Neuromyelitis optica in Brazil: a study on clinical and prognostic factors

2009 ◽  
Vol 15 (5) ◽  
pp. 613-619 ◽  
Author(s):  
DB Bichuetti ◽  
EML Oliveira ◽  
NA Souza ◽  
RLM Rivero ◽  
AA Gabbai

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.

2008 ◽  
Vol 66 (1) ◽  
pp. 1-4 ◽  
Author(s):  
Denis Bernardi Bichuetti ◽  
René Leandro Magalhães Rivero ◽  
Daniel May Oliveira ◽  
Nilton Amorin de Souza ◽  
Nitamar Abdala ◽  
...  

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.


2019 ◽  
Vol 6 (Supplement_2) ◽  
pp. S515-S515
Author(s):  
Ambreen Allana ◽  
Mohammed Samannodi ◽  
Michael Hansen ◽  
Rodrigo Hasbun

Abstract Background To describe the use and the impact on clinical outcomes of adjunctive steroids in adults with encephalitis. Methods We conducted a retrospective observational study of 230 adults (age >17 years) who met the international consortium definition for encephalitis. An adverse clinical outcome was defined as a Glasgow outcome score of 1–4. The study took place at three tertiary care hospitals in Houston TX, between August 2008 and September 2017. Results A total of 230 adults with encephalitis were enrolled, out of which 121 (52.6%) received steroids. Adjunctive steroids were given more frequently to those with focal neurological deficits (P = 0.013), had a positive cerebrospinal fluid (CSF) HSV PCR (P = 0.013), required mechanical ventilation (MV) (P = 0.011), required intensive care unit (ICU) admission (P < 0.001), had white matter abnormalities (P = 0.014) or had cerebral edema on the brain magnetic resonance imaging (MRI) (P = 0.003). An adverse outcome was seen in 139 (60.7%) of patients. Predictors for adverse outcomes included a Glasgow coma score (GCS) < 8, fever, seizures, ICU admission, and presence of edema on brain MRI. The use of adjunctive steroids did not impact clinical outcomes (P = 0.521). Independent prognostic factors on logistic regression analysis were edema on brain MRI (7.780 [1.717–35.263] P = 0.008), GCS < 8 (6.339 [1.992–20.168] P = 0.002), and fever (2.601 [1.342–5.038] P = 0.005). Conclusion Adults with encephalitis continues to be associated with significant adverse clinical outcomes in the majority of patients. Adjunctive steroids are used in the sicker patients and it is not associated with improved clinical outcomes. Disclosures All authors: No reported disclosures.


2009 ◽  
Vol 67 (3a) ◽  
pp. 633-638 ◽  
Author(s):  
Marco O. Py ◽  
Leonardo Maciel ◽  
Roberto C. Pedrosa ◽  
Jose H. M. Nascimento ◽  
Emiliano Medei

We previously demonstrated correlation between parasympathetic dysfunction and brain white matter lesions in chronic chagasic patients. OBJECTIVE: To correlate serum functional circulating antibodies with beta adrenergic (Ab-β), muscarinic (Ab-M) or muscarinic and beta adrenergic (Ab-Mβ) activity, the autonomic system function and brain lesions in chronic chagasic patients. METHOD: In fifteen consecutive chagasic patients, the autonomic nervous system was evaluated and brain magnetic resonance imaging (MRI) was performed. The sera of all patients were tested to the presence of circulating functional antibodies. RESULTS: Sera from 11 of 15 chronic chagasic patients had some activity (Ab-β: 7; Ab-M: 1; Ab-Mβ: 3); however, there was no significant correlation between the presence of antibodies and the autonomic system function or the presence of hyperintensities in MRI. CONCLUSION: The mechanism involved in the genesis of hyperintense lesions seen in brain MRI of chronic chagasic patients is still unresolved, although apparently related to parasympathetic dysfunction.


2007 ◽  
Vol 14 (2) ◽  
pp. 248-251 ◽  
Author(s):  
JA Cabrera-Gómez ◽  
A. Saiz-Hinarejos ◽  
F. Graus ◽  
A. González-Quevedo ◽  
R. Rodríguez-Rojas ◽  
...  

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


2013 ◽  
Vol 20 (6) ◽  
pp. 695-704 ◽  
Author(s):  
So-Young Huh ◽  
Ju-Hong Min ◽  
Woojun Kim ◽  
Su-Hyun Kim ◽  
Ho Jin Kim ◽  
...  

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.


