Area postrema syndrome as initial manifestation in neuromyelitis optica spectrum disorder patients: A retrospective study

Zhou, Chenguang; Liao, Linghui; Sun, Rong; Wang, J.; Di, Weiying; Zhu, Yinghui; He, Yingkun

doi:10.1016/j.neurol.2020.07.019

Cited by 14 publications

(25 citation statements)

References 31 publications

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“…APS is also reported to occur in other neurologic disorders such as anti-glial fibrillary acidic protein encephalomyelitis and Bickerstaff brainstem encephalitis. 5 Area postrema is highly rich in AQP4 water channel, so it is usually involved in AQP4 antibody-positive individual. There are only few cases reported in literature showing MOG-positive/AQP4-negative individuals with APS.…”

Section: Discussionmentioning

confidence: 99%

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Area Postrema Syndrome

et al. 2022

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Abstract:Objectives:Area postrema syndrome (APS) is one of the core clinical feature of neuromyelitis optic spectrum disorder (NMOSD). APS is mostly associated with neuromyelitis optica (NMO) and rarely reported in myelin oligodendrocyte glycoprotein antibody disease (MOGAD). We herein report a case of area postrema syndrome as the initial presentation of double seropositive aquaporin-4 (AQP-4) and myelin oligodendrocyte glycoprotein (MOG)antibody.Methods:The patient fulfilled the NMOSD diagnostic criteria. MRI brain, CSF studies, electrophysiological test and serum NMO and MOG antibody testing were done.Results:An elderly female initially presented in Gastroenterology outpatient department with a history of nausea, vomiting and hiccups for 3 weeks. Detailed medical evaluation including upper gastrointestinal endoscopy was normal with no improvement with symptomatic therapy. Neurological examination showed bilateral nystagmus, postural imbalance and gait ataxia. MRI brain showed T2/FLAIR hyperintensity in dorsal medulla involving area postrema. Both anti NMO and MOG antibodies were found to be positive in serum. She was treated with intravenous methyl prednisolone with complete symptomatic resolution.Discussion:Double seropositive APS-onset NMOSD has not been previously reported in literature. Early diagnosis and treatment results in resolution of APS- related symptoms and prevents further progression of disease.

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Section: Discussionmentioning

confidence: 99%

“…APS is also reported to occur in other neurologic disorders such as anti-glial fibrillary acidic protein encephalomyelitis and Bickerstaff brainstem encephalitis. 5…”

Section: Discussionmentioning

confidence: 99%

Area Postrema Syndrome

et al. 2022

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“…Coincidentally, AQP4-IgG-seronegative NMOSD with isolated APS onset was recently found in a retrospective study, but this hypothesis has rarely been discussed. 21 Assuming that false-negative AQP4-IgG results due to inappropriate methodology or immunosuppressive treatment can be excluded, the following explanations may account for the negative AQP4-IgG serology in patients with APS onset. Firstly, patients with APS onset (in particular isolated APS) usually present to a department of gastroenterology or psychiatry instead of a department of neurology, which can lead to substantial delays in diagnosis as well as in the performing of AQP4-IgG serological tests.…”

Section: Discussionmentioning

confidence: 99%

Disease Course and Outcomes in Patients With the Limited Form of Neuromyelitis Optica Spectrum Disorders and Negative AQP4-IgG Serology at Disease Onset: A Prospective Cohort Study

