Paediatric neuromyelitis optica: clinical, MRI of the brain and prognostic features.
J Neurol Neurosurg Psychiatry
; 86(4): 470-2, 2015 Apr.
Article
en En
| MEDLINE
| ID: mdl-25091363
ABSTRACT
BACKGROUND:
Neuromyelitis Optica (NMO) is a severe and rare inflammatory condition, where relapses are predictive of disability.METHODS:
We describe a national paediatric NMO cohort's clinical, MRI, outcome, and prognostic features in relation to Aquaporin-4 antibody (AQP4-Ab) status, and compared to a non NMO control cohort. OBSERVATIONS Twenty NMO cases (females = 90%; AQP4-Ab positive = 60%; median age = 10.5 yrs) with median follow-up = 6.1 yrs were compared to a national cohort sample of known sequential AQP4-Ab negative first episode CNS acquired demyelination cases (n = 29; females = 55%; all AQP4-Ab negative; median age = 13.6 yrs). At presentation, 40% NMO cases had unilateral optic neuritis (ON); 20% bilateral ON; 15% transverse myelitis (TM); 15% simultaneous TM&ON; 10% Acute disseminated encephalomyelitis. At follow up, 55% had a clinical demyelinating episode involving the brain; 30% of cases had abnormal brain MRI at onset and 75% by follow up. NMO brain scan lesions compared to controls were large (> 2 cm), acute lesions largely resolved on repeat imaging, and often showed T1 hypointense lesions. Mean time to relapse = 0.76 yrs (95% CI 0.43-1.1 yrs) for AQP4-Ab positive vs 2.4 yrs in AQP4-Ab negative cases (95% CI 1.1-3.6 yrs). In AQP4-Ab positive cases, 10/12 had visual acuity < 6/60 Snellen in ≥ 1 eye (0/8 AQP4-Ab negative), and 3 AQP4-Ab negative cases were wheelchair-dependent.CONCLUSIONS:
In children, NMO is associated with early recurrence and visual impairment in AQP4-Ab positivity and physical disability in AP4-Ab negative relapsing cases. Distinct MRI changes appear more commonly and earlier compared to adult NMO. Early AQP4-Ab testing may allow prompt immunomodulatory treatment to minimise disability.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Encéfalo
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Neuromielitis Óptica
Tipo de estudio:
Etiology_studies
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Incidence_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
Límite:
Adolescent
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Child
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Child, preschool
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Female
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Humans
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Infant
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Male
Idioma:
En
Revista:
J Neurol Neurosurg Psychiatry
Año:
2015
Tipo del documento:
Article
País de afiliación:
Reino Unido