Intractable vomiting as the initial presentation of neuromyelitis optica

Apiwattanakul, Metha; Popescu, Bogdan F. Gh.; Matiello, Marcelo; Weinshenker, Brian G.; Lucchinetti, Claudia F.; Lennon, Vanda A.; McKeon, Andrew; Carpenter, Adam F.; Miller, Gary M.; Pittock, Sean J.

doi:10.1002/ana.22121

Cited by 184 publications

(177 citation statements)

References 21 publications

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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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“…It has been now well established that brain symptoms are not only frequent during disease course (Table 5), but may antedate optic neuritis or acute transverse myelitis for months or years 17,29 . Incoercible vomiting and hiccups result from involvement of the area postrema at the floor of the fourth ventricle.…”

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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“…9 Area postrema, the emetic reflex center, 12 is comprised of 2 symmetric structures at the floor of the rhomboid fossa. Like other circumventricular organs (CVO), it consists of loose tissue containing glia and neurons, has a thin ependymal cover, and is penetrated by convoluted capillaries that lack tight endothelial junctions forming a permeable bloodbrain barrier (BBB).…”

mentioning

confidence: 99%

Neuromyelitis optica unique area postrema lesions

Popescu

Lennon²,

Parisi³

et al. 2011

Neurology

276

218

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Objective: To characterize the neuropathologic features of neuromyelitis optica (NMO) at the medullary floor of the fourth ventricle and area postrema. Aquaporin-4 (AQP4) autoimmunity targets this region, resulting in intractable nausea associated with vomiting or hiccups in NMO.Methods: This neuropathologic study was performed on archival brainstem tissue from 15 patients with NMO, 5 patients with multiple sclerosis (MS), and 8 neurologically normal subjects. Logistic regression was used to evaluate whether the presence of lesions at this level increased the odds of a patient with NMO having an episode of nausea/vomiting.Results: Six patients with NMO (40%), but no patients with MS or normal controls, exhibited unilateral or bilateral lesions involving the area postrema and the medullary floor of the fourth ventricle. These lesions were characterized by tissue rarefaction, blood vessel thickening, no obvious neuronal or axonal pathology, and preservation of myelin in the subependymal medullary tegmentum. AQP4 immunoreactivity was lost or markedly reduced in all 6 cases, with moderate to marked perivascular and parenchymal lymphocytic inflammatory infiltrates, prominent microglial activation, and in 3 cases, eosinophils. Complement deposition in astrocytes, macrophages, and/or perivascularly, and a prominent astroglial reaction were also present. The odds of nausea/ vomiting being documented clinically was 16-fold greater in NMO cases with area postrema lesions (95% confidence interval 1.43-437, p ϭ 0.02).

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Intractable vomiting as the initial presentation of neuromyelitis optica

Cited by 184 publications

References 21 publications

Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis

Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis

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

Neuromyelitis optica unique area postrema lesions

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