Paediatric neuromyelitis optica: clinical, MRI of the brain and prognostic features.

Journal article

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=10.5yrs) with median follow-up=6.1yrs 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=13.6yrs). At presentation, 40% NMO cases had unilateral optic neuritis (ON); 20% bilateral ON; 15% transverse myelitis (TM); 15% simultaneous TMandON; 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=0.76yrs (95% CI 0.43-1.1yrs) for AQP4-Ab positive vs 2.4yrs in AQP4-Ab negative cases (95% CI 1.1-3.6yrs). 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.

Authors: Absoud, M; Lim, M; Appleton, R; Jacob, A; Kitley, J

• Publication status: Published
• Peer review status: Peer reviewed
• Publisher: BMJ Publishing Group
• Journal: Journal of Neurology, Neurosurgery and Psychiatry
• Volume: 86
• Issue: 4
• Pages: 470-472
• Publication date: 2014-01-01
• DOI: 10.1136/jnnp-2014-308550
• EISSN: 1468-330X
• ISSN: 0022-3050
• Language: English
• Keywords: PAEDIATRIC; MRI; EPIDEMIOLOGY; MULTIPLE SCLEROSIS; NEUROIMMUNOLOGY