NMO-DBr: the Brazilian Neuromyelitis Optica Database System

OBJECTIVE: To present the Brazilian Neuromyelitis Optica Database System (NMO-DBr), a database system which collects, stores, retrieves, and analyzes information from patients with NMO and NMO-related disorders. METHOD: NMO-DBr uses Flux, a LIMS (Laboratory Information Management Systems) for data management. We used information from medical records of patients with NMO spectrum disorders, and NMO variants, the latter defined by the presence of neurological symptoms associated with typical lesions on brain magnetic resonance imaging (MRI) or aquaporin-4 antibody seropositivity. RESULTS: NMO-DBr contains data related to patient's identification, symptoms, associated conditions, index events, recurrences, family history, visual and spinal cord evaluation, disability, cerebrospinal fluid and blood tests, MRI, optic coherence tomography, diagnosis and treatment. It guarantees confidentiality, performs cross-checking and statistical analysis. CONCLUSION: NMO-DBr is a tool which guides professionals to take the history, record and analyze information making medical practice more consistent and improving research in the area.

Download Full-text

The usefulness of brain MRI at onset in the differentiation of multiple sclerosis and seropositive neuromyelitis optica spectrum disorders

Multiple Sclerosis Journal ◽

10.1177/1352458513506953 ◽

2013 ◽

Vol 20 (6) ◽

pp. 695-704 ◽

Cited By ~ 43

Author(s):

So-Young Huh ◽

Ju-Hong Min ◽

Woojun Kim ◽

Su-Hyun Kim ◽

Ho Jin Kim ◽

...

Keyword(s):

Multiple Sclerosis ◽

Neuromyelitis Optica ◽

Lateral Ventricle ◽

Brain Mri ◽

Brain Magnetic Resonance Imaging ◽

Cortical Lesions ◽

Magnetic Imaging ◽

Brain Involvement ◽

Spectrum Disorders ◽

Magnetic Resonance Imaging Mri

Background: Although neuromyelitis optica (NMO) is a central nervous system (CNS) autoimmune disease distinct from multiple sclerosis (MS). NMO and NMO spectrum disorder (NMOSD) sometimes show asymptomatic lesions on brain magnetic resonance imaging (MRI) at onset, and even present with symptomatic brain involvement. Objectives: We investigated whether brain MRI at onset can be helpful for the differentiation of MS and NMOSD. Methods: We retrospectively analyzed initial brain MRIs, performed within three months of onset, in patients with MS ( n = 51) and anti-aquaporin4-antibody-positive patients with NMOSD ( n = 67). Results: NMOSD patients met the Paty (37%) and Barkhof (13%) criteria, and the criteria of the European Magnetic Imaging in MS (MAGNIMS) study group (9%), for MS. Ovoid lesions perpendicular to the lateral ventricle, isolated juxtacortical lesions in U-fibers and isolated ovoid/round cortical lesions were found only in MS patients, whereas longitudinal corticospinal tract lesions, extensive hemispheric lesions, periependymal lesions surrounding the lateral ventricle and cervicomedullary lesions were found only in NMOSD patients. Conclusions: Our study suggests that it is difficult to differentiate MS from NMOSD by the fulfillment of the MRI criteria for MS on brain MRI at onset; however, the characteristic morphology of brain lesions is highly useful for the early differentiation of the two disorders.

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 in Brazil: a study on clinical and prognostic factors

Multiple Sclerosis Journal ◽

10.1177/1352458508101935 ◽

2009 ◽

Vol 15 (5) ◽

pp. 613-619 ◽

Cited By ~ 46

Author(s):

DB Bichuetti ◽

EML Oliveira ◽

NA Souza ◽

RLM Rivero ◽

AA Gabbai

Keyword(s):

