Area postrema syndrome: a neurological presentation of nausea, vomiting and hiccups

Chan, Ka Hong; Vorobeychik, Galina

doi:10.1136/bcr-2020-238588

Cited by 6 publications

(6 citation statements)

References 11 publications

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“…Typically referred to as the brainstem's "vomiting center, " it also plays other roles in the control of vasomotor/ angiotensin II, somatic development, and hunger. [3] APS is described as at least 48 h of intractable nausea, vomiting, or hiccups and is included as a core clinical criterion for NMOSD. [1] Unlike spinal and optic lesions seen in classical NMO, lesions in the AP usually demonstrate more inflammation than demyelination and necrosis, thus explaining the potential for complete recovery following treatment.…”

Section: Discussionmentioning

confidence: 99%

“…Typically referred to as the brainstem’s “vomiting center,” it also plays other roles in the control of vasomotor/ angiotensin II, somatic development, and hunger. [ 3 ]…”

Section: Discussionmentioning

confidence: 99%

“…[ 1 ] Area postrema syndrome (APS) is a rare neurological condition that manifests as uncontrollable episodes of nausea, vomiting, or hiccups. [ 2 , 3 ] It is easy to mistake APS, which manifests at the outset of NMOSD, for a gastrointestinal (GI) disease. Here, we would like to report a similar presentation in a 25-year-old female, who underwent extensive GI and other evaluation for intractable vomiting and hiccups and was confirmed to have APS on magnetic resonance imaging (MRI).…”

Section: Introductionmentioning

confidence: 99%

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Area postrema syndrome: An unusual presentation of neuromyelitis optica spectrum disorder

Raj¹,

Valappil²,

Alapatt³

et al. 2023

JNRP

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Isolated area postrema syndrome (APS) is a rare neurological presentation of, neuromyelitis optica spectrums disorder (NMOSD), recognizable by uncontrollable hiccups, nausea, or vomiting. When it occurs as the first presentation of NMOSD, it may present as a diagnostic challenge as the condition may be frequently attributed to gastrointestinal pathology, and the subsequent diagnostic delay may result in debilitating neurological sequelae such as optic neuritis or myelitis. We report such a case of isolated APS in a young woman who presented with a clinical picture of bouts of vomiting and intractable hiccups causing considerable distress and was finally diagnosed to be a case of seronegative NMOSD.

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Section: Discussionmentioning

confidence: 99%

“…Typically referred to as the brainstem’s “vomiting center,” it also plays other roles in the control of vasomotor/ angiotensin II, somatic development, and hunger. [ 3 ]…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Area postrema syndrome: An unusual presentation of neuromyelitis optica spectrum disorder

Raj¹,

Valappil²,

Alapatt³

et al. 2023

JNRP

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“…Patients with NMOSD may present with gastrointestinal symptoms. Area postrema syndrome is a characteristic subtype of NMOSD and occurs when demyelinating lesions are present in the area postrema of the dorsal medulla which may lead to nausea, vomiting, and hiccups ( 4 ). Up to 30% of patients with NMOSD present with typical brainstem lesion symptoms ( 5 ).…”

Section: Discussionmentioning

confidence: 99%

Neuromyelitis Optica Spectrum Disorder Manifested by Persistent Hiccups and Severe Esophagitis in an Adolescent Patient

