Childhood optic neuritis: The pediatric neurologist's perspective

doi:10.1016/j.ejpn.2008.09.003

European Journal of Paediatric Neurology

Volume 13, Issue 5, September 2009, Pages 452-457

https://doi.org/10.1016/j.ejpn.2008.09.003 Get rights and content

Abstract

Background

Optic neuritis in children may be an isolated, usually postinfectious event, or the symptom of a more widespread disorder.

Aim

To investigate the etiological spectrum of optic neuritis in children in association with diagnostic findings and follow-up results.

Methods

We retrospectively examined the records of 31 children aged 4–15 (mean 9.7 ± 2.9) years in whom isolated optic neuritis was the presenting neurological symptom.

Results

Monophasic bilateral optic neuritis was the most common presentation (45%), followed by the unilateral (32%) and recurrent (22%) forms. Initial cranial MRI was abnormal in 12/31 patients. During a mean follow-up of 2.2 years (6 months–15 years), 6/14 bilateral cases, 9/10 unilateral and 5/7 recurrent cases were diagnosed with various disorders including total eight with MS. The MS group tended to start with unilateral optic neuritis, was older (mean 11.6 ± 1.5 vs. 8.8 ± 2.9 years), and included more girls than the other groups.

Conclusions

Optic neuritis in children is frequently part of a systemic or neurological disorder even in the presence of normal cranial imaging. These patients should be evaluated and followed-up in pediatric neurology clinics.

Introduction

Acute optic neuropathy (ON) is an inflammatory disorder of the optic nerve. Its etiology, incidence and outcome in children differ from adults, and the clinician faces a list of differential diagnoses and appropriate laboratory investigations to detect treatable causes and estimate outcome.¹ However the various causes of childhood ON are not represented in most series reported so far. Our aim in this study was to review etiological factors, clinical presentation, magnetic resonance imaging (MRI) and final diagnoses in our series of childhood ON.

Section snippets

Materials and methods

We retrospectively analyzed the medical records of 0–16-year-old patients who presented to the departments of pediatric neurology of participating hospitals with acute ON as first and only neurological symptom between 2000 and 2008. The diagnosis was based on at least two of the following criteria: visual loss, relative afferent pupillary defect, or visual field defect, all in the absence of retinal and cortical lesions.1, 2 ON was classified as bilateral (BON) if both eyes were involved

Results

There were 31 patients (18 girls, 13 boys, F/M:1.3) who experienced their first optic neuritis episode between ages 4–15 (mean 9.7 ± 2.9) years. Two of them (cases 5 and 10, Table 1) have been reported before.3, 4 Mean follow-up period was 2.2 years (range: 6 months–15 years). Family history was positive for autoimmune disorder in first- or second-degree relatives in 8 patients (25%) (Table 1). Initial cranial MRI was abnormal in 12/31 patients, spinal MRI in 5/8, and orbital MRI in 3/8 patients.

Discussion

This series illustrates the etiological spectrum and management of ON in pediatric neurology clinics. It contains most, if not all, ON cases diagnosed in these institutions: occasional cases such as traumatic ON diagnosed in neurosurgery clinics, or toxic ON due to long-term use of certain drugs might have been followed-up in other departments and underrepresented in this series.

The etiology of childhood ON is most commonly postinfectious in many reports: a preceding viral infection has been

