Devic's NeuroMyelitis Optica. A Childhood Case And Review Of The Literature
Bencherif MZ, Karib H, Tachfouti S, Guedira K, Mohcine Z
J Fr Ophtalmol 2000 May;23(5):488-90
Hopital des Specialites, Service d'Ophtalmologie A, Rabat, Maroc
PMID# 10844309; UI# 20304822
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.
Follow-Up Studies On Pattern Reversal Visually Evoked Cortical Potentials In A 2-Year-Old Child With Optic Neuritis
Sato K, Adachi-Usami E, Mizota A
J Neuroophthalmol 1998 Mar;18(1):21-4
Chiba Univ, School of Medicine, Dept of Ophthalmology, Japan
PMID# 9532534; UI# 98193713
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.
Risk of Developing Multiple Sclerosis
After Childhood Optic Neuritis
Lucchinetti CF, Kiers L, O'Duffy A, Gomez MR, Cross S, Leavitt JA, O'Brien P, Rodriguez M
Neurology 1997 Nov;49(5):1413-1418
Mayo Clinic, Dept of Neurology, Rochester, MN, USA
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:
- 13% by 10 years
- 19% by 20 years
- 22% by 30 years
- 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.
Optic Neuritis In Children:
Recurrence And Subsequent Development
Of Multiple Sclerosis
Visudhiphan P, Chiemchanya S, Santadusit S
Pediatr Neurol 1995 Nov;13(4):293-5
Mahidol University, Dept of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Bangkok, Thailand
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.
Eye Problems In Children
With Multiple Sclerosis
Steinlin MI; Blaser SI; MacGregor DL; Buncic JR
Pediatr Neurol 1995 Apr;12(3):207-12
Hospital for Sick Children, Dept of Paediatrics, Toronto, Ontario, Canada
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., VIth 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.
The Clinical Features, MRI Findings, And Outcome Of Optic Neuritis In Children
Wilejto M, Shroff M, Buncic JR, Kennedy J, Goia C, Banwell B
Neurology 2006 Jul 25;67(2):258-62
Research Institute, Hospital for Sick Children, Department of NeuroRadiology, Toronto, Ontario, Canada
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.
The authors evaluated clinical features, maximal Visual deficit and recovery, Visual Evoked Potentials (VEPs), NeuroImaging, and outcome in a cohort of children with ON.
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.
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.