Isolated Area Postrema Syndrome Presenting as Intractable Nausea and Vomiting

Dandu, Vasuki; Siddamreddy, Suman; Meegada, Sreenath; Muppidi, Vijayadershan; Challa, Tejo

doi:10.7759/cureus.7058

Cited by 4 publications

(4 citation statements)

References 21 publications

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“…On the other hand, although APS is considered highly specific for AQP4-seropositivity [14,19,32,[46][47][48] APS in AQP4-seronegative patients (including patients with anti-Myeline oligodendrocyte glycoprotein "MOG"-IgGs) are being increasingly recognized [39]. Three AQP4-seronegative cases were identified in the present study (15%) and tested positive for anti-MOG.…”

Section: Discussionmentioning

confidence: 64%

“…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%

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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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Section: Discussionmentioning

confidence: 64%

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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“…In a neuropathologic study, Popescu et al demonstrated that the area postrema was a selective target of disease processes in patients with NMOSD and suggested that patients with lesions in this area were more likely to present with nausea and vomiting [ 7 ]. Some case studies reported patients with unexplained nausea and vomiting who were later diagnosed with NMOSD [ 8 – 12 ]. The reported frequency of isolated APS at the time of NMOSD onset is 7.1% to 14% [ 1 , 10 ].…”

Section: Discussionmentioning

confidence: 99%

A 51-Year-Old Woman with Advanced Breast Cancer and a 2-Week History of Nausea and Vomiting Due to Neuromyelitis Optica Spectrum Disorder (NMOSD) and Seropositivity to Aquaporin-4 (AQP-4)

Miyagishima,

Anezaki,

Fukuda

et al. 2023

Am J Case Rep

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Patient: Female, 51-year-old Final Diagnosis: Breast cancer • neuromyelitis optica spectrum disorder Symptoms: Nausea and vomiting Clinical Procedure: — Specialty: Neurology Objective: Rare coexistence of disease or pathology Background: Neuromyelitis optica spectrum disorder (NMOSD) is a demyelinating disease of the central nervous system that includes the triad of transverse myelitis, optic neuritis, and area postrema syndrome (APS), characterized by intractable nausea and vomiting. NMOSD can be part of a paraneoplastic syndrome and is associated with seropositivity to aquaporin-4 (AQP-4). We present a patient with uncontrollable nausea and vomiting who developed herpes zoster and acute myelitis and was finally diagnosed with paraneoplastic NMOSD due to breast cancer. Case Report: A 51-year-old woman was hospitalized due to 2 weeks of intractable nausea and vomiting. Although contrast-enhanced thoracoabdominal computed tomography (CT) on day 4 suggested breast cancer in her left breast, the etiology of her symptoms remained unknown. On day 13, she developed herpes zoster, followed by acute myelitis on day 25. Magnetic resonance imaging (MRI) showing longitudinal extensive transverse myelitis and an elevated serum AQP-4 antibody level led to the diagnosis of NMOSD. Brain MRI detected a small lesion in the dorsal medulla oblongata, which explained the preceding APS. After starting intravenous methylpredniso-lone pulse therapy, her nausea and vomiting rapidly subsided. Breast cancer was resected on day 63, and immunohistochemical staining revealed overexpression of AQP-4 in the tumor cells, suggesting paraneoplastic NMOSD. Conclusions: This report has highlighted the presentation and diagnosis of NMOSD and supports the possibility that this can present as part of a paraneoplastic syndrome. In addition, diagnosis of NMOSD preceded by APS requires meticulous history taking and careful interpretation of MRI in the dorsal medulla oblongata.

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“…Neuromyelitis optica spectrum disorder is a rare, serious autoimmune condition with significant morbidity and mortality. The disorder is characterized by demyelination and axonal damage that typically involve the optic nerve or spinal cord ( 1 , 2 ). NMOSD is typically associated with autoantibodies that target aquaporin-4, an abundant water channel in the central nervous system ( 1 ).…”

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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Isolated Area Postrema Syndrome Presenting as Intractable Nausea and Vomiting

Cited by 4 publications

References 21 publications

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

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

A 51-Year-Old Woman with Advanced Breast Cancer and a 2-Week History of Nausea and Vomiting Due to Neuromyelitis Optica Spectrum Disorder (NMOSD) and Seropositivity to Aquaporin-4 (AQP-4)

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

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