Atypical presentations of neuromyelitis optica

Sato, Douglas Kazutoshi; Fujihara, Kazuo

doi:10.1590/s0004-282x2011000600019

Cited by 35 publications

(20 citation statements)

References 23 publications

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“…The patients with AQP4 antibodies frequently had cervical lesions, including some extending to the medullary region, whereas patients with MOG antibodies more frequently had long lesions extending to the lumbar spinal cord. Brainstem symptoms, such as persistent nausea/vomiting and hiccups, as previously reported in NMOSD, 9,10 were more commonly found in the AQP4 antibody group than in the MOG antibody or seronegative groups. Typical pathologic NMO lesions in the area postrema have been found in postmortem specimens of AQP4 Figure 2 Recurrent longitudinally extensive transverse myelitis in a patient with MOG antibodies Sagittal T2-weighted MRI of the spinal cord from a 6-year-old girl with recurrent longitudinally extensive transverse myelitis and myelin oligodendrocyte glycoprotein (MOG) antibodies (titer 1:1,024) shows 2 longitudinally extensive lesions.…”

Section: Resultssupporting

confidence: 76%

“…All patients with MOG antibodies (6/6), all patients who were seronegative (43/43), and 89.1% (114/128) of patients with AQP4 antibodies had lesions covering 3 or more vertebral segments in the sagittal spinal cord MRI. The median (range) number of vertebral segments of the longest spinal cord lesion was 6.5 (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14) in patients with MOG antibodies, 6 (1-19) in patients with AQP4 antibodies, and 4.5 (3-19) in seronegative patients (p 5 0.3658). The involvement of the central portion of the spinal cord in the axial spinal cord MRI was found in 66.7% (4/6) of patients with MOG antibodies, 89.8% (115/128) of patients with Figure 1 Bilateral optic neuritis in a patient with MOG antibodies Axial short TI inversion recovery (STIR) and T1-weighted with gadolinium MRI of the optic nerves from a 28-year-old man with bilateral simultaneous optic neuritis and myelin oligodendrocyte glycoprotein (MOG) antibodies (titer 1:4,096) shows bilateral STIR hyperintense lesions with contrast enhancement.…”

Section: Resultsmentioning

confidence: 97%

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Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders

Mao¹,

Sato²,

Liu³

et al. 2014

Neurology

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Objective: To evaluate clinical features among patients with neuromyelitis optica spectrum disorders (NMOSD) who have myelin oligodendrocyte glycoprotein (MOG) antibodies, aquaporin-4 (AQP4) antibodies, or seronegativity for both antibodies.Methods: Sera from patients diagnosed with NMOSD in 1 of 3 centers (2 sites in Brazil and 1 site in Japan) were tested for MOG and AQP4 antibodies using cell-based assays with live transfected cells. Neuromyelitis optica (NMO) is characterized by severe attacks of optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM) with 3 or more vertebral segment spinal cord lesions observed on MRI.1 Limited forms of the disease are known as NMO spectrum disorders (NMOSD). NMOSD currently include patients with either ON or LETM (single or recurrent events of LETM or recurrent or simultaneous bilateral ON).2 Approximately 90% of the patients with NMO and more than half of the patients with NMOSD are positive for autoantibodies against aquaporin-4 (AQP4).3,4 Therefore, a proportion of patients with NMO or NMOSD remains AQP4 antibody-negative despite the use of the best assays available on serum samples collected during an acute attack before any treatment.Recently, autoantibodies against myelin oligodendrocyte glycoprotein (MOG) were reported in 4 patients who were clinically diagnosed with NMOSD and negative for AQP4 antibodies.5 High-titer MOG antibodies are predominantly of the immunoglobulin G (IgG) 1 subtype and efficiently mediate complement-dependent cytotoxicity in vitro.6 However, none of these previous studies investigated comprehensively the features that may distinguish patients with AQP4 antibodies from those with high-titer MOG antibodies or those who are negative for both antibodies, evenFrom the

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Section: Resultssupporting

confidence: 76%

Section: Resultsmentioning

confidence: 97%

Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders

Mao¹,

Sato²,

Liu³

et al. 2014

Neurology

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“…Sato and Fujihara 5 found that almost 20% of the patients had at least one short period of IH, and numerous studies on NMO have confirmed these results. 1,2,6-8 A previous study suggested that the afferent nerves of the hiccup reflex include vagus, phrenic, and sympathetic nerves of T6-T10.…”

