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

Michael Absoud; Ming J. Lim; Richard Appleton; Anu Jacob; Joanna Kitley; M. Isabel Leite; Michael G. Pike; Angela Vincent; Evangeline Wassmer; Patrick Waters; Mark Woodhall; Cheryl Hemingway; Jacqueline Palace

doi:10.1136/jnnp-2014-308550

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

Michael Absoud, Ming J. Lim, Richard Appleton, Anu Jacob, Joanna Kitley, M. Isabel Leite, Michael G. Pike, Angela Vincent, Evangeline Wassmer, Patrick Waters, Mark Woodhall, Cheryl Hemingway, Jacqueline Palace

Research output: Contribution to journal › Letter › peer-review

92 Citations (Scopus)

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.

Original language	English
Pages (from-to)	470-2
Number of pages	3
Journal	Journal of neurology, neurosurgery, and psychiatry
Volume	86
Issue number	4
DOIs	https://doi.org/10.1136/jnnp-2014-308550
Publication status	Published - Apr 2015

Keywords

Adolescent
Antibodies
Aquaporin 4
Brain
Child
Child, Preschool
Cohort Studies
Female
Humans
Infant
Magnetic Resonance Imaging
Male
Neuromyelitis Optica
Prognosis
Recurrence
Retrospective Studies
Treatment Outcome
Letter

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10.1136/jnnp-2014-308550

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@article{c3818ba135274f97b1a3e365d325517b,

title = "Paediatric neuromyelitis optica: clinical, MRI of the brain and prognostic features",

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.",

keywords = "Adolescent, Antibodies, Aquaporin 4, Brain, Child, Child, Preschool, Cohort Studies, Female, Humans, Infant, Magnetic Resonance Imaging, Male, Neuromyelitis Optica, Prognosis, Recurrence, Retrospective Studies, Treatment Outcome, Letter",

author = "Michael Absoud and Lim, {Ming J.} and Richard Appleton and Anu Jacob and Joanna Kitley and Leite, {M. Isabel} and Pike, {Michael G.} and Angela Vincent and Evangeline Wassmer and Patrick Waters and Mark Woodhall and Cheryl Hemingway and Jacqueline Palace",

note = "Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.",

year = "2015",

month = apr,

doi = "10.1136/jnnp-2014-308550",

language = "English",

volume = "86",

pages = "470--2",

journal = "Journal of neurology, neurosurgery, and psychiatry",

issn = "0022-3050",

publisher = "BMJ Publishing Group",

number = "4",

}

TY - JOUR

T1 - Paediatric neuromyelitis optica

T2 - clinical, MRI of the brain and prognostic features

AU - Absoud, Michael

AU - Lim, Ming J.

AU - Appleton, Richard

AU - Jacob, Anu

AU - Kitley, Joanna

AU - Leite, M. Isabel

AU - Pike, Michael G.

AU - Vincent, Angela

AU - Wassmer, Evangeline

AU - Waters, Patrick

AU - Woodhall, Mark

AU - Hemingway, Cheryl

AU - Palace, Jacqueline

N1 - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

PY - 2015/4

Y1 - 2015/4

N2 - 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.

AB - 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.

KW - Adolescent

KW - Antibodies

KW - Aquaporin 4

KW - Brain

KW - Child

KW - Child, Preschool

KW - Cohort Studies

KW - Female

KW - Humans

KW - Infant

KW - Magnetic Resonance Imaging

KW - Male

KW - Neuromyelitis Optica

KW - Prognosis

KW - Recurrence

KW - Retrospective Studies

KW - Treatment Outcome

KW - Letter

U2 - 10.1136/jnnp-2014-308550

DO - 10.1136/jnnp-2014-308550

M3 - Letter

C2 - 25091363

SN - 0022-3050

VL - 86

SP - 470

EP - 472

JO - Journal of neurology, neurosurgery, and psychiatry

JF - Journal of neurology, neurosurgery, and psychiatry

IS - 4

ER -

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

Abstract

Keywords

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