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 Neuromyelitis optica spectrum disorder with delayed lesion on spinal cord magnetic resonance imaging

2018 ◽  
Author(s):  
Jeong Yeon Kim
Keyword(s):  
Magnetic Resonance Imaging ◽  
Spinal Cord ◽  
Magnetic Resonance ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Resonance Imaging ◽  
Aquaporin 4 Antibody ◽  
Extensive Transverse Myelitis

The term Neuromyelitis optica spectrum disorder (NMOSD) is a recently proposed for neuromyelitis optica and related syndromes. Along with serum anti-aquaporin 4 antibody status, detection of a lesions extending over ≥3 contiguous spinal cord segments (longitudinally extensive transverse myelitis, LETM) associated with acute myelitis is the most specific neuroimaging characteristic of NMOSD. 1 We present a case of NMOSD with delayed lesion on spinal cord magnetic resonance imaging.

Neuromyelitis optica spectrum disorder and systemic lupus erythematosus

Lupus ◽  
2019 ◽  
Vol 28 (14) ◽  
pp. 1722-1726 ◽  
Author(s):  
M M Thabah ◽  
Sekar D ◽  
R Pranov ◽  
M M V Moulitej ◽  
A Ramesh ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Systemic Lupus ◽  
Autoimmune Conditions ◽  
Inflammatory Syndrome ◽  
Extensive Transverse Myelitis

Neuromyelitis optica spectrum disorder is an inflammatory syndrome that is associated with many autoimmune conditions. We present the case of a patient who had longitudinally extensive transverse myelitis and antibodies to aquaporin 4 IgG (AQP4-IgG). Based on presence of lymphopenia, further workup revealed strong ANA positivity, anti-Sm antibodies, and low serum complements suggesting presence of systemic lupus erythematosus. The patient promptly responded to intravenous pulse methylprednisolone and five sessions of plasma exchange. At 1 year, she is on maintenance treatment with low dose prednisolone, azathioprine, and hydroxychloroquine, she has had no relapse and no other clinical features of lupus. This case is an illustration that neuromyelitis optica spectrum disorder can be the first manifestation of systemic lupus erythematosus.

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 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.

scholarly journals Pediatric Neuromyelitis Optica Spectrum Disorder: Case Series and Literature Review

Life ◽  
2021 ◽  
Vol 12 (1) ◽  
pp. 19
Author(s):  
Michela Ada Noris Ferilli ◽  
Roberto Paparella ◽  
Ilaria Morandini ◽  
Laura Papetti ◽  
Lorenzo Figà Talamanca ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Pediatric Patients ◽  
Demyelinating Disease ◽  
Area Postrema ◽  
Case Series ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Periaqueductal Gray Matter ◽  
Aquaporin 4 Antibody

Neuromyelitis Optica Spectrum Disorder (NMOSD) is a central nervous system (CNS) inflammatory demyelinating disease characterized by recurrent inflammatory events that primarily involve optic nerves and the spinal cord, but also affect other regions of the CNS, including hypothalamus, area postrema and periaqueductal gray matter. The aquaporin-4 antibody (AQP4-IgG) is specific for NMOSD. Recently, myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been found in a group of AQP4-IgG negative patients. NMOSD is rare among children and adolescents, but early diagnosis is important to start adequate therapy. In this report, we present cases of seven pediatric patients with NMOSD and we review the clinical and neuroimaging characteristics, diagnosis, and treatment of NMOSD in children.

Brain magnetic resonance imaging findings in acute relapses of neuromyelitis optica spectrum disorders

2007 ◽  
Vol 14 (2) ◽  
pp. 248-251 ◽  
Author(s):  
JA Cabrera-Gómez ◽  
A. Saiz-Hinarejos ◽  
F. Graus ◽  
A. González-Quevedo ◽  
R. Rodríguez-Rojas ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Transverse Myelitis ◽  
Resonance Imaging ◽  
Igg Antibody ◽  
Magnetic Resonance Imaging Mri ◽  
Extensive Transverse Myelitis

We studied cranial magnetic resonance imaging (MRI) lesions in three women with acute attacks of recurrent longitudinally extensive transverse myelitis (r-LETM), recurrent-optic neuritis (r-ON) and r-LETM-CNS. Neuromyelitis Optica -immunoglobulin (IgG) antibody was positive in all cases. Brain MRI (1.5 Tesla) was performed according to protocol from consortium MS centre. We described the cranial lesions in brain MRI of acute relapses. These lesions were different from MS, most had an asymptomatic course which disappeared with time, protocol from consortium of MS centre criteria for brain MRI and seropositivity of NMO-IgG are useful tools for differentiate acute lesions of NMO/MS. Multiple Sclerosis 2008; 14: 248—251. http://msj.sagepub.com

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.

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