scholarly journals Prognostic relevance of MOG antibodies in children with an acquired demyelinating syndrome

Neurology ◽  
2017 ◽  
Vol 89 (9) ◽  
pp. 900-908 ◽  
Author(s):  
Eva-Maria Hennes ◽  
Matthias Baumann ◽  
Kathrin Schanda ◽  
Banu Anlar ◽  
Barbara Bajer-Kornek ◽  
...  
Keyword(s):  
Age At Onset ◽  
Disease Onset ◽  
Acute Disseminated Encephalomyelitis ◽  
Oligoclonal Bands ◽  
First Episode ◽  
Cell Counts ◽  
Csf Cells ◽  
Younger Age ◽  
Mog Antibodies

Objective:To assess the prognostic value of MOG antibodies (abs) in the differential diagnosis of acquired demyelinating syndromes (ADS).Methods:Clinical course, MRI, MOG-abs, AQP4-abs, and CSF cells and oligoclonal bands (OCB) in children with ADS and 24 months of follow-up were reviewed in this observational prospective multicenter hospital-based study.Results:Two hundred ten children with ADS were included and diagnosed with acute disseminated encephalomyelitis (ADEM) (n = 60), neuromyelitis optica spectrum disorder (NMOSD) (n = 12), clinically isolated syndrome (CIS) (n = 101), and multiple sclerosis (MS) (n = 37) after the first episode. MOG-abs were predominantly found in ADEM (57%) and less frequently in NMOSD (25%), CIS (25%), or MS (8%). Increased MOG-ab titers were associated with younger age (p = 0.0001), diagnosis of ADEM (p = 0.005), increased CSF cell counts (p = 0.011), and negative OCB (p = 0.012). At 24-month follow-up, 96 children had no further relapses. Thirty-five children developed recurrent non-MS episodes (63% MOG-, 17% AQP4-abs at onset). Seventy-nine children developed MS (4% MOG-abs at onset). Recurrent non-MS episodes were associated with high MOG-ab titers (p = 0.0003) and older age at onset (p = 0.024). MS was predicted by MS-like MRI (p < 0.0001) and OCB (p = 0.007). An MOG-ab cutoff titer ≥1:1,280 predicted a non-MS course with a sensitivity of 47% and a specificity of 100% and a recurrent non-MS course with a sensitivity of 46% and a specificity of 86%.Conclusions:Our results show that the presence of MOG-abs strongly depends on the age at disease onset and that high MOG-ab titers were associated with a recurrent non-MS disease course.

Clinical and diagnostic aspects of multiple sclerosis and acute monophasic encephalomyelitis in pediatric patients: a single centre prospective study

2009 ◽  
Vol 15 (3) ◽  
pp. 363-370 ◽  
Author(s):  
M Atzori ◽  
PA Battistella ◽  
P Perini ◽  
M Calabrese ◽  
M Fontanin ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Prospective Study ◽  
Pediatric Patients ◽  
Age At Onset ◽  
Acute Disseminated Encephalomyelitis ◽  
First Episode ◽  
Laboratory Findings ◽  
Clinical Onset ◽  
Magnetic Resonance Imaging Mri

Objective The purpose of the study was to compare and contrast the initial presenting demographic, clinical, neuroimaging, and laboratory features in a cohort of children affected from multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM). Methods A 12-year prospective study was conducted in 68 pediatric patients (age ≤ 17 years) who presented with a first episode of central nervous system inflammation suggestive of a demyelinating multifocal pathology. All patients had undergone magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) examination. The mean follow-up period, as at ending on December 31, 2007, was 6.8 ± 2.7 years (range 3.2–12.6 years). Results At clinical onset, children who developed MS during the follow-up (48 patients; 34 females, 14 males; mean age at onset: 14.4 ± 2.5) significantly differed from children affected by ADEM (20 patients; 8 females, 12 males; mean age at onset: 8.1 ± 3.8 ) for the following parameters: prevalence of females affected (female/male ratio: 2.8 versus 0.6, P = 0.03); mean age at onset ( P < 0.001); monosymptomatic onset (73% vs 30%, P = 0.002); encephalopathy-like onset (0% vs 50%, P < 0.001); presence of oligoclonal IgG bands (IgGOB) in CSF (83% vs 10%, P < 0.001); and periventricular (79% vs 20%, P < 0.001), brain stem (12.5% vs 60%, P = 0.000), and basal ganglia (10% vs 50%, P < 0.001) lesions at MRI. Conclusions Our findings depict a pattern of demographic, clinical, neuroimaging, and laboratory findings that can help to distinguish, at clinical onset, children suffering from ADEM from those who will develop MS. Childhood-onset MS seems not to differ from adult-onset MS from both clinical and paraclinical features.

