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

2021 ◽  
Vol 57 (1) ◽  
Author(s):  
Eman M. Khedr ◽  
Hassan M. Farweez ◽  
Noha Abo Elfetoh ◽  
Eman R. Badawy ◽  
Sara Hassanein ◽  
...  
Keyword(s):  
Clinical Features ◽  
Area Postrema ◽  
Clinical Status ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
University Hospitals ◽  
Rare Presentation ◽  
History Of ◽  
Spinal Mri

Abstract 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.

scholarly journals Clinical profile of patients with paraneoplastic neuromyelitis optica spectrum disorder and aquaporin-4 antibodies

2017 ◽  
Vol 24 (13) ◽  
pp. 1753-1759 ◽  
Author(s):  
Maria Sepúlveda ◽  
Nuria Sola-Valls ◽  
Domingo Escudero ◽  
Bojan Rojc ◽  
Manuel Barón ◽  
...  
Keyword(s):  
Clinical Features ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Nausea And Vomiting ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Severe Nausea ◽  
Longitudinal Extensive Transverse Myelitis ◽  
Clinical Records

Background: In a minority of patients with neuromyelitis optica spectrum disorder (NMOSD) and aquaporin-4 antibodies (AQP4-IgG), the disease has a paraneoplastic origin. It is unknown whether these patients have distinctive clinical features. Objective: To report the clinical features of a series of patients with paraneoplastic NMOSD and AQP4-IgG and to review previously reported cases. Methods: Retrospective analysis of clinical records of 156 patients with NMOSD and AQP4-IgG and review of previously reported patients with paraneoplastic NMOSD and AQP4-IgG. Paraneoplastic patients were defined as those with cancer identified within 2 years of the diagnosis of NMOSD. Results: Five (3.2%) of 156 patients had paraneoplastic NMOSD, and 12 previously reported patients were identified. The most common tumors were adenocarcinoma of the lung (five patients) and breast (five). Compared with the 151 non-paraneoplastic NMOSD patients, the 17 (5 current cases and 12 previously reported) were older at symptom onset (median age = 55 (range: 17–87) vs 40 (range: 10–77) years; p = 0.006), more frequently male (29.4% vs 6.6%; p = 0.009), and presented with severe nausea and vomiting (41.2% vs 6.6%; p < 0.001). The frequency of longitudinal extensive transverse myelitis (LETM) as heralding symptom was similar in both groups, but patients with paraneoplastic NMOSD were older than those with non-paraneoplastic NMOSD (median age: 63 (range: 48–73) vs 43 (range: 14–74) years; p = 0.001). Conclusion: Patients, predominantly male, with NMOSD and AQP4-IgG should be investigated for an underlying cancer if they present with nausea and vomiting, or LETM after 45 years of age.

Aquaporin-4-IgG positive neuromyelitis optica spectrum disorder and systemic autoimmune diseases overlap syndrome: a single-center experience

Lupus ◽  
2019 ◽  
Vol 28 (11) ◽  
pp. 1302-1311 ◽  
Author(s):  
E Martín-Nares ◽  
G Hernandez-Molina ◽  
H Fragoso-Loyo
Keyword(s):  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Area Postrema ◽  
Transverse Myelitis ◽  
Overlap Syndrome ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Single Center ◽  
Imaging Characteristics

Objective To describe the clinical and radiological characteristics and outcomes of patients with aquaporin-4-immunoglobulin G (AQP4-IgG) seropositive neuromyelitis optica spectrum disorder (NMOSD) coexisting with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS) in a single center. Methods We included patients with diagnosis of NMOSD and a concomitant diagnosis of SLE or pSS. Demographic, clinical, serological and imaging characteristics were retrieved from clinical charts. Results Twelve patients were included, of whom 11 (91.7%) were women. Seven (58.3%) had SLE and five (41.7%) pSS. In five (41.7%) patients NMOSD followed SLE/pSS onset, four (33.3%) patients had a simultaneous presentation, and in three (25%) NMOSD preceded pSS onset. The mean age at first neurological event was 39 years. Eleven patients (91.7%) experienced acute transverse myelitis/longitudinally extensive transverse myelitis, five (41.7%) optic neuritis, three (25%) a cerebral syndrome and two (16.7%) each area postrema syndrome, acute brainstem syndrome and cerebellar syndrome. Eleven (91.7%) patients went into either total or partial NMOSD remission at median follow-up of 89.5 months. Conclusion AQP4-IgG seropositive NMOSD arose in the context of quiescent SLE and pSS with extraglandular features. As NMOSD coexisting with SLE/pSS is rare, collaborative multicenter studies are needed to clarify the natural history and outcomes of this overlap syndrome.