Neuromyelitis Optica ◽

Age Of Onset ◽

Care Center ◽

Brain Mri ◽

Tertiary Care ◽

Brain Magnetic Resonance Imaging ◽

Brain Lesions ◽

Relapsing Remitting ◽

First Relapse ◽

Magnetic Resonance Imaging Mri

Objectives To describe the clinical characteristics of patients with relapsing neuromyelitis optica (NMO) from a tertiary care center in Brazil and compare the groups with normal and abnormal brain magnetic resonance imaging (MRI). Methods Retrospective review of 41 patients followed at the Neuroimmunology Clinic of the Federal University of São Paulo, Brazil, from 1994 to 2007. Results All patients had relapsing-remitting optic-spinal disease, long extending spinal cord lesions, and brain MRI not meeting Barkhof criteria for multiple sclerosis (MS), thus fulfilling the 1999 and 2006 Wingerchuck criteria for NMO. Mean follow-up time was 52 months; mean age of onset was 32.6 years. The mean relapse rate (RR) and progression index (PI) were 1.0 and 0.9, respectively. Twenty-four patients had brain lesions not compatible with MS on MRI, and there were no statistical differences on PI and RR between patients who had brain lesions and patients who did not. Incomplete recovery, but not the type of first relapse, correlated with a worse prognosis. Seventeen patients were tested for NMO-IgG (anti-aquaporin-4 antibody) with 41% positivity. Conclusions In this series, we did not find a statistical difference of disease progression between patients with and without brain lesions, suggesting that the presence of brain abnormalities is not a marker of disease severity.

Download Full-text

Neuromyelitis optica: brain abnormalities in a Brazilian cohort

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2008000100001 ◽

2008 ◽

Vol 66 (1) ◽

pp. 1-4 ◽

Cited By ~ 21

Author(s):

Denis Bernardi Bichuetti ◽

René Leandro Magalhães Rivero ◽

Daniel May Oliveira ◽

Nilton Amorin de Souza ◽

Nitamar Abdala ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Demyelinating Disease ◽

Brain Mri ◽

Brain Magnetic Resonance Imaging ◽

Resonance Imaging ◽

Radiological Features ◽

Relapsing Remitting ◽

Magnetic Resonance Imaging Mri ◽

Brazilian Cohort ◽

Abnormal Brain

Neuromyelitis optica (NMO) is a demyelinating disease consisting of relapsing-remitting optic neuritis and myelitis with a more severe course than Multiple Sclerosis. Recently, it has been shown that almost 50% of patients with NMO can have brain magnetic resonance imaging (MRI) abnormalities. We report on six Brazilian patients with NMO, fulfilling the 1999 Wingerchuck criteria for this disease, with abnormal brain MRI and discuss their clinical and radiological features.

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

Factors associated with the effectiveness of plasma exchange for the treatment of NMO-IgG-positive neuromyelitis optica spectrum disorders

Multiple Sclerosis Journal ◽

10.1177/1352458512471875 ◽

2012 ◽

Vol 19 (9) ◽

pp. 1216-1218 ◽

Cited By ~ 33

Author(s):

Young-Min Lim ◽

So Young Pyun ◽

Bong-Hui Kang ◽

Jimin Kim ◽

Kwang-Kuk Kim

Keyword(s):

Plasma Exchange ◽

Neuromyelitis Optica ◽

Functional Improvement ◽

Factors Associated ◽

Mri Features ◽

Lower Baseline ◽

Before And After ◽

Primary Determinant ◽

Spectrum Disorders ◽

Magnetic Resonance Imaging Mri

To identify factors associated with plasma exchange response in neuromyelitis optica (NMO) spectrum disorders, the clinical and magnetic resonance imaging (MRI) features of 31 NMO-IgG-positive patients receiving plasma exchange for steroid-resistant exacerbations were analyzed. Functional improvement was observed in 65% of the patients. A lower baseline Expanded Disability Status Scale score was associated with favorable response ( p = 0.040). Patients without cord atrophy had a higher success rate than patients with atrophy ( p = 0.016). Levels of NMO-IgG did not differ between responders and non-responders before and after plasma exchange. In conclusion, a minimal pre-existing disability is the primary determinant of the effectiveness of plasma exchange.

