Cytomegalovirus optic neuropathy in a young immunocompetent patient.

Cytomegalovirus (CMV) infection is common worldwide, but is usually a subclinical or self-limited infection in immunocompetent patients. On the contrary, most of the ocular and central nervous system involvement occurs in immunosuppressed patient, and usually has severe consequences. Ocular manifestations of CMV infection are frequent in immunosuppressed patients (notably keratouveitis, retinitis and retinal branch angiitis), but a few cases of optic neuropathy (mostly papillitis) have been reported in the literature in immunocompetent patients. We report a case of a young and previously healthy female patient who developed a CMV optic neuropathy after a presumed CMV reinfection. Viral copies were detected in both blood and cerebrospinal fluid, as well as a high IgG titre and no evidence of immunosuppression was found. Clinical improvement was seen after high-dose corticosteroids and ganciclovir.

Herpes Zoster Optic Neuropathy.

BACKGROUND: Herpes zoster optic neuropathy (HZON) is a rare manifestation of herpes zoster ophthalmicus (HZO). The aim of our study was to better characterize the clinical features, therapeutic choices, and visual outcomes in HZON. METHODS: A retrospective chart review was performed at multiple academic eye centers with the inclusion criteria of all eyes presenting with optic neuropathy within 1 month of cutaneous zoster of the ipsilateral trigeminal dermatome. Data were collected regarding presenting features, treatment regimen, and visual acuity outcomes. RESULTS: Six patients meeting the HZON inclusion criteria were identified. Mean follow-up was 2.75 months (range 0.5-4 months). Herpes zoster optic neuropathy developed at a mean of 14.1 days after initial rash (range 6-30 days). Optic neuropathy was anterior in 2 eyes and retrobulbar in 4 eyes. Other manifestations of HZO included keratoconjunctivitis (3 eyes) and iritis (4 eyes). All patients were treated with systemic antiviral therapy in addition to topical and/or systemic corticosteroids. At the last follow-up, visual acuity in 3 eyes had improved relative to presentation, 2 eyes had worsened, and 1 eye remained the same. The 2 eyes that did not receive systemic corticosteroids had the best observed final visual acuity. CONCLUSION: Herpes zoster optic neuropathy is an unusual but distinctive complication of HZO. Visual recovery after HZON is variable. Identification of an optimal treatment regiment for HZON could not be identified from our patient cohort. Systemic antiviral agents are a component of HZON treatment regimens. Efficacy of systemic corticosteroids for HZON remains unclear and should be considered on a case-by-case basis.

Parvovirus B19 infection associated with hemolytic anemia and cranial polyneuropathy.

Parvovirus B19 (PB19) is a common, widespread, small, single-stranded DNA virus which has been linked with a broad spectrum of clinical illnesses, including a variety of neurological complications such as encephalitis, meningitis, myelitis, stroke, cerebellar ataxia, and neuropathy. The authors describe a case of PB19 infection associated with hemolytic anemia and cranial polyneuropathy involving the second and third cranial nerves in a 23-year-old immunocompetent woman. The diagnosis of acute PB19 infection was established with detection of positive DNA and anti-PB19 IgM antibodies in blood samples. Antiganglioside antibody studies were performed and serum anti-GD1b IgG was strongly positive. Further investigation was normal or negative, excluding other infectious or autoimmune disorders. The patient was initially treated with a 5-day course of intravenous immunoglobulin (IGIV). Because of incomplete neurological recovery, methylprednisolone was also administered 7 days after IGIV therapy initiation. Complete resolution of clinical symptoms was observed 3 months after disease onset at follow-up visit, despite the persistence of PB19 DNA and anti-PB19 IgM antibodies in serum 5 months after the initial presentation. Our report provides evidence that PB19 could affect both the central and peripheral nervous system, possibly by triggering an autoimmune mechanism that leads to autoantibody production.

Zika virus and the eye.

