Meningitis as a recurrent manifestation of anti-AQP4/anti-MOG negative neuromyelitis optica spectrum disorder: a case report

Abstract Background Neuromyelitis optica spectrum disorders (NMOSD), a group of autoimmune neurological diseases, involve the optic nerve, spinal cord, and brain. Meningitis is rarely reported as the primary clinical manifestation of both anti-aquaporin-4 (AQP4)/ anti-myelin oligodendrocyte glycoprotein (MOG) antibody-negative NMOSD (NMOSDneg). Case presentation A 30-year-old man initially presented with fever, headache, and neck stiffness. Lumbar puncture revealed mixed cell reaction and decreased glucose levels. As a result, tuberculous meningitis was suspected. After 1 month, the patient developed longitudinally extensive transverse myelitis and area postrema syndrome. This was followed by the presentation of meningitis-like symptoms once again in the third attack, but his condition eventually improved after corticosteroid treatment without relapse for 2 years. However, he was readmitted to our hospital owing to symptoms of diplopia, hiccup, and numbness in the right hand. Brain magnetic resonance imaging (MRI) revealed that the area postrema still contained lesions. Spinal MRI revealed several segmental enhancements at the C4–C5, T1, and T5 levels. Anti-AQP4 and anti-MOG antibodies were persistently absent in the serum and cerebrospinal fluid (CSF). The patient was finally diagnosed with NMOSDneg. Conclusions Meningitis could be a recurrent manifestation of NMOSDneg and requires more careful evaluation.

Download Full-text

Overlapping anti-N-methyl-D-aspartate receptor (NMDAR) Encephalitis With Neuromyelitis Optica Spectrum Disorders: A Case Report

10.21203/rs.3.rs-907816/v1 ◽

2021 ◽

Author(s):

Jialin Pan ◽

Begench Ovlyakulov ◽

Lili zhou

Keyword(s):

Neuromyelitis Optica ◽

Brain Magnetic Resonance Imaging ◽

Chinese Patient ◽

Cerebellar Hemisphere ◽

Double Positive ◽

Blurred Vision ◽

Aspartate Receptor ◽

Nmdar Encephalitis ◽

Magnetic Resonance Imaging Mri ◽

The Right

Abstract BackgroundAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis can coexist with neuromyelitis optica spectrum disorder (NMOSD). Patients with overlapping anti-NMDAR encephalitis with positive NMDAR antibodies and aquaporin 4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD are rare but should not be ignored.Case presentationThis report describes a unique case of anti-NMDAR encephalitis coexisting with NMOSD is presented. A 27-year-old male presented with blurred vision, cognitive impairment, psychosis, dysphagia, gait instability and urinary incontinence. Brain magnetic resonance imaging (MRI) showed abnormal signals in the right cerebellar hemisphere, temporal lobe, and corpus callosum. NMDAR antibodies were positive in the CSF. AQP4-IgG antibodies were positive in the serum. The patient's condition was stable following intravenous gamma globulin, corticosteroids, immunosuppressants and symptomatic treatments. ConclusionsThis case provides further evidence for the occurrence of anti-NMDAR encephalitis overlapping NMOSD with AQP4-IgG-seropositive in a Chinese patient. However, the mechanisms underlying the occurrence of double positive antibodies remain elusive.

Download Full-text

Neuromyelitis Optica Spectrum Disorder and Anti-MOG Syndromes

Biomedicines ◽

10.3390/biomedicines7020042 ◽

2019 ◽

Vol 7 (2) ◽

pp. 42 ◽

Cited By ~ 18

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.

Download Full-text

Occurrence of acute large and edematous callosal lesions in neuromyelitis optica

Multiple Sclerosis Journal ◽

10.1177/1352458509103301 ◽

2009 ◽

Vol 15 (6) ◽

pp. 695-700 ◽

Cited By ~ 60

Author(s):

M Nakamura ◽

T Misu ◽

K Fujihara ◽

I Miyazawa ◽

I Nakashima ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Medical Records ◽

Brain Magnetic Resonance Imaging ◽

Transverse Myelitis ◽

Resonance Imaging ◽

Longitudinally Extensive Transverse Myelitis ◽

Lower Margin ◽

Chronic Stage ◽

The Brain ◽

Extensive Transverse Myelitis

Background The corpus callosum is commonly involved in multiple sclerosis (MS), but the characteristics of callosal lesions in neuromyelitis optica (NMO) are unknown. Objective To reveal the features of callosal lesions in NMO in comparison to MS. Methods We retrospectively reviewed the medical records and the brain magnetic resonance imaging films of 56 patients with MS and 22 patients with NMO. Results In MS, 36 (64.3%) of 56 patients had callosal lesions, but only four patients had acute lesions. All such acute lesions were small, isolated and non-edematous, and the intensity was homotonic. Chronic lesions were observed in 34 patients with MS, and 32 (94%) of them presented small lesions located at the callosal lower margin (“hemi-oval pattern”). Meanwhile, four (18.2%) patients with NMO had callosal lesions, and three of them had acute lesions. Those acute lesions were multiple, large edematous ones with heterogeneous intensity (“marbled pattern”). In the chronic stage, the lesions shrank or disappeared. Conclusions Acute large, edematous callosal lesions occasionally occur in NMO. Similar to longitudinally extensive transverse myelitis, such callosal lesions may reflect severe edematous inflammation in NMO, and may provide additional evidence that the pathogenesis in NMO is different from that in MS.

