The Etiology and Outcome of Area Postrema Syndrome in Childhood: Two Cases and a Literature Review.

BACKGROUND: Area postrema syndrome (APS), a rare childhood condition, manifests as intractable nausea and hiccups. APS has high diagnostic significance in neuromyelitis optica syndrome spectrum disorders (NMOSD) and can be the initial presentation of other critical diseases, including brainstem glioma. METHODS: We described two representative cases of unrelated Japanese patients with APS. An etiologic evaluation, including a detailed intracranial neuroradiological examination and autoantibodies assessment, was performed. We also reviewed the literature focusing on the prognosis of pediatric APS symptoms. RESULTS: A 14-year-old girl with aquaporin-4 antibody-positive NMOSD showed a good prognosis with immunotherapy, whereas another nine-year-old girl with irresectable medullary low-grade glioma had persistent symptoms for more than 10 years. All reported children aged >12 years were diagnosed with NMOSD, and patients aged <13 years showed heterogeneous etiologies. CONCLUSIONS: Distinctive time courses and neuroimaging features were key clinical findings for the diagnostic and therapeutic processes in these patients. This literature review highlights the wide spectrum and prognosis of pediatric-onset APS.

Area postrema syndrome caused by medullary infarction.

In the present study, we report a case of persistent intractable nausea and vomiting after a medullary infarction. Area postrema syndrome due to ischemic stroke is very rare. In this case, brain magnetic resonance imaging revealed an ischemic lesion in the lateral medulla extending caudally and dorsomedially. The patient presented with sustained nausea, vomiting, and poor oral intake over one month after the index stroke, even after resolution of dizziness and disappearance of nystagmus. She did not respond to intravenous metoclopramide with ondansetron. However, their intractable nausea and vomiting eventually resolved with concomitant use of domperidone and itopride orally in addition to intravenous metoclopramide with ondansetron.

MRI to detect and localize the area postrema in multiple sclerosis: The role of 3D-DIR and 3D-FLAIR.

BACKGROUND AND PURPOSE: Area postrema (AP) is a highly vascularized paired 2 mm-long anatomical structure, localized on the dorsal inferior surface of the medulla oblongata, at the caudal end of the fourth-ventricle. AP is principally affected in AP syndrome, which is commonly associated with autoimmune inflammatory diseases, including essentially neuromyelitis optica spectrum disorder (NMOSD). The aim of this study is to assess the best cerebral MRI sequences and planes for AP detection in order to assist or aid in the diagnosis of difficult NMOSD cases. METHODS: 3DT1, 2DT2, 3D-fluid-attenuated inversion recovery (3DFLAIR), and 3D-double inversion recuperation (3DDIR), routinely used in inflammatory diseases, were analyzed and scored based on quality (0-2), and ability to detect AP in each plane (0 = no detection, 1 = probable detection, 2 = obvious detection). Based on image availability, subjects were divided into three groups: Group-1, including 100 randomly selected subjects with 3DT1 and 3DFLAIR, Group-2, including 30 multiple sclerosis (MS) patients from the "Observatoire Français de la Sclérose En Plaques" (OFSEP) with 3DT1, 3DFLAIR, and 3DDIR, and Group-3, including 164 OFSEP MS patients with 3DFLAIR and 2DT2. RESULTS: AP was undetectable on 3DT1 and 2DT2. AP was detected in 87% of 3DFLAIR in Group-1, 90% in Group-2, and 90% in Group-3. AP was also detected in 100% of 3DDIR images in the axial plane. CONCLUSIONS: As evidenced, AP was easily assessed on 3DDIR and 3DFLAIR emphasizing the importance of adding these sequences to NMOSD MRI-protocols. Moreover, the most effective imaging plane in identifying AP was the axial plane.

Síndrome del área postrema aislado con anticuerpos anti-MOG, una asociación poco frecuente / Isolated area postrema syndrome with anti-MOG antibodies, a rare association

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[Isolated area postrema syndrome with anti-MOG antibodies, a rare association]. / Síndrome del área postrema aislado con anticuerpos anti-MOG, una asociación poco frecuente.

TITLE: Síndrome del área postrema aislado con anticuerpos anti-MOG, una asociación poco frecuente.

Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG-positive NMOSD.

OBJECTIVE: To define the frequency, duration, and severity of intractable nausea, vomiting, or hiccups in aquaporin-4-immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and propose diagnostic criteria and a severity scale for area postrema syndrome (APS). METHODS: An International NMOSD database was interrogated for frequency of APS. Patients with AQP4-IgG-positive NMOSD completed an APS symptom questionnaire. Nausea and vomiting severity was derived from the Pregnancy-Unique Quantification of Emesis and Nausea (PUQE) score. The diagnostic criteria, severity scale, and immunotherapy response was applied to a prospective validation cohort of patients from multiple centers. RESULTS: Analysis of an international database for AQP4-IgG-seropositive NMOSD (n = 430) revealed a high prevalence of isolated APS attacks (onset 7.1%-10.3%; subsequent 9.4%-14.5%) across continents. For 100 patients with 157 episodes of APS, nausea (n = 127, 81%) lasted for a median of 14 days (range 2-365), vomiting (113, 72%) with a median of 5 episodes/d (2-40) lasted 1-20 minutes, and hiccups (102, 65%) lasted a median of 14 days (2-365). Symptoms consistently and completely resolved following immunotherapy. Data were used to propose APS diagnostic criteria and repurpose PUQE score (hiccups severity grade based on symptom duration). The clinical utility was demonstrated in a prospective validation cohort. CONCLUSION: Isolated APS attacks are frequently encountered both at onset and during the NMOSD course. The diagnostic criteria proposed here will assist clinicians in recognizing APS. Diagnosis of an APS attack earlier than 48 hours is possible if a dorsal medulla lesion is detected. Accurate diagnosis and evaluation of APS attack severity will assist in outcome measurement in NMOSD clinical trials.