Progressive Leukodystrophy-Like Demyelinating Syndromes with MOG-Antibodies in Children: A Rare Under-Recognized Phenotype

2021 ◽  
Author(s):  
Elise Yazbeck ◽  
Hélène Maurey ◽  
Carole Leroy ◽  
Philippe Horellou ◽  
Silvia Napuri ◽  
...  
Keyword(s):  
Brain Magnetic Resonance Imaging ◽  
Brain Biopsy ◽  
Active Immunotherapy ◽  
Oligoclonal Bands ◽  
Second Child ◽  
Fluid Analysis ◽  
Appropriate Treatment ◽  
Neurological Prognosis ◽  
Mog Antibodies

AbstractAcquired demyelinating syndromes (ADS) are frequently associated with myelin oligodendrocytes glycoprotein (MOG) antibodies in children. Clinical phenotypes are heterogeneous and may delay the diagnosis, especially when they relapse and are atypical, mimicking diseases such as multiple sclerosis or neuromyelitis optica spectrum disorders . Here, we describe two children: one with a progressive cognitive and behavioral deterioration with seizures after only one relapse and the other with similar clinical impairments associated with multiple relapses. Brain magnetic resonance imaging revealed a subsequent progressive leukodystrophy-like lesion with diffuse bilateral white matter injuries in both patients. Cerebrospinal fluid analysis showed pleiocytosis, increased level of proteins with no oligoclonal bands. Metabolic and inflammatory blood markers were all negative. Brain biopsy was performed in the second child and nonspecific inflammatory lesions with no argument for histiocytosis or tumor were observed. Clinical and radiological stabilization were obtained after active immunotherapy. Retrospective analysis of anti-MOG antibodies in these two children was positive at the earlier stage of the disease and turned negative after treatment and during follow-up. Leukodystrophy-like ADS with anti-MOG-antibodies may display distinct progressive phenotype and have a severe neurological prognosis. Early diagnosis and appropriate treatment may improve outcome in these children.

Temporomandibular Joint Involvement in Children with Juvenile Idiopathic Arthritis

2010 ◽  
Vol 38 (3) ◽  
pp. 510-515 ◽  
Author(s):  
ELVIRA CANNIZZARO ◽  
SILKE SCHROEDER ◽  
LUKAS M. MÜLLER ◽  
CHRISTIAN J. KELLENBERGER ◽  
ROTRAUD K. SAURENMANN
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Temporomandibular Joint ◽  
Upper Extremity ◽  
Age At Onset ◽  
Disease Onset ◽  
Joint Involvement ◽  
Hla B27 ◽  
Radiological Findings ◽  
Rheumatology Clinic ◽  
Younger Age

Objective.To determine the rate of temporomandibular joint (TMJ) involvement and find factors associated with TMJ arthritis in a single-center cohort of patients with juvenile idiopathic arthritis (JIA).Methods.Retrospective analysis of all patients with JIA visiting the rheumatology clinic between January 1, 2005, and December 31, 2006. Followup information was included until August 2008. A diagnosis of TMJ arthritis was based on clinical rheumatological and/or radiological findings.Results.After a mean followup time for JIA of 4.6 years (range 0.08–14.17), 86/223 patients (38.6%) had developed TMJ arthritis. The rate of TMJ involvement differed significantly among JIA subtypes (p = 0.0016), with 61% in extended oligoarticular, 52% in polyarticular rheumatoid factor (RF)-negative, 50% in psoriatic, 36% in systemic, 33% in polyarticular RF-positive, 33% in persistent oligoarticular, 30% in unclassified JIA, and 11% in enthesitis-related arthritis. The rate of TMJ involvement in our cohort was statistically significantly lower for patients who were HLA-B27-positive (p = 0.0002). In a multivariate analysis, the association of the following factors was confirmed: JIA subtype (p = 0.0001), a higher erythrocyte sedimentation rate (ESR) at diagnosis (p = 0.0038), involvement of joints of the upper extremity (p = 0.011), the absence of HLA-B27 (p = 0.023), and younger age at onset of JIA (p = 0.050).Conclusion.In our cohort of children with JIA, the overall rate of TMJ involvement was 38.6%. Patients with certain JIA subtypes, a higher ESR at disease onset, involvement of upper extremity joints, and younger age at diagnosis were more likely to develop TMJ arthritis. The presence of HLA-B27 seemed to be protective.