Download Full-text

Diffuse and heterogeneous T2-hyperintense lesions in the splenium are characteristic of neuromyelitis optica

Multiple Sclerosis Journal ◽

10.1177/1352458512454772 ◽

2012 ◽

Vol 19 (3) ◽

pp. 308-315 ◽

Cited By ~ 22

Author(s):

Takahiro Makino ◽

Shoichi Ito ◽

Masahiro Mori ◽

Tadahiro Yonezu ◽

Yoshitsugu Ogawa ◽

...

Keyword(s):

Corpus Callosum ◽

Neuromyelitis Optica ◽

Odds Ratio ◽

Brain Magnetic Resonance Imaging ◽

Japanese Patients ◽

Resonance Imaging ◽

Inferior Aspect ◽

Fluid Attenuated Inversion Recovery ◽

Inversion Recovery Image ◽

Magnetic Resonance Imaging Mri

Background: Callosal lesions in multiple sclerosis (MS) are usually focal, involving the inferior aspect of the corpus callosum on brain magnetic resonance imaging (MRI), but little is known about callosal lesions in neuromyelitis optica (NMO). Objective: To clarify MRI abnormalities in callosal lesions of NMO. Methods: Japanese patients with NMO ( n=28) or MS ( n=22) were assessed. The distributions and appearances of callosal lesions were evaluated on a brain mid-sagittal T2-weighted image (T2WI) or a fluid-attenuated inversion recovery image with a 1.5T MRI scanner. Logistic regression analysis identified which characteristics of the callosal lesions were useful for discriminating NMO from MS. Results: Callosal lesions were present in 79% of NMO and 82% of MS patients. Callosal abnormalities of NMO, including splenial lesions (57% in NMO versus 27% in MS, odds ratio (OR)=4.23, p=0.04), diffusely spreading lesions from the lower to upper edges of the corpus callosum (71% versus 23%, OR=7.18, p=0.0024), and heterogeneous T2 hyperintense lesions (71% versus 9%, OR=44.3, p=0.0006), were feasible for discriminating NMO from MS. Conclusion: Diffuse and heterogeneous T2 hyperintense splenial lesions were characteristic of NMO. These findings could help distinguish NMO from MS on MRI.

Download Full-text

A Case of Persistent Generalized Retrograde Autobiographical Amnesia Subsequent to the Great East Japan Earthquake in 2011

Case Reports in Psychiatry ◽

10.1155/2017/5173605 ◽

2017 ◽

Vol 2017 ◽

pp. 1-3 ◽

Cited By ~ 3

Author(s):

Yuji Odagaki

Keyword(s):

Stress Disorder ◽

Brain Magnetic Resonance Imaging ◽

Psychological Trauma ◽

The Pacific ◽

Social Abilities ◽

Spectrum Disorders ◽

Magnetic Resonance Imaging Mri ◽

General Physical ◽

Own Name ◽

Northern Japan

Functional retrograde autobiographical amnesia is often associated with physical and/or psychological trauma. On 11 March 2011, the largest earthquake on record in Japan took place, and subsequent huge tsunami devastated the Pacific coast of northern Japan. This case report describes a patient suffering from retrograde episodic-autobiographical amnesia for whole life, persisting for even more than five years after the disaster. A Japanese man, presumably in his 40s, got police protection in April 2016 but was unable to respond to question about his own name. He lost all information about his personal identity, and his memory was wholly lost until the disaster on 11 March 2011. He was able to recall his life after the disaster, and semantic memories and social abilities were largely preserved. A medical examination performed on 1 November 2016 verified that he was awake, alert, and oriented to time, place, and person (except for himself). General physical and neurological examinations revealed no pathological findings. He also experienced some symptoms associated with posttraumatic stress disorder (PTSD), such as intrusive thoughts, flashbacks, and nightmares. No abnormalities were detected by biochemical test and brain magnetic resonance imaging (MRI). Physicians and other professionals who take care of victims of disaster should be aware of dissociative spectrum disorders, such as psychogenic amnesia.

Download Full-text