2021

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Patients with persistent hiccups are often referred to gastrointestinal services. Hiccups lasting greater than 48 hours are classified as persistent, while those lasting longer than 2 months are termed intractable. The etiology of hiccups is broad and can include many organ systems. Here, we present the case of an 18-year-old male patient who presented to the emergency department with an 8-day history of nausea, emesis, and intermittent hiccups. The patient was admitted to the gastroenterology service and underwent workup including esophagogastroduodenoscopy and imaging. Esophagogastroduodenoscopy revealed severe distal esophagitis. He was started on high-dose proton pump inhibitor and sucralfate. During the course of hospital admission, the patient's intermittent hiccups worsened to become constant. Several medical therapies led to little improvement. We initially felt that the esophagitis was the etiology of the patient's symptoms, however, due to ongoing symptoms unresponsive to typical therapy, we obtained a neurological consult. Brain MRI revealed a dorsal medullary lesion. Subsequent work up confirmed the diagnosis of neuromyelitis optica spectrum disorder. Neuromyelitis optica spectrum disorder is a rare, serious, sometimes fatal autoimmune condition of the central nervous system. Early diagnosis and treatment are important predictors of future relapses. Our patient was started on high-dose intravenous steroids with rapid improvement in symptoms. He was discharged home on steroids, proton pump inhibitor and close neurology and gastroenterology follow up. This case outlines the importance of consideration of neurological causes of hiccups or other centrally mediated conditions with gastrointestinal manifestations, even when a concomitant gastrointestinal condition is present.

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“…Most studies of NMOSD have focused on the classic opticospinal presentations, while only few outlined other presentations including APS and were mainly case reports [11][12][13][14][15][16][17][18][19][20][21]. By conducting this study, we aimed to investigate the frequency and diagnostic challenges in NMOSD patients with APS from the south of Egypt with a description of their clinical and radiological features.…”

Section: Introductionmentioning

confidence: 99%

Area postrema syndrome in neuromyelitis optica spectrum disorder: diagnostic challenges and descriptive patterns

Khedr

Farweez

El-Fetoh

et al. 2021

Egypt J Neurol Psychiatry Neurosurg

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Background Although area postrema syndrome (APS) is one of the core clinical features of neuromyelitis optic spectrum disorder (NMOSD), it is frequently misdiagnosed as gastrointestinal or systemic disorders. In this study, we describe the diagnostic challenges in NMOSD patients with APS and their characteristic clinical and radiological features. All patients who attended our university hospitals during the period from March 2019 to August 2020 with a diagnosis of NMOSD according to the latest diagnostic criteria were admitted and evaluated clinically, radiologically with gadolinium-enhanced brain and spinal MRI, measures of serum Anti-Aquaporin 4 (Anti-AQP4) and clinical status using the Expanded Disability Status Scale (EDSS) scores. APS was diagnosed if there was a history of intractable nausea, vomiting, or hiccups (INVH) that had lasted longer than 1 week with the exclusion of other etiologies, or less than 48 h if associated with a lesion in the dorsal medulla on MRI scan. Results Twenty out of 90 (22.2%) identified patients with a diagnosis of NMOSD had a history of unexplained intractable nausea, vomiting or hiccoughs lasting an average of 20 days. Seventeen patients were anti-Aquaporin 4 seropositive. Seven patients (35%) presented initially with isolated clinical features of APS and were diagnosed only after subsequent relapse. Patients with APS preceding other core clinical presentations (13 cases, 65%) were diagnosed after development of motor manifestations. All patients developed acute myelitis during the course of illness. Brain and spinal MRI scans showed that 13 had a linear lesion in the dorsal tegmentum of the medulla oblongata adjacent to the fourth ventricle. Otherwise, longitudinally extensive transverse myelitis was found in 80%, while 35% showed extension of the cord lesion to the AP. Conclusions APS as a core clinical characteristic of NMOSD is not a rare presentation as was previously thought and can occur in both AQP4-seropositive and seronegative NMOSD.

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Area postrema syndrome: a neurological presentation of nausea, vomiting and hiccups

Cited by 6 publications

References 11 publications

Area postrema syndrome: An unusual presentation of neuromyelitis optica spectrum disorder

Area postrema syndrome: An unusual presentation of neuromyelitis optica spectrum disorder

Neuromyelitis Optica Spectrum Disorder Manifested by Persistent Hiccups and Severe Esophagitis in an Adolescent Patient

Area postrema syndrome in neuromyelitis optica spectrum disorder: diagnostic challenges and descriptive patterns

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