Optic Neuritis & MS In Children

Purpose
To describe the clinical characteristics of Optic Neuritis in children, including final Visual Acuity and development of Multiple Sclerosis (MS).

Methods
Charts were reviewed of all patients < 15 years of age who presented with Optic Neuritis to the Bascom Palmer Eye Institute or the Miami Children's Hospital between 1986 and 1998.

Results
Fifteen patients were identified. There was a slight female predilection in the study group (60%), with a mean age of 9.8 years at presentation.

A preceding febrile illness within 2 weeks of Visual symptoms was reported in 66% of patients.

Initial Visual Acuity ranged from 20/15 to no light perception. Involvement was bilateral in 66% of patients, and disc swelling was present in 64% of involved eyes.

Of the patients who underwent Magnetic Resonance Imaging, 33% had focal DeMyelinating lesions in the Brain, and 63% of affected Nerves were enlarged or enhanced with Gadolinium.

Eleven patients were treated with intravenous Steroids. Final Visual Acuity was > or = 20/40 in 58.3% of eyes.

Thirty percent of the patients had Vision of finger counting or worse. Four (26%) patients developed MS. The mean age of patients with MS was 12 years, compared with 9 years in children who did not develop MS.

Patients with unilateral involvement had an excellent Visual prognosis (100% > 20/40), but a higher rate of development of MS (75%). Two patients had positive serology for Lyme disease.

Conclusions
Optic Neuritis presents differently in children than in adults. Children typically have bilateral involvement with Papillitis following an antecedent Viral illness.

Although Visual prognosis is poorer in children than adults, the development of MS is less common in children.

Children who present with unilateral involvement have a better Visual prognosis; however, they also develop MS at a greater frequency than children with bilateral involvement.

Patients who developed MS were, on average, older at presentation with Optic Neuritis than those who did not develop MS.

NeuroMyelitis Optical (NMO) or Devic's Disease is an uncommon clinical syndrome associating unilateral or bilateral Optic Neuritis and Transverse Myelitis within about 8 weeks.

Usually reported in adults, childhood cases constitute a distinctive clinical entity with good Visual and Neurological prognosis without long-term recurrence or sequelae.

The PathoGenesis is explained by AutoImmune DeMyelination triggered by a Viral Disease. Some authors suggest an infectious cause.

The CT scan is usually negative while MRI shows nonspecific small foci in the White Matter.

Unlike Multiple Sclerosis, the main differential diagnosis, in NMO the CerebroSpinal Fluid may show PleoCytosis with elevated protein level without an OligoClonal Band.

We report a case of NeuroMyelitis Optica occurring in a 4-year-old child following a flu-like syndrome. Clinical outcome was favorable with a short course of high-dose CorticoSteroids.

A 2-year and 7-month-old boy had sudden Visual loss in both eyes and showed bilateral Optic Neuritis without systemic symptoms. Steroid therapy improved his Visual Acuity from 0.077 and 0.053 to 1.0 at 7 months after onset.

Magnetic Resonance Imaging (MRI) of the Brain showed high density in both Optic Nerves and multiple lesions in the White Matter that were enhanced by Gadolinium.

We considered the diagnosis of DeMyelinating Disease. Follow-up MRI showed no abnormal Lesion.

Both transient and steady-state pattern Visually Evoked Cortical Potentials were nondetectable at the onset, and the P100 component of the transient pattern reversed Visually Evoked Cortical Potential appeared to be delayed thereafter.

It has since become shorter in parallel with Visual Acuity improvement.

We reviewed the records of all children (younger than 16 years of age) who presented with a diagnosis of Optic Neuritis (ON).

Identified through the comprehensive records-linkage system at the Mayo Clinic and identified 94 cases between 1950 and 1988 with a documented history of Idiopathic ON.

Detailed follow-up information was available on 79 patients, with a median length of follow-up of 19.4 years.

Life-table analysis showed that of the 79 patients with isolated ON, the progression rate to Clinically or Laboratory-Supported Definite Multiple Sclerosis (MS) were as follows:
1. 13% by 10 years
2. 19% by 20 years
3. 22% by 30 years
4. 26% by 40 years

Gender, age, funduscopic findings, Visual Acuity, or family history of either ON or MS did not predict the development of MS.