Author(s):  
Neelu Desai ◽  
Rahul Badheka ◽  
Nitin Shah ◽  
Vrajesh Udani

AbstractReversible cerebral vasoconstriction syndrome (RCVS) has been well described in adults, but pediatric cases are yet under recognized. We describe two children with RCVS and review similar already published pediatric cases. The first patient was a 10-year-old girl who presented with severe headaches and seizures 3 days after blood transfusion. Brain magnetic resonance imaging (MRI) showed changes compatible with posterior reversible encephalopathy syndrome and subarachnoid hemorrhage. Magnetic resonance angiogram showed diffuse vasoconstriction of multiple cerebral arteries. The second patient was a 9-year-old boy who presented with severe thunderclap headaches. Brain MRI showed isolated intraventricular hemorrhage. Computed tomography/MR angiogram and digital subtraction angiogram were normal. A week later, he developed focal neurological deficits. Repeated MR angiogram showed diffuse vasospasm of multiple intracranial arteries. Both children recovered completely. A clinico-radiological review of previously reported childhood RCVS is provided.


2013 ◽  
Vol 71 (5) ◽  
pp. 275-279 ◽  
Author(s):  
Denis Bernardi Bichuetti ◽  
Enedina Maria Lobato de Oliveira ◽  
Nilton Amorin de Souza ◽  
Mar Tintoré ◽  
Alberto Alain Gabbai

Although neuromyelitis optica (NMO) is known to be a more severe disease than relapsing-remitting multiple sclerosis (RRMS), few studies comparing both conditions in a single center have been done.Methods:Comparison of our previously published cohort of 41 NMO patients with 177 RRMS patients followed in the same center, from 1994 to 2007.Results:Mean age of onset was 32.6 for NMO and 30.2 for RRMS (p=0.2062) with mean disease duration of 7.4 years for NMO and 10.3 years for RRMS. Patients with NMO had a higher annualized relapse rate (1.0 versus 0.8, p=0.0013) and progression index (0.9 versus 0.6, p≪0.0001), with more patients reaching expanded disability status scale (EDSS) 6.0 (39 versus 17%, p=0.0036). The odds ratio for reaching EDSS 6.0 and being deceased due to NMO in comparison to RRMS were, respectively, 3.14 and 12.15.Conclusion:Patients with NMO have a more severe disease than patients with RRMS, including higher risk of dying of a demyelinating disease.


Medicina ◽  
2021 ◽  
Vol 57 (8) ◽  
pp. 836
Author(s):  
In-Chul Nam ◽  
Hye-Jin Baek ◽  
Kyeong-Hwa Ryu ◽  
Jin-Il Moon ◽  
Eun Cho ◽  
...  

Background and objective: This study was conducted to assess the prevalence and clinical implications of parotid lesions detected incidentally during brain magnetic resonance imaging (MRI) examination. Materials and Methods: Between February 2016 and February 2021, we identified 86 lesions in the brain MRI reports of 84 patients that contained the words “parotid gland” or “PG”. Of these, we finally included 49 lesions involving 45 patients following histopathological confirmation. Results: Based on the laboratory, radiological or histopathological findings, the prevalence of incidental parotid lesions was low (1.2%). Among the 45 study patients, 41 (91.1%) had unilateral lesions, and the majority of the lesions were located in the superficial lobe (40/49, 81.6%). The mean size of the parotid lesions was 1.3 cm ± 0.4 cm (range, 0.5 cm–2.8 cm). Of these, 46 parotid lesions (93.9%) were benign, whereas the remaining three lesions were malignant (6.1%). Conclusions: Despite the low prevalence and incidence of malignancy associated with incidental parotid lesions detected on brain MRI, the clinical implications are potentially significant. Therefore, clinical awareness and appropriate imaging work-up of these lesions are important for accurate diagnosis and timely management.


2021 ◽  
Author(s):  
Jialin Pan ◽  
Begench Ovlyakulov ◽  
Lili zhou

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.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Cheng-Chung Li ◽  
Meng-Yun Wu ◽  
Ying-Chou Sun ◽  
Hung-Hsun Chen ◽  
Hsiu-Mei Wu ◽  
...  

AbstractThe extraction of brain tumor tissues in 3D Brain Magnetic Resonance Imaging (MRI) plays an important role in diagnosis before the gamma knife radiosurgery (GKRS). In this article, the post-contrast T1 whole-brain MRI images had been collected by Taipei Veterans General Hospital (TVGH) and stored in DICOM format (dated from 1999 to 2018). The proposed method starts with the active contour model to get the region of interest (ROI) automatically and enhance the image contrast. The segmentation models are trained by MRI images with tumors to avoid imbalanced data problem under model construction. In order to achieve this objective, a two-step ensemble approach is used to establish such diagnosis, first, classify whether there is any tumor in the image, and second, segment the intracranial metastatic tumors by ensemble neural networks based on 2D U-Net architecture. The ensemble for classification and segmentation simultaneously also improves segmentation accuracy. The result of classification achieves a F1-measure of $$75.64\%$$ 75.64 % , while the result of segmentation achieves an IoU of $$84.83\%$$ 84.83 % and a DICE score of $$86.21\%$$ 86.21 % . Significantly reduce the time for manual labeling from 30 min to 18 s per patient.


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