scholarly journals Acute disseminated encephalomyelitis followed by recurrent or monophasic optic neuritis in pediatric patients

2012 ◽  
Vol 19 (7) ◽  
pp. 941-946 ◽  
Author(s):  
Peter Huppke ◽  
Kevin Rostasy ◽  
Michael Karenfort ◽  
Brenda Huppke ◽  
Rainer Seidl ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Optic Neuritis ◽  
Pediatric Patients ◽  
Case Series ◽  
Acute Disseminated Encephalomyelitis ◽  
Oligoclonal Bands ◽  
Central Nervous System Disorders ◽  
Established Disease

Background: Some pediatric patients with inflammatory demyelinating central nervous system disorders cannot be classified under any of the established disease entities, making their treatment and prognosis difficult. Objective: The objective of this study is to characterize a subgroup of pediatric patients with recurrent demyelinating central nervous system disorders. Methods: This study includes a case series of pediatric patients with monophasic or recurrent acute disseminated encephalomyelitis (ADEM) who later presented with either monophasic or recurrent optic neuritis (ON). Results: We describe seven patients with a median follow-up of six years (five females, two males) who presented at a median age of 6 years (range 4–8 years) with monophasic ( n = 4) or recurrent ADEM (two to four attacks) followed by monophasic ( n = 3) or recurrent ON (two to nine attacks). Cranial magnetic resonance imaging (MRI) was typical for ADEM ( n = 6) with complete or almost complete resolution of lesions on follow-up. Cerebrospinal (CSF) studies at the time of ADEM showed a pleocytosis in six patients and were negative for oligoclonal bands (OCBs) in all. In all patients high titers for serum anti-MOG antibodies were detected. Conclusion: ADEM followed by ON is a rare but distinct clinical phenotype among pediatric patients. Further studies are needed to allow recommendations on treatment or prognosis.

scholarly journals Antipsychotic treatment resistance in first-episode psychosis: prevalence, subtypes and predictors

2017 ◽  
Vol 47 (11) ◽  
pp. 1981-1989 ◽  
Author(s):  
A. Demjaha ◽  
J. M. Lappin ◽  
D. Stahl ◽  
M. X. Patel ◽  
J. H. MacCabe ◽  
...  
Keyword(s):  
Negative Symptoms ◽  
Age At Onset ◽  
Treatment Resistance ◽  
First Episode Psychosis ◽  
First Episode ◽  
Antipsychotic Treatment ◽  
Treatment Resistant ◽  
Illness Onset ◽  
Episode Psychosis

BackgroundWe examined longitudinally the course and predictors of treatment resistance in a large cohort of first-episode psychosis (FEP) patients from initiation of antipsychotic treatment. We hypothesized that antipsychotic treatment resistance is: (a) present at illness onset; and (b) differentially associated with clinical and demographic factors.MethodThe study sample comprised 323 FEP patients who were studied at first contact and at 10-year follow-up. We collated clinical information on severity of symptoms, antipsychotic medication and treatment adherence during the follow-up period to determine the presence, course and predictors of treatment resistance.ResultsFrom the 23% of the patients, who were treatment resistant, 84% were treatment resistant from illness onset. Multivariable regression analysis revealed that diagnosis of schizophrenia, negative symptoms, younger age at onset, and longer duration of untreated psychosis predicted treatment resistance from illness onset.ConclusionsThe striking majority of treatment-resistant patients do not respond to first-line antipsychotic treatment even at time of FEP. Clinicians must be alert to this subgroup of patients and consider clozapine treatment as early as possible during the first presentation of psychosis.