Download Full-text

Acute anosmia in neuromyelitis optica spectrum disorder

Multiple Sclerosis Journal ◽

10.1177/1352458520907909 ◽

2020 ◽

Vol 26 (14) ◽

pp. 1958-1960 ◽

Cited By ~ 1

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.

Download Full-text

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

Multiple Sclerosis Journal ◽

10.1177/1352458507082617 ◽

2007 ◽

Vol 14 (2) ◽

pp. 248-251 ◽

Cited By ~ 21

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

Download Full-text

Unrecognized neuromyelitis optica spectrum disorder with pontine and corpus callosum microhemorrhage

Vojnosanitetski pregled ◽

10.2298/vsp201126086n ◽

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.

Download Full-text

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

Ethiopian Journal of Health Sciences ◽

10.4314/ejhs.v30i5.25 ◽

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.

Download Full-text

Magnetic resonance imaging in neuromyelitis optica

Multiple Sclerosis Journal ◽

10.1177/1352458514531087 ◽

2014 ◽

Vol 20 (9) ◽

pp. 1153-1164 ◽

Cited By ~ 41

Author(s):

George Tackley ◽

Wilhelm Kuker ◽

Jacqueline Palace

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Neuromyelitis Optica ◽

Area Postrema ◽

Brain Mri ◽

Transverse Myelitis ◽

Resonance Imaging ◽

Demyelinating Disorder ◽

The Central Nervous System ◽

Magnetic Resonance Imaging Mri

Neuromyelitis optica (NMO), or Devic’s disease, is a rare demyelinating disorder of the central nervous system that has a predilection for the optic nerve and spinal cord. Magnetic resonance imaging (MRI) is required to diagnose NMO. Longitudinally extensive transverse myelitis is NMO’s imaging hallmark and the presence of a brain MRI that is not diagnostic of multiple sclerosis (MS) also remains part of the diagnostic criteria. It is increasingly recognised that MS and NMO brain imaging can, however, have similar appearances but differences do exist: hypothalamic, periaqueductal grey and area postrema lesions implicate NMO whilst cortical, U-fibre or Dawson’s finger lesions are suggestive of MS. The timing of image acquisition, age, ethnicity and aquaporin-4 antibody status are all likely to alter the findings at MRI. This review therefore aims to overview and update the reader on NMO imaging, to provide clinically relevant guidance for diagnosing NMO and differentiating it from MS in order to guide management, and to highlight recent research insights.

Download Full-text

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

Lupus ◽

10.1177/0961203319877255 ◽

2019 ◽

Vol 28 (11) ◽

pp. 1302-1311 ◽

Cited By ~ 4

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.

Download Full-text

Elucidating distinct clinico-radiologic signatures in the borderland between neuromyelitis optica and multiple sclerosis

Journal of Neurology ◽

10.1007/s00415-021-10619-1 ◽

2021 ◽

Author(s):

Maciej Juryńczyk ◽

Elżbieta Klimiec-Moskal ◽

Yazhuo Kong ◽

Samuel Hurley ◽

Silvia Messina ◽

...

Keyword(s):

Multiple Sclerosis ◽

Neuromyelitis Optica ◽

Unmet Need ◽

Transverse Myelitis ◽

Overlap Syndrome ◽

Brain Lesions ◽

Central Vein ◽

Normal Brain ◽

Group 4 ◽

Group 2

Abstract Background Separating antibody-negative neuromyelitis optica spectrum disorders (NMOSD) from multiple sclerosis (MS) in borderline cases is extremely challenging due to lack of biomarkers. Elucidating different pathologies within the likely heterogenous antibody-negative NMOSD/MS overlap syndrome is, therefore, a major unmet need which would help avoid disability from inappropriate treatment. Objective In this study we aimed to identify distinct subgroups within the antibody-negative NMOSD/MS overlap syndrome. Methods Twenty-five relapsing antibody-negative patients with NMOSD features underwent a prospective brain and spinal cord MRI. Subgroups were identified by an unsupervised algorithm based on pre-selected NMOSD/MS discriminators. Results Four subgroups were identified. Patients from Group 1 termed “MS-like” (n = 6) often had central vein sign and cortical lesions (83% and 67%, respectively). All patients from Group 2 (“spinal MS-like”, 8) had short-segment myelitis and no MS-like brain lesions. Group 3 (“classic NMO-like”, 6) had high percentage of bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM, 80% and 60%, respectively) and normal brain appearance (100%). Group 4 (“NMO-like with brain involvement”, 5) typically had a history of NMOSD-like brain lesions and LETM. When compared with other groups, Group 4 had significantly decreased fractional anisotropy in non-lesioned tracts (0.46 vs. 0.49, p = 0.003) and decreased thalamus volume (0.84 vs. 0.98, p = 0.04). Conclusions NMOSD/MS cohort contains distinct subgroups likely corresponding to different pathologies and requiring tailored treatment. We propose that non-conventional MRI might help optimise diagnosis in these challenging patients.

Download Full-text