Section: Discussionmentioning

confidence: 86%

Intractable hiccup caused by spinal cord lesions in demyelination disease

Hao¹,

Wang²,

Yan³

et al. 2013

The Journal of Spinal Cord Medicine

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Objective: This study aimed to summarize the clinical features of patients who presented intractable hiccup (IH) without brain and medulla oblongata (MO) lesions. Method: This study included six patients who were diagnosed with inflammatory demyelinating myelitis, categorized as neuromyelitis optica (NMO), multiple sclerosis (MS), and myelitis. Patients who presented IH with cervical lesions but without MO lesions were also included. Clinical profiles, laboratory data, and magnetic resonance imaging findings were analyzed. Results: Three out of six patients were diagnosed with NMO, whereas the remaining three were diagnosed with acute myelitis, recurrent myelities, and MS, respectively. The duration of hiccup was from 2 to 23 days (average = 9.33 ± 8.64 days). Five patients (83.33%, patients 1-5) had long segmental lesions and one had a patchy lesion. None of these patients had any MO lesions. Half of them were successfully treated with high-dose methylprednisolone combined with gamma-aminobutyric acid (GABA) inhibitor. Conclusion: IH occurred in patients without MO lesion. However, the mechanism remained unclear. Immune factors of demyelinating neuropathy stimulated the hiccup reflex arch. Cervical cord lesions may activate the hiccup center. In general, IH can be controlled by IVMP combined with GABA inhibitor. Unilateral phrenic nerve block may elicit no effect. Keywords: Intractable hiccup, Demyelinating disease, Myelitis, Multiple Sclerosis, Neuromyelitis opticaHiccup is a repetitive, involuntary, spasmodic, and temporary contraction of the diaphragm accompanied by sudden closure of the glottis.1 In general, the hiccup and vomiting centers are located in the medulla oblongata (MO). 2 Hiccup and vomiting are common symptoms of neuromyelitis optica (NMO), which have been also reported in some cases of multiple sclerosis (MS). Reports on intractable hiccup (IH) without MO lesions are rare, but cervical spinal lesions can also cause hiccup. However, the mechanism remains unclear. In this study, six patients with different diseases, all of whom presented with IH without MO lesion found in the magnetic resonance imaging (MRI) scan, were investigated. To our knowledge, this report is one of a few reports about such condition. We summarized the clinical features of these patients and provided a brief review of literature. MethodsIH is characterized by hiccup and nausea that last for more than 48 hours 2 even with common therapeutic management. Inflammatory demyelinating diseases, including NMO, MS, as well as acute and recurrent myelitis were considered in this study. The diagnosis of NMO was based on the revised diagnostic criteria, 3 whereas the diagnosis of MS

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“…Decreased mental status, impairment of consciousness, confusion and seizures have been reported as presenting symptoms of AQP4 autoimmunity. Optic neuritis or myelitis followed the cerebral symptoms at a variable time interval ranging from 3 to 110 months 41,46,47 .…”

Section: Requirement Of Optic Neuritis or Myelitis Presence As A Pitfmentioning

confidence: 99%

The expanded spectrum of neuromyelitis optica: evidences for a new definition

Lana-Peixoto

Callegaro

2012

Arq. Neuro-Psiquiatr.

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Neuromyelitis optica (NMO) has been traditionally described as the association of recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM). Identification of aquaporin-4 antibody (AQP4-IgG) has deeply changed the concept of NMO. A spectrum of NMO disorders (NMOSD) has been formulated comprising conditions which include both AQP4-IgG seropositivity and one of the index events of the disease (recurrent or bilateral optic neuritis and LETM). Most NMO patients harbor asymptomatic brain MRI lesions, some of them considered as typical of NMO. Some patients with aquaporin-4 autoimmunity present brainstem, hypothalamic or encephalopathy symptoms either preceding an index event or occurring isolatedly with no evidence of optic nerve or spinal involvement. On the opposite way, other patients have optic neuritis or LETM in association with typical lesions of NMO on brain MRI and yet are AQP4-IgG seronegative. An expanded spectrum of NMO disorders is proposed to include these cases.

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Atypical presentations of neuromyelitis optica

Cited by 35 publications

References 23 publications

Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders

Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders

Intractable hiccup caused by spinal cord lesions in demyelination disease

The expanded spectrum of neuromyelitis optica: evidences for a new definition

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