Published online October 6, 2008
PEDIATRICS (doi:10.1542/10.1542/peds.2007-2758)

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ARTICLE

Spectrum of Pediatric Neuromyelitis Optica

Timothy E. Lotze, MD^a,b, Jennifer L. Northrop, MD, PhD^c, George J. Hutton, MD^b, Benjamin Ross, MD^b, Jade S. Schiffman, MD^d and Jill V. Hunter, MD^e

^a Section of Child Neurology, Department of Pediatrics, Texas Children's Hospital, Houston, Texas
^b Departments of Neurology
^c Molecular and Human Genetics
^e Radiology, Baylor College of Medicine, Houston, Texas
^d Section of Ophthalmology, Department of Head and Neck Surgery, University of Texas M.D. Anderson Cancer Center, Houston, Texas

OBJECTIVE. Our goal was to describe the spectrum of clinical phenotypes, laboratory and imaging features, and treatment in pediatric patients with neuromyelitis optica.

PATIENTS AND METHODS. The study consisted of a retrospective chart review of patients followed in a pediatric multiple sclerosis center with a diagnosis of neuromyelitis optica spectrum disorder.

RESULTS. Nine patients with neuromyelitis optica spectrum disorders were included, all of whom were female. There were 4 black children, 2 Latin American children, 2 white children, and 1 child of mixed Latin American/white heritage. Median age at initial attack was 14 years (range: 1.9–16 years). Median disease duration was 4 years (range: 0.6–9 years). Tests for neuromyelitis optica immunoglobulin G were positive for 7 patients. Eight patients had transverse myelitis and optic neuritis, and 1 patient had longitudinally extensive transverse myelitis without optic neuritis but had a positive neuromyelitis optica immunoglobulin G antibody titer. Cerebral involvement on MRI was found in all subjects, 5 of whom were symptomatic with encephalopathy, seizures, hemiparesis, aphasia, vomiting, or hiccups. Immunosuppressive therapy reduced attack frequency and progression of disability.

CONCLUSIONS. Pediatric neuromyelitis optica has a diverse clinical presentation and may be difficult to distinguish from multiple sclerosis in the early stages of the disease. The recognition of the broad spectrum of this disease to include signs and symptoms of brain involvement is aided by the availability of a serum biomarker: neuromyelitis optica immunoglobulin G. Early diagnosis and immunosuppresive treatment may help to slow the accumulation of severe disability.

Key Words: neuromyelitis optica • multiple sclerosis • central nervous system • encephalomyelitis • spinal cord

Abbreviations: NMO—neuromyelitis optica • CNS—central nervous system • MS—multiple sclerosis • ON—optic neuritis • TM—transverse myelitis • IgG—immunoglobulin G • AQP4—aquaporin 4 • LETM—longitudinally extensive transverse myelitis • SIADH— syndrome of inappropriate antidiuretic hormone secretion • FANA—fluorescent antinuclear antibody • EDSS—Expanded Disability Status Scale • FLAIR—fluid attenuated inversion recovery • IVIg—intravenous immunoglobulin • CSF—cerebrospinal fluid • WBC—white blood cell • dsDNA—double-stranded DNA

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Accepted Jul 15, 2008.

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