Brainstem manifestations in neuromyelitis optica: a multicenter study of 258 patients

Kremer, Laurent; Mealy, Maureen A.; Jacob, Anu; Nakashima, Ichiro; Cabre, Philippe; Bigi, Sandra; Paul, Friedemann; Jarius, Sven; Aktaş, Orhan; Elsone, Liene; Mutch, Kerry; Lévy, Michaël; Tokura, Y.; Collongues, Nicolas; Banwell, Brenda; Fujihara, Kazuo; Seze, J. de

doi:10.1177/1352458513507822

Cited by 168 publications

(118 citation statements)

References 34 publications

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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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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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“…A multicentre international study noted brainstem symptoms in 81 of 258 patients (31.4%). 11 Of these, vomiting (33.1%) and hiccups (22.3%) were most common; oculomotor abnormalities (19.8%) and pruritus (12.4%) were also frequently reported. Less commonly reported were hearing loss, facial palsy, and trigeminal neuralgia (2.5% each); vertigo or vestibular ataxia (1.7%); and other cranial nerve abnormalities (3.3%).…”

Section: Discussionmentioning

confidence: 99%

“…[11][12][13] Neuropathic pruritus in neuromyelitis optica may reflect the typical central cord lesions affecting dorsal horn neurones, the dorsal horn being rich in mediators of pruritus,or lesions surrounding periaqueductal grey matter. 14 The AQP4 serum autoantibody, also known as NMOIgG, has a sensitivity of 91% and specificity of 100% for NMO, and is positive in up to 80% of patients.…”

Section: Discussionmentioning

confidence: 99%

Neuropathic pruritus: an early indicator of neuromyelitis optica spectrum disorders

et al. 2018

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Neuromyelitis optica is a relapsing, inflammatory astrocytopathic disorder, affecting predominantly the optic nerves and spinal cord. It is associated with antiaquaporin-4 immunoglobulin G (AQP4-IgG) in up to 70% of patients. Spinal cord involvement typically presents as a longitudinally-extensive transverse myelitis, with associated sensorimotor and sphincter dysfunction. Sensory symptoms such as numbness, dysaesthesia, pain and tonic spasms are common. Here, we present a case of a 25years old female who came to the medicine OPD, with the chief complaints of intense itching over face and forehead, which was later on progressed to quadriparesis after 3 weeks. This case highlights neuropathic pruritus as an under-recognised early feature of neuromyelitis optica.

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“…Erschwert ist die Diagnosestellung aber bei Patienten mit inkompletter oder atypischer Präsentation (vor allem bei isolierter Optikusneuritis, isolierter langstreckiger Myelitis und Hirnstammenzephalitis; siehe . Infobox 1; [1,15,37]). …”

Section: Diagnosekriterien Der Neuromyelitis Opticaunclassified

Biopsie bei tumorverdächtiger spinaler Raumforderung: Fallstrick Neuromyelitis optica

et al. 2014

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Considering possible serious sequelae of spinal biopsy procedures, testing for AQP4-ab is mandatory in patients with unclear longitudinally extensive spinal cord lesions and should be performed preoperatively in all cases. In light of the heterogeneity of available assays, different detection methods should be used in doubtful cases. The relationship between NMO and APS needs further clarification; however, AQP4 IgG testing is recommended in patients presenting with APS and myelitis, optic neuritis or brainstem encephalitis.

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Brainstem manifestations in neuromyelitis optica: a multicenter study of 258 patients

Abstract: This study confirms the high frequency of brainstem symptoms in NMO with a majority of vomiting and hiccups. The prevalence of these manifestations was higher in the non Caucasian population.

Cited by 168 publications

References 34 publications

Neuromyelitis optica spectrum disorders in children and adolescents

Neuromyelitis optica spectrum disorders in children and adolescents

Neuropathic pruritus: an early indicator of neuromyelitis optica spectrum disorders

Biopsie bei tumorverdächtiger spinaler Raumforderung: Fallstrick Neuromyelitis optica

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