scholarly journals Aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder with recurrent short partial transverse myelitis and favorable prognosis: Two new cases

2017 ◽  
Vol 23 (14) ◽  
pp. 1950-1954 ◽  
Author(s):  
Jinhua Zhang ◽  
Fang Liu ◽  
Yiqi Wang ◽  
Ying Yang ◽  
Yuehong Huang ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Acute Attack ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Attack Phase ◽  
Long Term Prognosis

Understanding the characteristics of neuromyelitis optica spectrum disorder (NMOSD) with recurrent short partial transverse myelitis (SPTM), which is very rare, contributes to the differential diagnosis of multiple sclerosis (MS). We present two Chinese aquaporin-4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD cases who had at least twice SPTM during 4 and 6 years of follow-up, respectively. Their SPTMs have been mild and responded well to corticosteroids just like in the case of MS. The findings highlight the need of searching for serum AQP4-IgG (cell-based assay strongly recommended) in patients with recurrent SPTM and suggest that those patients may have a mild acute attack phase and favorable long-term prognosis.

scholarly journals Neuromyelitis Optica Spectrum Disorder and Anti-MOG Syndromes

Biomedicines ◽  
2019 ◽  
Vol 7 (2) ◽  
pp. 42 ◽  
Author(s):  
Marco A. Lana-Peixoto ◽  
Natália Talim
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Area Postrema ◽  
Water Channel ◽  
Clinical Manifestations ◽  
Transverse Myelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Immunosuppressive Drugs ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder

Neuromyelitis optica spectrum disorder (NMOSD) and anti-myelin oligodendrocyte glycoprotein (anti-MOG) syndromes are immune-mediated inflammatory conditions of the central nervous system that frequently involve the optic nerves and the spinal cord. Because of their similar clinical manifestations and habitual relapsing course they are frequently confounded with multiple sclerosis (MS). Early and accurate diagnosis of these distinct conditions is relevant as they have different treatments. Some agents used for MS treatment may be deleterious to NMOSD. NMOSD is frequently associated with antibodies which target aquaporin-4 (AQP4), the most abundant water channel in the CNS, located in the astrocytic processes at the blood-brain barrier (BBB). On the other hand, anti-MOG syndromes result from damage to myelin oligodendrocyte glycoprotein (MOG), expressed on surfaces of oligodendrocytes and myelin sheaths. Acute transverse myelitis with longitudinally extensive lesion on spinal MRI is the most frequent inaugural manifestation of NMOSD, usually followed by optic neuritis. Other core clinical characteristics include area postrema syndrome, brainstem, diencephalic and cerebral symptoms that may be associated with typical MRI abnormalities. Acute disseminated encephalomyelitis and bilateral or recurrent optic neuritis are the most frequent anti-MOG syndromes in children and adults, respectively. Attacks are usually treated with steroids, and relapses prevention with immunosuppressive drugs. Promising emerging therapies for NMOSD include monoclonal antibodies and tolerization.

scholarly journals Case Report: Postvaccination Anti–Myelin Oligodendrocyte Glycoprotein Neuromyelitis Optica Spectrum Disorder

2020 ◽  
Vol 22 (2) ◽  
pp. 85-90 ◽  
Author(s):  
Neha Kumar ◽  
Kelsey Graven ◽  
Nancy I. Joseph ◽  
John Johnson ◽  
Scott Fulton ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
First Trimester ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Future Research ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
The Central Nervous System

