAO, MRCPI, Department of Neurology, Cork University Hospital, Wilton, Cork, Ireland; E-mail: [e-mail] e34 FIG. 1. Funduscopic appearance of the right eye at presentation. O’Brien et al: J Neuro-Ophthalmol 2021; 41: e34-e35 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence FIG. 2. Funduscopic appearance of the right eye after completing antiviral treatment at 4-week follow-up. symptoms with concomitant improvement in funduscopic appearance (Fig. 2) and visual field defect (See Supplemental Digital Content 1, Figure E1, http://links.lww.com/ WNO/A393). Repeat serology 3 months postdischarge was negative for CMV IgM and remained positive for CMV IgG, whereas his repeat CMV PCR demonstrated a reduced viral load (,500 copies/mL). He underwent evaluation with an Immunology specialist and no immunodeficiency was identified. One year postdischarge, at ophthalmology follow-up, he had minimal residual visual field deficit and remained clinically well. CMV infection produces a spectrum of human illness and is mostly dependent on the host. Ocular CMV infections are typically observed in immunocompromised patients manifesting as retinitis that can progress to permanent blindness. In our case, the temporal association with and physical proximity to his young daughter, who was suffering from a viral illness, lead to the hypothesis that this patient was exposed to a strain of CMV to which he was not immune. Young children have previously been shown to transmit CMV to their parents (1). Cases of isolated CMV papillitis remain rare with just a handful of published case reports. In 2008, De Silva et al (2) described a 22-year-old woman presenting with an O’Brien et al: J Neuro-Ophthalmol 2021; 41: e34-e35 infectious-mononucleosis syndrome and visual loss secondary to bilateral papillitis that responded well to intravenous ganciclovir. In 2012, Chang et al (3) reported a 33-year-old woman suffering bilateral eye pain, headaches, and tinnitus, who was subsequently diagnosed with bilateral papillitis and again successfully treated with oral valganciclovir. As in our case, in these 2 previous cases, the diagnosis was made indirectly with positive serology and a positive serum PCR for viral DNA as opposed to intraocular fluid analysis which is invasive and may not be necessary in establishing the diagnosis. In addition, both previously reported cases had a complete response to antiviral treatment as observed in our patient. Our case is different, because it reported unilateral papillitis that responded to oral antiviral treatment. Serum angiotensin converting enzyme (ACE) was raised in our patient; however, in the absence of clinical or radiological evidence of sarcoidosis, this finding is considered nonspecific. ACE can increase in both infectious and inflammatory conditions, but the underlying cause of this phenomenon remains unknown. Maintaining a high index of clinical suspicion for causes of optic papillitis other than demyelination or autoimmunity is paramount. Prompt diagnosis and treatment of CMV papillitis can prevent irreversible visual loss. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: T. N. O’Brien and S. Lefter; b. Acquisition of data: T. N. O’Brien and S. Lefter; c. Analysis and interpretation of data: T. N. O’Brien and S. Lefter. Category 2: a. Drafting the manuscript: T. N. O’Brien; b. Revising it for intellectual content: T. N. O’Brien. Category 3: a. Final approval of the completed manuscript: S. Lefter. REFERENCES 1. Pass RF, Little EA, Stagno S, Britt WJ, Alford CA. Young children as a probable source of maternal and congenital cytomegalovirus infection. N Engl J Med. 1987;316:1366. 2. De Silva SR, Chohan G, Jones D, Hu M. Cytomegalovirus papillitis in an immunocompetent patient. J Neuroophthalmol. 2008;28:126–127. 3. Chang PY, Hamam R, Giuliari GP, Foster CS. Cytomegalovirus panuveitis associated with papillitis in an immunocompetent patient. Can J Ophthalmol. 2012;47:e12–3 e35 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.