NMO in pediatric patients: brain involvement and clinical expression

Peña, Joaquín; Ravelo, María Elena; Cruz, Eduardo Mora-La; Montiel-Nava, Cecilia

doi:10.1590/s0004-282x2011000100008

Cited by 23 publications

(20 citation statements)

References 23 publications

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“…Also, it is possible that older children might present more clinical episodes which helps clarify the diagnosis of MS. Most of the patients were mestizos and white non-caucasian, probably reflecting the ethnic characteristics of our country 20 . We found a high rate of motor impairment and brainstem/cerebellum signs at disease manifestation about 30% each, followed by sensory symptoms and optic neuritis (10%).…”

Section: Discussionmentioning

confidence: 87%

Pediatric multiple sclerosis in Venezuela

Peña

Ravelo²,

E³

et al. 2012

Arq. Neuro-Psiquiatr.

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OBJECTIVE: To describe the epidemiological and clinical characteristics of Venezuelan pediatric patients with multiple sclerosis (MS). METHODS: Database records from the National Program for MS were searched for patients with an established diagnosis of MS whose first symptoms appeared before age 18. RESULTS: The national database held records of 1.710 patients; 3.8% had onset of the first symptoms before age 18. 46.7% were boys, yielding an F:M ratio of 1.13:1. Many children had a disease onset characterized by motor impairment (30.7%), brainstem/cerebellum and spinal cord affectation (27.6%), headache (26%). Less frequent symptoms were sensory symptoms (8%) and optic neuritis (7%). DISCUSSION: Pediatric MS patients in Venezuela represent a significant proportion of all MS cases. The clinical pattern is characterized by motor symptoms at onset, and predominantly monosymptomatic presentation with a relapsing-remitting pattern. This is the first systematic attempt to estimate the prevalence of pediatric MS in Venezuela.

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Section: Discussionmentioning

confidence: 87%

Pediatric multiple sclerosis in Venezuela

Peña

Ravelo²,

E³

et al. 2012

Arq. Neuro-Psiquiatr.

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“…4 NEUROIMAGING FINDINGS IN CHILDREN According to previous reports, brain lesions appear more often in pediatric-onset anti-AQP4 antibody-positive NMOSD compared to adult-onset cases. 4,[17][18][19][20] These brain lesions are usually large (.2 cm) 18 and tend to localize to areas of high AQP4 expression such as periventricular regions of the III (diencephalic) and IV ventricles (brainstem), supratentorial and infratentorial white matter, midbrain, and cerebellum. 4 Gadolinium enhancement can be seen in around one-third of patients with brain lesions, 4,17 often with a cloud-like pattern of enhancement.…”

Section: Demographics and Epidemiologymentioning

confidence: 99%

Neuromyelitis optica spectrum disorders in children and adolescents

Tenembaum¹,

Chitnis²,

Nakashima³

et al. 2016

Neurology

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Neuromyelitis optica (NMO) is a severe autoimmune disease of the CNS characterized by recurrent inflammatory events primarily involving the optic nerves and spinal cord. NMO is infrequent in children, but early recognition is important to start adequate treatment. In this article, we review the evolving diagnostic criteria of NMO and provide an update on the clinical and neuroimaging spectrum of the disorder in pediatric patients, including current knowledge on immunopathogenesis and treatment recommendations for children with NMO. Although neuromyelitis optica (NMO) was previously considered a subtype of multiple sclerosis (MS), the identification of autoimmune antibodies (NMO-immunoglobulin G [IgG]) targeted against the aquaporin-4 (AQP4) water channel in the majority of adult patients identified NMO as a different autoimmune disease entity. 1,2The high specificity of AQP4-IgG for NMO has allowed the identification of seropositive patients with atypical presentations of the disease: NMO spectrum disorders (NMOSD). 3Although adults are predominantly affected, NMO/NMOSD has been increasingly recognized in children, who demonstrate a spectrum of disease attacks that often involve CNS areas beyond the optic nerves and spinal cord. 4 A comprehensive update on NMO/NMOSD in the pediatric population is provided in this article. DEMOGRAPHICS AND EPIDEMIOLOGYThe mean age at NMO presentation ranges from 32 to 45 years in major case series. Population-based studies from Europe, Asia, and South America suggest that the incidence of NMO ranges from 0.05 to 4/100,000/y and the prevalence from 0.52 to 4.4/100,000. 5 Nevertheless, most of these studies have not stratified data by pediatric or adult age at onset.5 Pediatric onset is relatively rare.In a comprehensive prevalence study from Cuba, a country with a multiracial population, NMO onset prior to the age of 20 years constituted 3.4% of all NMO cases with an overall prevalence rate of 0.12/100,000 persons. 6 In this study, diagnosis was based on the Wingerchuk et al. 7 1999 criteria, in which AQP4 antibody testing was not included.In a large study of patient samples collected at the Mayo Clinic, 5% of AQP4 antibody-positive patients were children (,18 years), with a mean age at clinical onset of 12 years, a clear female preponderance (7:1), mixed ethnic background, and a poorer prognosis compared with childhood MS. 4 In 3 prospective studies of children with a first demyelinating syndrome, 0.6% (2/302), 8 3.5% (3/86), 9 and 3.7% (3/81) 10 of patients were identified as having NMO over 3-6 years of follow-up. A similar study on 44 children with optic neuritis (ON) reported a final diagnosis of NMO in 7% of patients.

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“…It is believed to be a consequence of an autoimmune reaction to myelin triggered by a virus or vaccination. 49 NMO can be a fulminant monophasic disease associated with varying degrees of recovery, or a polyphasic disease with multiple relapses and clinical remissions. Relapsing forms of ADEM can be very difficult to distinguish from MS.…”

Section: Epidemiologymentioning

confidence: 99%

“…Relapsing forms of ADEM can be very difficult to distinguish from MS. 49 In 60% of patients a relapse will occur within the first year of the initial attack and in 90% of patients a relapse will occur within the first 3 years of presentation. Recurrent ADEM is characterized by a second episode after 3 months of the initial neurological event involving the same neuroanatomic area.…”

Section: Epidemiologymentioning

confidence: 99%