The presence of bilateral sequential or recurrent ON increased the risk of developing MS (p = 0.002; hazard ratio = 5.09). Whereas the presence of infection within 2 weeks before the onset of ON decreased the risk of developing MS (p = 0.060; hazard ratio = 0.24).

This study of childhood ON supports the lower risk of recurrence and progression to MS compared with adults.

Recurrent Optic Neuritis and the subsequent development of Multiple Sclerosis in children, particularly in Asian countries, are not well known.

We report on recurrent Optic Neuritis and subsequent Multiple Sclerosis in 22 Thai children who were younger than 15 years of age at the onset of Optic Neuritis, had no previous Neurologic illness, and were monitored for 6-20 years.

Improvement of Vision was observed in 8, 10, and 2 patients after the first, second, and third week of onset, respectively.

Two patients had recurrent Optic Neuritis and the other 2 patients subsequently developed Clinically Definite Multiple Sclerosis.

Characterized by Hemiparesis at 6 months and Myelopathy at 2 years later in 1 patient and Myelopathy and BrainStem Dysfunction in the other at 4 and 6 years later.

The frequency of subsequent development of Multiple Sclerosis after Optic Neuritis may be similar to that reported from the United States and European countries.

In a retrospective review, the eye symptoms of 17 children (mean age: 13 1/2 years) who had definite Multiple Sclerosis (Poser's Criteria) and 15 who had Probable Multiple Sclerosis over the last 18 years were evaluated.

Follow-up varied from 3 weeks to 6 years. Of 94% of children (16 of 17) with Ophthalmologic symptoms, 47% (8 of 17) presented with an initial disturbance of Vision.

Twelve children had Optic Neuritis, 1 Progressive Uveitis, and 4 BrainStem symptoms (i.e., VI^th Nerve Palsy, 1 ^1/2 Syndrome, InterNuclear OphthalMoplegia).

Four children had Cerebellar Signs (Nystagmus, Saccadic Pursuit). In 4 children, clinical localization was less specific.

Recovery was generally good in most of the children; Cerebellar problems were most persistent. Multimodal potentials were more helpful for investigation of Optic Neuritis and Cerebellar lesions than for BrainStem lesions.

In the cohort of probable Multiple Sclerosis of 15 children, 11 had eye symptoms (5 with NeuroMyelitis Optica, 4 Optic Neuritis, 1 InterNuclear Ophthalmoplegia, and 1 Cerebellar symptoms).

Ophthalmologic symptoms are slightly more frequent in children with Multiple Sclerosis than in adults and should be specifically investigated to establish the diagnosis.

Background
Optic Neuritis (ON in childhood is thought to be more likely bilateral and less likely to lead to Multiple Sclerosis (MS) vs ON in adults.

Methods
The authors evaluated clinical features, maximal Visual deficit and recovery, Visual Evoked Potentials (VEPs), NeuroImaging, and outcome in a cohort of children with ON.

Results
Records of 36 children (female/male ratio 1.6), ages 2.2 to 17.8 (mean 12.2) years, were reviewed.

ON was unilateral in 58% and bilateral in 42%. Maximal Visual deficit was severe in 69%, but full recovery occurred in 39 of 47 affected eyes (83%).

VEPs were abnormal in 88%. Neurologic abnormalities in addition to those associated with ON were documented in 13 children. NeuroImaging studies of the Optic Nerves were abnormal in 55%.

Brain MRI in 35 children demonstrated White Matter lesions separate from the Optic Nerves in 54%. Follow-up is 2.4 years (0.3 to 8.3 years).

To date, 13 children (36%) have been diagnosed with MS and 1 has Devic Disease.

Bilateral ON was more likely to be associated with MS outcome (p = 0.03).

All 13 children with MS had White Matter lesions on Brain MRI. None of the children with a normal Brain MRI have developed MS to date.

Conclusions
Contrary to expectations, Optic Neuritis (ON) in childhood was more likely to be unilateral, Multiple Sclerosis (MS) risk was high (36% at 2 years), and bilateral rather than unilateral ON was associated with a greater likelihood of MS.

Clinical findings extrinsic to the Visual System on baseline examination (p < 0.0001) and MRI evidence of White Matter lesions outside the Optic Nerves (p < 0.0001) were strongly correlated with MS outcome.