PURPOSE OF REVIEW: The aim of this study was to review the ocular findings related to the Zika virus (ZIKV) based on the main studies published to date, describe the patterns of the lesions and risk factors, and identify the public health implications and scientific importance of this emerging disease. RECENT FINDINGS: In most studies, the ZIKV seems related to congenital ocular lesions and most mothers reported mild symptoms during the first pregnancy trimester. Five fundus patterns were seen most often: macular chorioretinal atrophy, chorioretinal atrophy elsewhere, focal pigmentary changes in the macular region, optic nerve abnormalities and combined types. A few studies have suggested that the ZIKV might damage the anterior segment of these babies' eyes. Few reports have described the ocular complications seen in adults during the acute infection, including conjunctivitis, iridocyclitis and chorioretinitis. SUMMARY: Infants with congenital Zika syndrome might have vision-threatening fundus abnormalities. Although the full spectrum of ocular lesions caused by the ZIKV infection is not yet determined, a distinctive new disease has been observed. Recognition of these lesions by ophthalmologists can help ensure appropriate etiologic evaluation and clinical investigation to define the range of anomalies in an affected infant and determine essential follow-up and ongoing care.

Relationship between Human Immunodeficiency Virus Neuroretinal Disorder and Vision-Specific Quality of Life among People with AIDS.

PURPOSE: Some human immunodeficiency virus (HIV)-infected individuals have evidence of optic nerve or retinal dysfunction that manifests as decreased contrast sensitivity, even with good best-corrected visual acuity (BCVA). This condition, termed HIV-related neuroretinal disorder (HIV-NRD), is a risk factor for vision impairment (BCVA <20/40), blindness (BCVA ≤20/200), and increased mortality. We investigated the effect of HIV-NRD on vision-specific quality of life (QOL). DESIGN: Cross-sectional analysis of data from a prospective, observational study. PARTICIPANTS: Individuals from the Longitudinal Study of the Ocular Complications of AIDS cohort who completed the National Eye Institute 25-item Visual Function Questionnaire (VFQ-25), had BCVA of 20/40 or better, and had no evidence of ocular opportunistic infection or cataract. METHODS: We compared QOL by HIV-NRD status, adjusting for potential confounding variables, using multiple linear regression. Among those with HIV-NRD, we assessed the relationship between VFQ-25 and the logarithm of contrast sensitivity (logCS), using Spearman correlation. We defined a minimum clinically important difference (MCID) as 1 standard error of measurement from a well-characterized, historical population of individuals with a variety of ophthalmic disorders. MAIN OUTCOME MEASURES: Subscales and composite VFQ-25 scores (0 = worst, 100 = best). RESULTS: A total of 813 individuals met study criteria. Those with HIV-NRD (n = 39 [4.8%]) had a lower mean composite score than those without HIV-NRD (81 vs. 89; P = 0.0002) and lower mean scores in the following subscales: near activities (77 vs. 86; P = 0.004), distance activities (85 vs. 91; P = 0.01), social functioning (89 vs. 96; P = 0.0005), mental health (75 vs. 87; P = 0.0001), dependency (81 vs. 94; P < 0.0001), driving (75 vs. 85; P = 0.02), color vision (90 vs. 97; P < 0.0001), and peripheral vision (85 vs. 91; P = 0.0496). Score differences for each of these subscales met criteria for MCID. Among those with HIV-NRD, there was a positive correlation between logCS and composite score (r = 0.36; 95% confidence interval, 0.04-0.60). CONCLUSIONS: HIV-NRD has a statistically significant and clinically meaningful association with decreased vision-specific QOL among people with AIDS and good BCVA.

Visual loss related to macular subretinal fluid and cystoid macular edema in HIV-related optic neuropathy.

Optic nerve involvement may occur in various infectious diseases, but is rarely reported after infection by the human immunodeficiency virus (HIV). We report the atypical case of a 38-year-old patient in whom the presenting features of HIV infection were due to a bilateral optic neuropathy associated with macular subretinal fluid and cystoid macular edema, which responded well to antiretroviral therapy.