Abstract Stimulation of the immune response after vaccination can occasionally result in adverse effects, including demyelination of the central nervous system. The most common presentation of postvaccination demyelination is acute disseminated encephalomyelitis, but cases of optic neuritis, transverse myelitis, and multiple sclerosis relapses have been reported. More recently, an increasing number of postvaccination neuromyelitis optica spectrum disorder (NMOSD) cases have surfaced in the literature, especially in patients with aquaporin-4 antibodies. In this article, we report an unusual case of myelin oligodendrocyte glycoprotein antibody–related NMOSD after the receipt of multiple vaccines in a first-trimester pregnant woman from Africa. We review the reported cases of postvaccination demyelination in the past decade, with a focus on the relationship between NMOSD and vaccination in patients with aquaporin-4 or myelin oligodendrocyte glycoprotein antibodies. Finally, we discuss the clinical relevance of the present case and similar reported cases as it relates to patient care in the neuroimmunology clinic and identify potential areas for future research.

Area postrema syndrome: A short history of a pearl in demyelinating diseases

2018 ◽  
Vol 25 (3) ◽  
pp. 325-329 ◽  
Author(s):  
Carlos R Camara-Lemarroy ◽  
Jodie M Burton
Keyword(s):  
Optic Neuritis ◽  
20Th Century ◽  
Medulla Oblongata ◽  
Medical Literature ◽  
Area Postrema ◽  
Transverse Myelitis ◽  
Demyelinating Diseases ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Short History ◽  
History Of

In this topical review, we discuss the history of the area postrema syndrome, with special attention given to early studies aimed at identifying the area postrema and its function, possible early cases of the syndrome and its current relevance in neuroimmunology and demyelinating diseases. In 1896, Retzius named a structure in the posterior medulla oblongata as the area postrema. The work of Borison in the middle of the 20th century led to the elucidation of its function as a “vomiting center.” The historical medical literature is filled with excellent examples that could be described as “area postrema syndrome.” While severe and bilateral optic neuritis and transverse myelitis still constitute the classic components of neuromyelitis optica spectrum disorder (NMOSD), intractable vomiting and hiccups due to area postrema involvement is now recognized as essentially pathognomonic, indeed a shiny pearl in neuroimmunology and demyelinating diseases.

A case of cerebral aquaporinopathy

2010 ◽  
Vol 16 (10) ◽  
pp. 1252-1254 ◽  
Author(s):  
A. Tanaka ◽  
T. Yoshida ◽  
T. Yamada ◽  
R. Isayama ◽  
Y. Fujiwara ◽  
...  
Keyword(s):  
Area Postrema ◽  
Brain Mri ◽  
Transverse Myelitis ◽  
Brain Regions ◽  
Abnormal Signal ◽  
Spinal Mri ◽  
Magnetic Resonance Imaging Mri ◽  
Antibody Levels ◽  
Dorsal Medulla

A 35-year-old woman was hospitalized due to impaired consciousness. Magnetic resonance imaging (MRI) revealed multiple parenchymal lesions in supra and infratentorial brain regions, which were considered responsible for her declining consciousness level. She was treated with intravenous methylprednisolone. Neurological symptoms improved and she was discharged. She was readmitted 14 months later due to intractable hiccups. A follow-up brain MRI revealed an abnormal signal near the area postrema in the dorsal medulla. Serum aquaporin-4 antibody levels were positive, but there were no visual manifestations or myelitis. Spinal MRI was negative for longitudinally extended transverse myelitis throughout the clinical course.