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In incidence of ON in Canada was lower at 0.2/100,000 person years, based on a national survey of pediatricians between 2004 and 2007 and 219 children with ADS [2]. The mean age of presentation among children is 9 to 11 years [3–7]. Younger children tend to present with bilateral disease.
Clinical Characteristics of Pediatric Optic Neuritis With Myelin Oligodendrocyte Glycoprotein Seropositive: A Cohort Study
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ON is a common clinical entity that involves a visible part of the optic nerve that may help us to understand the pathology and prognoses of central nerve system demyelinating disorders.11 ON in the pediatric population has some unique features distinct from adults: bilateral involvement, disc edema, and favorable visual outcomes.12-14 Increasing evidence has shown that ON in adults with serum MOG antibodies have some distinct features from typical MS or NMO phenotypes.3,7,15,16
The clinical characteristics of patients with pediatric optic neuritis with seropositive myelin oligodendrocyte glycoprotein antibodies in Asia have not been reported.
Patients ≤18 years-old with acute-onset optic neuritis were enrolled. Serum myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies were detected and patients were followed for at least six months. The clinical features were evaluated among myelin oligodendrocyte glycoprotein-seropositive optic neuritis, aquaporin-4-seropositive optic neuritis, and double seronegative optic neuritis. Best-corrected visual acuity, thickness of optic disc retinal nerve fiber layer, and macular ganglion cell complex were measured by optical coherence tomography.
Among myelin oligodendrocyte glycoprotein-optic neuritis, aquaporin-4-optic neuritis, and seronegative-optic neuritis, the percentages of best-corrected visual acuity measured better than 0.8 (20/25) at the six-month visit were 89.47%, 33.33%, and 82.26%, respectively, a rate that is significantly better in patients with myelin oligodendrocyte glycoprotein-optic neuritis and seronegative-optic neuritis (P = 0.02). The average peripapillary retinal nerve fiber layers were 58.03 ± 8.73 µm, 64.34 ± 12.88 µm, and 78.12 ± 13.34 µm for the patients with myelin oligodendrocyte glycoprotein-optic neuritis, aquaporin-4-optic neuritis, and seronegative-optic neuritis, respectively, which showed no statistical difference between patients with myelin oligodendrocyte glycoprotein-optic neuritis and aquaporin-4-optic neuritis (P = 0.089), but were both thinner than patients with seronegative-optic neuritis (P = 0.001).
The recovery of visual acuity in patients with myelin oligodendrocyte glycoprotein-optic neuritis was as good as in patients with seronegative-optic neuritis, and the retinal nerve fiber layer of the optic nerve head showed thinning as severe as that of the patients with aquaporin-4-optic neuritis.
Visual Loss: Optic Neuropathies
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The diagnosis of optic neuropathy is usually considered under two circumstances: (1) when visual loss is associated with an anomalous, swollen, or pale optic disc or (2) when the fundus examination is normal but deficits in acuity, color, and visual field are accompanied by an afferent pupillary defect. In each of these situations the examiner must rely upon historical information, examination findings, and diagnostic testing first to confirm the presence of optic nerve dysfunction, then to determine its etiology.
An understanding of optic nerve anatomy is important when approaching patients with optic nerve dysfunction, and this chapter reviews this topic. Typical clinical features of optic neuropathies are then detailed. The subsequent discussion suggests an approach to patients with an optic neuropathy and outlines a strategy used to distinguish the various optic neuropathies. The features (presentation, examination, and management) of the different causes of optic nerve dysfunction are then addressed.
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Optic neuritis in children may be an idiopathic, isolated event; an autoimmune response to infection or immunization; or a manifestation of a systemic demyelinating disorder. In younger children, optic neuritis is more likely to occur following infection or vaccination, or as part of acute disseminated encephalomyelitis (ADEM).10,22 In children older than 10-12 years of age, optic neuritis more commonly occurs in association with a primary demyelinating disease such as MS or NMO.5,23
Optic neuritis is rare in children in comparison to adults, but accounts for approximately 25% of pediatric acute demyelinating syndromes. Features of pediatric optic neuritis that differ from adults include a higher rate of bilaterality, poor visual acuity on presentation, and papillitis. Diagnostic work-up includes brain magnetic resonance imaging, lumbar puncture, and blood tests to exclude infectious and inflammatory disorders. Pediatric optic neuritis may occur following infection or vaccination, or in association with a systemic demyelinating process such as acute disseminated encephalomyelitis, neuromyelitis optica, or multiple sclerosis. Treatment is controversial, but most practitioners administer corticosteroids. Most children with optic neuritis experience full visual recovery. The recently launched Pediatric Optic Neuritis Prospective Data Collection Study (PON1) aims to provide estimates of visual acuity outcome and assess the potential to recruit for a future pediatric optic neuritis treatment trial.
Demyelinating, inflammatory, infectious, and infiltrative optic neuropathies
2016, Taylor and Hoyt's Pediatric Ophthalmology and Strabismus, Fifth Edition

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Original articleChildhood optic neuritis: The pediatric neurologist's perspective

Abstract

Background

Aim

Methods

Results

Conclusions

Introduction

Section snippets

Materials and methods

Results

Discussion

J AAPOS

Pediatr Neurol

Pediatr Neurol

Eur J Obstet Gynecol Reprod Biol

Optic neuritis in adults and children

Semin Ophthalmol

The clinical features, MRI findings, and outcome of optic neuritis in children

Neurology

Bilateral isolated acute optic neuritis in a child after acute rubella infection

Jpn J Ophthalmol

Optic neuropathy in primary antiphospholipid syndrome in childhood

J Child Neurol

Recovery after optic neuritis in childhood

J Neurol Neurosurg Psychiatry

Mikrobiyol Bul

Persistent hypoglycorrhachia in neurobrucellosis

Pediatr Infect Dis J

Optic neuritis in children

J Pediatr Ophthalmol Strabismus

Original article
Childhood optic neuritis: The pediatric neurologist's perspective