Neuro-Ophthalmic Manifestations of HIV Infection.

Purpose: To review the broad spectrum of clinical neuro-ophthalmic presentations associated with human immunodeficiency virus (HIV) infection. Methods: Critical review of the literature regarding neuro-ophthalmic consequences of HIV infection and its sequelae. Results: Neuro-ophthalmological diseases are common in both asymptomatic HIV-positive patients and those who profound immunosuppression with acquired immune deficiency syndrome (AIDS). Neuro-ophthalmic manifestations of HIV infection can involve the afferent or efferent visual pathway. Common clinical presentations include headache, papilledema, chorioretinitis, optic nerve involvement, meningitis, and cranial nerve palsies. Other neuro-ophthalmic manifestations include involvement of the visual pathway in the brain producing visual field defects such as occur in progressive multifocal encephalopathy. Pupil abnormalities have also been reported. Discussion: Neuro-ophthalmic consequences of HIV are important to recognize as it is critical to identify underlying neoplastic or infectious diseases which could be amenable to treatment.

Retinal Nerve Fiber Layer Thickness in Human T-cell Lymphotropic Virus Type 1 Patients.

PURPOSE: To determine whether there is an optic neuropathy (ON) in patients with human T-cell lymphotropic virus type 1 (HTLV-1) infection. METHODS: We included HTLV-1 asymptomatic carriers (a.c.HTLV-1) and tropical spastic paraparesis/HTLV-1 associated myelopathy (TSP/HAM) patients between January 1st, 2014 and March 31st, 2015. All patients had complete eye examination. The visual acuity (VA) and retinal nerve fiber layer (RNFL) thickness were measured and compared to age- and sex-matched control groups including patients seen in our refraction clinic with no previous medical or surgical history. RESULTS: Thirty-one a.c.HTLV-1 (group 1) and 29 TSP/HAM patients (group 2) were included. The average RNFL thickness was 99.9 ± 14.3 µm in group 1 and 87.8 ± 19.2 µm in group 2. The average RFNL thicknesses were lower in both groups, when compared to controls. The difference was significant in patients with TSP/HAM (87.8 ± 19.2 µm vs. 97 ± 7.8 µm; p = 0.003) who also had significantly decreased VA. CONCLUSIONS: We report here the first study about the RNFL thickness in patients with TSP/HAM. In these patients, there is decrease of the RNFL thickness with subtle but definite decrease of VA. This suggests that subclinical ON occurs in the natural history of the disease. The diagnosis of TSP/HAM must be evoked as a differential of primary progressive multiple sclerosis in a population at risk. Moreover, RNFL thinning with no evidence of glaucoma should raise suspicion for HTLV-1 infection and TSP/HAM in a population at risk.

Optic chiasm, optic nerve, and retinal involvement secondary to varicella-zoster virus.

Immunocompromised patients are known to be at risk for varicella-zoster virus reactivation, often in atypical manners. We describe a 30-year-old man with simultaneous involvement of the retina, optic chiasm, and optic nerve with varicella-zoster virus who had a bitemporal visual field defect.

Optic neuropathy preceding acute retinal necrosis in acquired immunodeficiency syndrome.

OBJECTIVE: To describe the clinical course of varicella-zoster optic neuropathy preceding acute retinal necrosis in patients with acquired immunodeficiency syndrome. DESIGN: Case series. SETTING: Two tertiary care centers in San Diego, Calif, and London, England. PATIENTS: Three human immunodeficiency virus-positive men with previous cutaneous zoster infection, optic neuropathy, and necrotizing retinitis. RESULTS: All patients had an episode of zoster dermatitis treated with acyclovir. Visual loss consistent with an optic neuropathy ensued, followed by typical herpetic retinitis. The cause of visual loss was not suspected to be varicella-zoster until after the retinitis occurred. Despite aggressive medical treatment, 4 of 6 eyes progressed to retinal detachment. CONCLUSIONS: Varicella-zoster may cause an optic neuropathy in patients with acquired immunodeficiency syndrome, especially in those with previous shingles. A high index of suspicion is necessary to establish the diagnosis and begin early antizoster treatment.

