Intractable hiccup and nausea with periaqueductal lesions in neuromyelitis optica

Misu, Tatsuro; Fujihara, Kazuo; Nakashima, Ichiro; Satō, Shigeru; Itoyama, Yasuto

doi:10.1212/01.wnl.0000183151.19351.82

Cited by 310 publications

(242 citation statements)

References 9 publications

(17 reference statements)

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“…AP syndrome should be considered in any episode of otherwise unexplained and intractable nausea, emesis or singultus, especially in combination with neurological deficits indicating brainstem dysfunction. We recommend contrast‐enhanced brain MRI, CSF analysis, neurophysiological examination in addition to anti‐ganglioside and anti‐AQP‐4‐IgG antibody testing, the latter to rule out NMO/NMOSD as a common trigger for AP syndrome 12, 13, 14, 15. Besides causal treatment, a multimodal anti‐emetic concept is crucial for the management of AP syndrome.…”

Section: Discussionmentioning

confidence: 99%

Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis

Zeiner

Brandhofe

Müller–Eschner

et al. 2018

Ann Clin Transl Neurol

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ObjectiveArea postrema (AP) syndrome (defined as: nausea and/or emesis and/or singultus at onset of brainstem dysfunction) comprises complex pathophysiologic mechanisms triggered by different entities. The first objective was to assess the frequency of AP syndrome as a clinical feature in brainstem encephalitis (BE). Finding an especially high prevalence of AP syndrome in Bickerstaff brainstem encephalitis (BBE), we also analyzed the frequency of AP syndrome in other autoimmune diseases with anti‐ganglioside antibodies (Guillain–Barré syndrome (GBS) and its variants).MethodsWe systematically evaluated the prevalence of AP syndrome in BE in all patients treated at our university hospital during a 15‐year period. In a second step, BBE patients were compared to GBS and Miller Fisher syndrome (MFS) patients as clinical subtypes of a disease continuum without brainstem dysfunction.ResultsWe found AP syndrome in 8 of 21 BE patients, including 3 of 7 BBE and in 4 of 112 GBS/MFS patients. AP syndrome was as a frequent but under‐recognized feature of BE with a significant impact on patients’ well being.InterpretationManifestation of AP syndrome in BBE but also in GBS and its subtypes point toward a role of autoimmune antibodies that should be investigated in future studies. Considerable misdiagnosis or nonrecognition complicates diagnostic and therapeutic management. Therefore, AP syndrome should be considered in any episode of otherwise unexplained nausea, emesis, or singultus.

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Section: Discussionmentioning

confidence: 99%

Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis

Zeiner

Brandhofe

Müller–Eschner

et al. 2018

Ann Clin Transl Neurol

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“…In our review of 144 cases of NMO spectrum disorders, 30 patients (21%) had hiccups and 24 (17%) had nausea 18 . In another study, nearly a fifth of NMO patients presented with these symptoms whereas none of MS patients had reported such complains 19 . The episodes resolved spontaneously or with methylprednisolone treatment.…”

Section: Hiccups Nausea and Vomitingmentioning

confidence: 93%

Atypical presentations of neuromyelitis optica

Sato

Fujihara

2011

Arq. Neuro-Psiquiatr.

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Neuromyelitis optica (NMO) is an inflammatory disease of central nervous system classically characterized by acute, severe episodes of optic neuritis and longitudinally extensive transverse myelitis, usually with a relapsing course. The identification of an autoantibody exclusively detected in NMO patients against aquaporin-4 (AQP-4) has allowed identification of cases beyond the classical phenotype. Brain lesions, once thought as infrequent, can be observed in NMO patients, but lesions have different characteristics from the ones seen in multiple sclerosis. Additionally, some AQP-4 antibody positive patients may present with a variety of symptoms not being restricted to optic neuritis and acute myelitis during the first attack or in a relapse. Examples are not limited to, but may include patients only with brain and/or brainstem lesions, narcolepsy with hypothalamic lesions or patients with intractable hiccups, nausea and vomiting. The prompt identification of NMO patients with atypical presentations may benefit these patients with institution of early treatment to reduce disability and prevent further attacks. Key words: neuromyelitis optica, aquaporin 4, myelitis, optic neuritis, diagnosis, differential, nausea, vomiting, hiccup, brain diseases.Apresentações atípicas da neuromielite óptica RESUMO Neuromielite óptica (NMO) é uma doença inflamatória do sistema nervoso central caracterizada classicamente por neurite óptica grave e mielite transversa longitudinalmente extensa, com um curso usualmente recorrente. A identificação do anticorpo detectado exclusivamente nos pacientes com NMO contra a aquaporina-4 (AQP-4) permitiu a identificação de casos além do fenótipo clássico. Lesões cerebrais, que antes eram descritas como infrequentes, podem ser observadas em pacientes com NMO, mas as lesões possuem características diferentes das lesões observadas na esclerose múltipla. Além disso, alguns pacientes positivos para o anticorpo contra a AQP-4 podem apresentar uma variedade de sintomas não restritos à neurite óptica e mielite aguda, seja durante o primeiro ataque seja em uma recorrência. Exemplos não estão limitados aos descritos a seguir, mas incluem pacientes com lesões cerebrais e/ou tronco cerebral, narcolepsia com lesões hipotalâmicas ou pacientes com quadros intratáveis de soluços, náusea e vômitos. A identificação rápida dos pacientes com NMO com apresentações atípicas pode beneficiar estes pacientes com a instituição precoce do tratamento a fim de reduzir a incapacidade e prevenir ataques subsequentes. Palavras-Chave: neuromielite óptica, aquaporina 4, mielite, neurite óptica, diagnóstico diferencial, náusea, vômito, soluço, encefalopatias.

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“…Early reports of hiccups in MS may have conflated MS and NMO, as a recent study found persistent hiccups rare in MS (0/130 cases) but common in NMO (8/47 cases-17%, 88% women) 16 . Indeed, persistent hiccup in demyelinating disease, often heralding an attack, favors a diagnosis of NMO in children 17 as well as adults 18 , with autoimmune disorders being common accompaniments 18 .…”

Section: Discussionmentioning

confidence: 99%

Hiccups due to Central Nervous System Disease: Analysis of 71 Inpatients

Keane

2010

Can. j. neurol. sci.

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Intractable hiccup and nausea with periaqueductal lesions in neuromyelitis optica

Cited by 310 publications

References 9 publications

Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis

Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis

Atypical presentations of neuromyelitis optica

Hiccups due to Central Nervous System Disease: Analysis of 71 Inpatients

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