Pediatric optic neuritis

Abstract

Optic neuritis (ON) is a common presenting symptom in pediatric CNS demyelinating disorders and may be associated with dramatic visual loss. Knowledge regarding clinical presentation, associated diseases, therapy, and outcomes in ON in children has grown over the past decade. These studies have shown that younger children (,10 years of age) are more likely to present with bilateral ON and older children with unilateral ON. Furthermore, studies focusing on visual recovery have shown excellent recovery of high-contrast visual acuity in the majority of children, but functional and structural studies have shown evidence of irreversible injury and functional decline after ON in children. Although randomized controlled treatment trials have not been performed in children and adolescents with ON, standard of care suggests that the use of highdose pulse steroids is safe and likely effective. This article reviews current knowledge about the clinical presentation and management of pediatric ON, with attention to associated syndromes and evaluative tools that may inform diagnosis and interventions. Neurology ® 2016;87 (Suppl 2): S53-S58 GLOSSARY ADEM 5 acute disseminating encephalomyelitis; AQP4 5 aquaporin-4; HCVA 5 high-contrast VA; CRION 5 chronic relapsing inflammatory optic neuropathy; IVIg 5 IV immunoglobulin; LCLA 5 low-contrast letter acuity; LCVA 5 low-contrast VA; MOG 5 myelin oligodendrocyte glycoprotein; MS 5 multiple sclerosis; NMO 5 neuromyelitis optica; NMOSD 5 NMO spectrum disorder; OCT 5 optical coherence tomography; ON 5 optic neuritis; ONTT 5 Optic Neuritis Treatment Trial; RNFL 5 retinal nerve fiber layer; TPE 5 therapeutic plasma exchange; VA 5 visual acuity; VEP 5 visual evoked potential; VF 5 visual field.

Retinal nerve fibre layer thinning is associated with worse visual outcome after optic neuritis in children with a relapsing demyelinating syndrome

“…Optic neuritis, defined as inflammation of one or both optic nerves in association with visual dysfunction, is one of the most common presentations of acquired central nervous system (CNS) demyelination in childhood, with an incidence of approximately 0.2 per 100 000 . Core deficits in visual acuity, colour perception, and visual field are commonly accompanied by ocular pain and headache . Sixty per cent to 77% of children suffer severely decreased visual acuity (worse than logarithm of the minimum angle of resolution [logMAR] 1.0 or Snellen 20/200) in the acute phase .…”

Clinical profile and neuroimaging in pediatric optic neuritis in Indian population: A case series

“…It can be a presenting symptom of various pediatric central nervous system disorders and may be associated with dramatic visual loss. [1] It is widely accepted that in children, attacks of ON usually occur following a febrile illness, tend to affect both eyes, are frequently associated with swollen discs, improve rapidly, with good outcomes. [2345] The rate of conversion to multiple sclerosis (MS) is not exactly determined; studies have reported rates from 4% to 36%.…”

First Presentation of an Acquired Demyelinating Syndrome

“…The clinical presentation of optic neuritis in children includes decreased visual acuity, dyschromatopsia (impaired color vision), and visual field deficits that progress over hours to days. 6 Pain with eye movements is common but not always present. [7][8][9] Physical examination findings may also include a relative afferent pupillary defect and optic nerve pathology on fundoscopic examination, specifically papillitis in the acute stage (seen in 40-67%) [6][7][8][9] and optic nerve pallor in the chronic stage.…”

“…[7][8][9] Physical examination findings may also include a relative afferent pupillary defect and optic nerve pathology on fundoscopic examination, specifically papillitis in the acute stage (seen in 40-67%) [6][7][8][9] and optic nerve pallor in the chronic stage. 6,8 When the optic appears normal but the clinical history and remainder of examination support optic neuritis, a demyelinating event along the optic nerve posterior to the optic disc is suspected, and the term retrobulbar optic neuritis is used. Optic neuritis may be unilateral or bilateral; the likelihood of simultaneous bilateral optic neuritis is higher in children younger than 10 years.…”

“…Optic neuritis may be unilateral or bilateral; the likelihood of simultaneous bilateral optic neuritis is higher in children younger than 10 years. 6 In children, the differential diagnosis of optic neuritis due to a demyelinating event includes optic neuropathies of infectious, other inflammatory, compressive, ischemic, hereditary, neoplastic, metabolic, and nutritional causes. 10 Depending on the clinical scenario, workup may include testing to discriminate optic neuritis from mimicking disorders (see ►Table 1).…”