Acute anosmia in neuromyelitis optica spectrum disorder

2020 ◽  
Vol 26 (14) ◽  
pp. 1958-1960 ◽  
Author(s):  
Joseph Marshall ◽  
Iris Kleerekooper ◽  
Indran Davagnanam ◽  
S Anand Trip
Keyword(s):  
Neuromyelitis Optica ◽  
Area Postrema ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Resonance Imaging ◽  
Longitudinal Extensive Transverse Myelitis ◽  
Magnetic Resonance Imaging Mri ◽  
Aquaporin 4 Antibody ◽  
Extensive Transverse Myelitis

The cardinal features of neuromyelitis optica spectrum disorder (NMOSD) are optic neuritis, longitudinal extensive transverse myelitis and area postrema syndrome. Olfactory dysfunction is not listed as a feature in the NMOSD diagnostic criteria. Here, we present an aquaporin-4 antibody positive patient who, in addition to classical features of NMOSD, developed acute anosmia with magnetic resonance imaging (MRI) evidence of olfactory bulb abnormalities. While the association of anosmia and NMOSD has been rarely noted previously, to our knowledge, no prior cases have found this to be one of the presenting features of a relapse nor have they identified acute radiological correlates.

scholarly journals Unrecognized neuromyelitis optica spectrum disorder with pontine and corpus callosum microhemorrhage

2021 ◽  
pp. 86-86
Author(s):  
Igor Nosek ◽  
Jasmina Boban ◽  
Dmitar Vlahovic ◽  
Biljana Radovanovic ◽  
Dejan Kostic ◽  
...  
Keyword(s):  
Corpus Callosum ◽  
Neuromyelitis Optica ◽  
Area Postrema ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Cross Sectional ◽  
Immune Mediated ◽  
Demyelinating Lesions ◽  
Improve Patient

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) represents an immune-mediated neuroinflammatory syndrome, classified as separate entity after discovery of aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). The neuroimaging spectrum of NMOSD classically consisted of bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM), recently broadened with lesions in area postrema, diencephalon, brainstem and cerebellum, and extensive cord atrophy. Case report: Here we present a case of an AntiAQP4-positive 65-year old female patient who initially presented with underappreciated LETM and developed multiple cerebral and cerebellar lytic demyelinating lesions associated with acute long segment optic nerve involvement two years later. Two new imaging findings are described in this case: the involvement of complete cross-sectional area of pons and microhemorrhage in the pons and corpus callosum. Conclusion: Raising suspicion of NMOSD is of a crucial importance in cases with isolated LETM in order to prevent relapses in Anti-AQP4 positive cases, improve patient outcome and recovery.

scholarly journals Aquaporin-4-IgG Positive Neuromyelitis Optica Spectrum Disorder from Ethiopia: A Case Report

2020 ◽  
Vol 30 (5) ◽  
Author(s):  
Dereje Melka
Keyword(s):  
Neuromyelitis Optica ◽  
Addis Ababa ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Inflammatory Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Mri Imaging ◽  
Limb Weakness ◽  
The Right

BACKGROUND: Neuromyelitis Optica spectrum disorder is an inflammatory disorder affecting the central nervous system), most commonly attacking the spinal cord or optic nerves. Limited cases of neuromyelitis optica have been reported in east Africa. Based on my review, if published, this would be the second published case of Neuromyelitis Optica spectrum disorder and the first published case of seropositive Neuromyelitis Optica spectrum disorder reported from Ethiopia. It signifies the need to have a high index of suspicion to promptly identify and properly treat these patients.CASE PRESENTATION: I am reporting a 32 years old female patient from Addis Ababa, Ethiopia, who presented with recurrent lower limb weakness and impairment of right eye vision of two-year duration. She was diagnosed based on Neuromyelitis Optica spectrum disorder diagnostic criteria, by having transverse myelitis, optic neuritis, confirmed by MRI imaging and high level of aquaporin-4-antibodies. Symptoms improved after providing five days of Methylprednisolone followed by low doses of corticosteroids and Azathioprine. The patient is now fully functional except for the right eye vision impairment.CONCLUSION: The patient described here signifies a classic manifestation of Neuromylitis Optica disorder with aquaporin- 4-IgG occurring in Ethiopian woman. This case highlights the existence of Devic’s disease within our setting and the need toproperly diagnose this condition even in a resource-limited setting to avert disability.

Sign in / Sign up

Export Citation Format

Share Document