Optic nerve morphometry following axonal degeneration from SAIDS in rhesus monkeys.

In order to further study AIDS (acquired immunodeficiency syndrome) related neuropathologic findings, specifically progressive diffuse leuko-encephalopathy (PDL) and optic neuropathy, ten macaque monkeys (Macaca mulatta) were infected with simian immunodeficiency virus (SIV), observed for varying periods, and then sacrificed and tissue prepared for analysis. A marked difference from human AIDS pathology was found: in all monkeys, there was significant peripheral axonal dropout, as opposed to relatively little dropout in the central areas of the nerves (t stat. = -5.9, p < .001). In those monkeys infected with SIV for over one year, the axonal loss in the periphery was also greater than in the central optic nerve (t stat. = -5.03, p < .001); monkeys infected with SIV for less than one year had slightly less peripheral than central loss (t stat. = -4.5, p = .001). When compared with humans, however, it was found that the overall loss of axons was less (15% in monkeys vs. up to 45% in humans). There was also a lack of discernible retinal pathology, such as cotton wool spots, in the monkey tissue.

Mechanism of enterovirus involvement in epidemic neuropathy: hypothesis regarding pathophysiology.

During the epidemic of optic and peripheral neuropathy which occurred in Cuba in 1992-1993, viruses antigenically related to the Coxsackie viruses were isolated from cerebrospinal fluid of patients. Concurrently with the virologic studies, epidemiologic, toxicologic, nutritional, immunologic, and histopathologic investigations were also carried out. Although it was demonstrated that the illness was associated with toxic and nutritional risk factors, it has not been possible to identify a specific etiology for the symptoms observed. Taking into consideration the results obtained in all of the various investigations, we have formulated an integral, multifactorial hypothesis which attempts to explain a pathophysiologic mechanism by which the viruses isolated could participate in the pathogenesis of the illness. We propose that the viral agents produce a persistent infection, and the possibility that they may act as mediator of an autoimmune pathogenic process.

Optic neuropathy and central retinal vascular obstruction as initial manifestations of acute retinal necrosis.

BACKGROUND: The purpose of this brief communication is to alert ophthalmologists that optic neuropathy may herald acute retinal necrosis (ARN). CASE: A previously healthy 54-year-old man exhibited optic neuropathy as an initial presentation of ARN, 8 weeks after varicella-zoster dermatitis. OBSERVATIONS: Central retinal vascular obstruction developed subsequently in his left eye. Later, the classic presentation of ARN appeared in his contralateral eye. Systemic acyclovir therapy stopped the progression of retinitis and resulted in healing of retinal lesions in his right eye. CONCLUSIONS: This case suggests that optic neuropathy, especially with preceding herpetic dermatitis, should be suspected as the prodrome of ARN.

[Recurrent unilateral optic neuropathy associated with human immunodeficiency virus (HIV)]. / Neuropathie optique unilatérale récidivante liée au VIH.

HIV-related optic neuropathy is rare compared to optic neuropathies secondary to opportunistic infections in seropositive patients. We report the case of a 39-year-old HIV-positive woman referred for unilateral visual loss leading to the diagnosis of recurrent, unilateral, inflammatory optic neuropathy directly associated with HIV. Despite initial recovery after steroid treatment, she relapsed twice. Absence of any opportunist infections or toxic causes and presence of a very high viral load due to non-compliance with treatment led to the diagnosis of HIV-related optic neuropathy. Steroids and effective anti-retroviral treatment resulted in definitive and complete recovery. Inflammatory, degenerative and/or vascular mechanisms have been hypothesized to explain the occurrence of these rare HIV-related optic neuropathies. This diagnosis remains a diagnosis of exclusion to be considered in the work-up of seropositive patients with optic neuropathies.