Chen

Zhou

et al. 2022

J Clin Neurol

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Background and Purpose Patients presenting with clinical characteristics that are strongly suggestive of neuromyelitis optica spectrum disorders (NMOSD) have a high risk of developing definite NMOSD in the future. Little is known about the clinical course, treatment, and prognosis of these patients with likely NMOSD at disease onset. Methods This study prospectively recruited and visited 24 patients with the limited form of NMOSD (LF-NMOSD) at disease onset from November 2012 to June 2021. Their demographics, clinical course, longitudinal aquaporin-4 immunoglobulin G (AQP4-IgG) serology, MRI, therapeutic management, and outcome data were collected and analyzed. Results The onset age of the cohort was 38.1±12.0 years (mean±standard deviation). The median disease duration was 73.5 months (interquartile range=44.3–117.0 months), and the follow-up period was 54.2±23.8 months. At the end of the last visit, the final diagnosis was categorized into AQP4-IgG-seronegative NMOSD ( n =16, 66.7%), AQP4-IgG-seropositive NMOSD ( n =7, 29.2%), or multiple sclerosis ( n =1, 4.2%). Seven of the 24 patients (29.2%) experienced conversion to AQP4-IgG seropositivity, and the interval from onset to this serological conversion was 37.9±21.9 months. Isolated/mixed area postrema syndrome (APS) was the predominant onset phenotype (37.5%). The patients with isolated/mixed APS onset showed a predilection for conversion to AQP4-IgG seropositivity. All patients experienced a multiphasic disease course, with immunosuppressive therapy reducing the incidence rates of clinical relapse and residual functional disability. Conclusions Definite NMOSD may be preceded by LF-NMOSD, particularly isolated/mixed APS. Intensive long-term follow-up and attack-prevention immunotherapeutic management is recommended in patients with LF-NMOSD.

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“…There are markers in the imaging of APS patients, and the occurrence of "inverted V sign" in medulla oblongata segment on axial MRI has high speci city for the diagnosis of NMOSD. In addition, sagittal MRI of most APS patients also showed "linear sign", but "linear sign" were also seen in other diseases besides NMOSD [17,18]. Case 2 underwent cranial MRI procedures several times before the diagnosis, but no obvious lesions on the dorsolateral medulla oblongata were identi ed.…”

Section: Mri Ndingsmentioning

confidence: 96%

Clinical Analysis of NMOSD with Area Postrema Syndrome as Initial Symptom

Liu

Liang

et al. 2021

Preprint

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Objective: Patients with neuromyelitis optica spectrum disease (NMOSD) with the initial manifestation of area postrema syndrome (APS) often have unexplained nausea and vomiting and are easily misdiagnosed for the NMOSD. The purpose of this study was to report and discuss clinical analysis, including diagnosis and treatment of 4 cases of NMOSD with APS as the first symptom.Methods: Four patients with intractable nausea and vomiting were selected for the analysis and finally the cases were confirmed for the NMOSD. All of these patients started with misdiagnosis and mismanagement initially.Results: Among the 4 patients included in this study, 3 were admitted to the department of gastroenterology at the onset of the disease, and 2 of them were not correctly diagnosed and treated on time due to misdiagnosis. Therefore, their symptoms worsened, and they needed to be transferred to ICU for life support. No obvious early medulla lesions were found in one patient. One patient was treated with intravenous immunoglobulin, methylprednisolone, and plasma exchange but there was no significant clinical improvement, and then the disease was relapsed during the treatment with low-dose rituximab.Conclusion: The clinical manifestations of NMOSD are complex and diverse, and the initial symptoms, onset age of the patient, and MRI findings can all influence the clinicians' judgment of the disease. Early identification of the APS and timely therapy can prevent visual and physical disabilities, even respiratory failure and cardiac arrest. Therefore, it is necessary to identify specific and sensitive serum and imaging markers for predicting the prognosis and recurrence of the disease.

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Area postrema syndrome as initial manifestation in neuromyelitis optica spectrum disorder patients: A retrospective study

Cited by 14 publications

References 31 publications

Area Postrema Syndrome

Area Postrema Syndrome

Disease Course and Outcomes in Patients With the Limited Form of Neuromyelitis Optica Spectrum Disorders and Negative AQP4-IgG Serology at Disease Onset: A Prospective Cohort Study

Clinical Analysis of NMOSD with Area Postrema Syndrome as Initial Symptom

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