Journal of Neuro-Ophthalmology:
De Silva, Samantha Roshani MA, BMBCh, MRCP; Chohan, Gurjit MBChB, MRCP; Jones, Dean MBBS, BSc (Hons); Hu, Michele PhD, MRCP
Department of Neurology, Radcliffe Infirmary, Oxford, United Kingdom.
Address correspondence to Samantha Roshani De Silva, MA, BMBCh, MRCP, Department of Neurology, Radcliffe Infirmary, Oxford OX2 6HE, UK; E-mail: email@example.com
A 22-year-old immunocompetent woman who presented with a 3-week history of fever, headache, and visual loss and was found to have subnormal visual acuity and bilateral optic disc swelling. Serum cytomegalovirus (CMV) IgM and IgG and polymerase chain reaction results were positive, indicating an acute CMV infection. No cause of immunocompromise was found. After treatment with intravenous ganciclovir, the papillitis and systemic CMV illness resolved with no residual deficit. This is the first reported case of primary CMV papillitis to be successfully treated with ganciclovir alone.
In immunocompromised patients, ocular and neurologic complications of cytomegalovirus (CMV) infection are well recognized. On the other hand, CMV infection in immunocompetent patients is generally asymptomatic or presents with a mononucleosis syndrome (1). We report a case of CMV papillitis in an immunocompetent patient.
A 22-year-old woman presented with a 3-week history of fever, headache, and blurred vision. Best-corrected visual acuities were 20/60 in the right eye and 20/30 in the left eye. Ophthalmoscopy revealed bilateral optic disc swelling with no evidence of retinitis (Fig. 1). Results of neurologic examination were otherwise unremarkable. Physical examination revealed a temperature of 39.3C and splenomegaly.
Goldmann visual fields showed generalized constriction bilaterally. Electroretinographic (ERG) potentials (30 Hz) and visual evoked potentials were within normal limits. Investigations revealed pancytopenia (hemoglobin 9.0 g/dL, mean corpuscular volume [MCV] of 79 fL, white blood cell (WBC) count of 2.4 109/L, neutrophil count of 1.1 109/L, and platelet count of 87 109/l) with atypical lymphocytes. Activated partial thromboplastin time (APTT) was 35.4 seconds.
CMV IgM antibody and IgG antibody and serum CMV polymerase chain reaction (PCR) results were positive. (IgM was measured at >240 units, or strongly positive). Toxoplasma IgM and IgG, parvovirus, syphilis, and HIV serology results were negative. Epstein Barr virus IgG was positive. Cerebrospinal fluid PCR results for CMV, herpes simplex, herpes zoster, and enterovirus were negative. Brain MRI, including orbital views, and CT of chest, abdomen, and pelvis was normal.
The ophthalmic diagnosis was CMV papillitis. A 2-week course of intravenous ganciclovir treatment was commenced. Two months after initial presentation, best-corrected visual acuities and color vision were normal bilaterally. Ophthalmoscopy showed a normal optic disc on the right and mild nasal optic disc swelling on the left. The pancytopenia had improved and the coagulopathy had resolved.
CMV infection in immunocompetent patients often presents with a mononucleosis-like syndrome featuring malaise, fever, sweats, mild abnormalities in liver function tests (2), and lymphocytosis with atypical lymphocytes (3). It is usually mild and self-limited. CMV encephalitis, meningitis, transverse myelitis, and retinitis have been described (3,4).
CMV optic neuritis has been described in immunocompromised patients and is usually a rapidly blinding disease (5), but there is only one previously reported case of CMV papillitis in an immunocompetent individual (1). That report involved a 32-year-old healthy woman who developed bilateral papillitis after a flu-like illness and was found to have positive serum CMV IgM and IgG. CMV antigen was present in the urine. She did not have any other systemic complications of CMV disease. The patient was treated with foscarnet and intravenous methylprednisolone and had complete resolution of the visual symptoms and signs. Our patient was treated with ganciclovir alone; steroids were not used. Thus, our patient is the first reported case of primary CMV infection causing papillitis to resolve after treatment with antiviral medication alone.
It has been noted previously that CMV infection in immunocompetent patients may be relapsing in nature with symptoms lasting up to 32 weeks (2). Thus, the time taken for recovery in our patient is in keeping with this time frame. Relapse can be silent with stable visual acuity and fundus appearance, so central visual field testing is important (5).
A range of extracutaneous and cutaneous features can occur in CMV infection in immunocompetent patients. It is thought that these symptoms are not the direct consequence of CMV proliferation but of an immunologic response to the virus (6). Some characteristics of the CMV infection appear to predispose to a transitory immunocompromised state. The atypical lymphocytes seen in acute CMV infection are mainly CD8+ T cells. There is also a decrease in CD4+ cells, altering the CD4/CD8 ratio (7). This altered ratio could promote the appearance of CMV neuroretinal manifestations in healthy individuals (5). It remains unclear as to why some patients develop retinitis and others develop optic neuritis.
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