scholarly journals Retrospective analysis of 73 cases of elastofibroma

2020 ◽  
Vol 102 (2) ◽  
pp. 84-93
Author(s):  
R Haihua ◽  
W Xiaobing ◽  
P Jie ◽  
H Xinxin
Keyword(s):  
Soft Tissue ◽  
Age At Onset ◽  
Disease Onset ◽  
Pathological Examination ◽  
Soft Tissue Tumour ◽  
Tissue Tumour ◽  
Short And Long Term ◽  
The Right

Objective Elastofibroma is a rare soft-tissue tumour. This study retrospectively analysed and summarised the clinical, imaging and typical pathological features, together with the short- and long-term surgical outcomes of patients with pathologically confirmed soft-tissue elastofibroma to improve their management. Materials and methods We enrolled 73 patients with pathologically confirmed soft-tissue elastofibroma from January 2010 to December 2018. The general, clinical, diagnostic and treatment-related data, operation notes, pathological examination results and follow-up status were obtained by reviewing inpatient medical records. Disease onset age, sex, tumour location and size were statistically analysed using the chi square and rank sum tests. Results A total of 90 lesions from 73 patients were examined. Among these, 56 patients had single lesions: 27 were under the right scapula, 26 were under the left scapula, 1 at the umbilicus, 1 on the aortic valve, 1 on the right hip and 17 at the bilateral inferior angles of the scapula. The average age at onset was 56.4 years (range: 6–82 years). The male-to-female incidence ratio was about one to three. Tumour diameter and follow-up duration ranged from 2cm to 12cm and from one month to nine years, respectively; recurrence was not observed. The main postoperative complication was wound effusion, occurring in 24 sites among the 90 lesions, corresponding to an incidence rate of 26.7%. Conclusions A correct diagnosis of elastofibroma can be made prior to surgical resection by examining typical clinical features and characteristic imaging findings. Short- and long-term outcomes of local excision are good, with no further recurrence.

scholarly journals Short Term Presence of Subretinal Fluid in Central Serous Chorioretinopathy Affects Retinal Thickness and Function

2020 ◽  
Vol 9 (11) ◽  
pp. 3429
Author(s):  
Maciej Gawęcki ◽  
Agnieszka Jaszczuk ◽  
Andrzej Grzybowski
Keyword(s):  
Central Serous Chorioretinopathy ◽  
Retinal Thickness ◽  
Fundus Autofluorescence ◽  
Disease Onset ◽  
Subretinal Fluid ◽  
First Episode ◽  
Inclusion Criteria ◽  
Duration Of Symptoms ◽  
Short Period

Background: Acute central serous chorioretinopathy (CSCR), with subretinal fluid (SRF) resolving spontaneously within a few months from disease onset, has been considered as a benign and self-limiting disease for many years. This study sought to discover if a short presence of SRF can result in morphological and functional damage to the retina. Materials and methods: The study included patients treated by subthreshold diode micropulse laser (SDM) application for acute CSCR at the Dobry Wzrok Ophthalmological Clinic between January 2018 and November 2019. Inclusion criteria were: first episode of CSCR; duration of symptoms of two months or less; complete resolution of subretinal fluid (SRF) after a single session of SDM; and a lack of any retinal pathology, previous CSCR episode, significant anisometropia or amblyopia in the collateral eye. Fifteen patients fulfilled the inclusion criteria, including 13 males and two females aged 42.3 ± 9.5 years. The mean duration of symptoms before treatment was 4.7 ± 1.3 weeks on average. Baseline and follow-up examinations were performed in both the affected and collateral eyes and included best-corrected visual acuity (BCVA); spectral-domain optical coherent tomography measurements such as central retinal thickness (CRT) and minimal foveal thickness (MFT) (at the follow-up visit only); fluorescein angiography (at presentation only) and fundus autofluorescence. The first follow-up visit, when the total resolution of SRF was noted, was conducted between 8 and 12 weeks after SDM. Results: Resolved CSCR eyes had significantly poorer BCVA, CRT, and MFT findings in comparison with healthy collateral eyes (respectively, 0.11 +/− 0.1 vs. 0.01 +/− 0.04 logMAR; 238.80 +/− 23.39 vs. 264.87 +/− 21.22 µm and 178.93 +/− 16.88 vs. 199.47 +/− 17.87 µm) despite the short period of CSCR duration (maximum of 14 ± 2.15 weeks on average). Conclusion: Short presence of SRF typical for acute CSCR can affect retinal function and morphology resulting in poorer visual outcome.

scholarly journals Fate of contralateral asymptomatic bullae in patients with primary spontaneous pneumothorax

2020 ◽  
Vol 58 (2) ◽  
pp. 365-370
Author(s):  
Hyo Jun Jang ◽  
Jun Ho Lee ◽  
Seung Hyuk Nam ◽  
Sun Kyun Ro
Keyword(s):  
Spontaneous Pneumothorax ◽  
Multivariable Analysis ◽  
Primary Spontaneous Pneumothorax ◽  
First Episode ◽  
High Resolution Computed Tomography ◽  
Contralateral Lung ◽  
Younger Age ◽  
Independent Risk Factors ◽  
The Impact

Abstract OBJECTIVES This retrospective cohort study aimed to analyse the impact of asymptomatic blebs/bullae on the occurrence of primary spontaneous pneumothorax (PSP) by monitoring the natural course of contralateral blebs/bullae in patients with ipsilateral pneumothorax. METHODS From January 2003 to December 2017, 1055 patients [age 19.6 ± 3.98 years (mean ± standard deviation), 953 men] experiencing the first episode of unilateral PSP were enrolled in this study, excluding patients aged 30 years or more. The presence, number and maximal size of the blebs/bullae were investigated in contralateral asymptomatic lungs based on high-resolution computed tomography. RESULTS Multiple and single blebs/bullae were noted in contralateral lungs in 425 (40.3%) and 88 (8.3%) patients, respectively. The median follow-up period was 44.0 (interquartile range 71.5) months. The 1-, 3- and 5-year cumulative occurrence rates of PSP in contralateral lungs were 7.9%, 13.7% and 16.7%, respectively. On multivariable analysis, younger age [hazard ratio (HR) 1.19, 95% confidence interval (CI) 1.12–1.27; P &lt; 0.001) and multiple bullae (HR 4.42, 95% CI 3.06–6.38; P &lt; 0.001) were independent risk factors for spontaneous pneumothorax in the contralateral lung. The 5-year cumulative occurrence rates of PSP were significantly higher in patients with multiple blebs/bullae than in those with no or a single bleb/bulla (28.2% vs 8.5%, respectively; P &lt; 0.001). CONCLUSIONS Asymptomatic blebs/bullae often lead to PSP. If the patient is eligible for surgery for pneumothorax, preemptive surgery for contralateral bullae could be considered, especially in patients with multiple blebs/bullae.

Comparative analysis of immunosuppressive therapies for myelin oligodendrocyte glycoprotein antibody-associated optic neuritis: a cohort study

2021 ◽  
pp. bjophthalmol-2020-318769
Author(s):  
Lindan Xie ◽  
Huanfen Zhou ◽  
Honglu Song ◽  
Mingming Sun ◽  
Mo Yang ◽  
...  
Keyword(s):  
Cohort Study ◽  
Optic Neuritis ◽  
Cohort Analysis ◽  
Age At Onset ◽  
Disease Onset ◽  
Treated Group ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Class Iii ◽  
Chinese Cohort

AimsThe optimal immunosuppressive therapy (IST) in patients with myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) remains uncertain. This study aimed to observe the disease course of MOG-ON and evaluate the therapeutic efficacy and tolerability of conventional immunosuppressants through Chinese cohort analysis.MethodsThis bidirectional cohort study included 121 patients with MOG-ON between January 2015 and December 2018. The clinical features and annualised relapse rate (ARR) of patients with and without IST were analysed.ResultsThe median age at onset was 17.5 years, and the sex ratio (F:M) was 1.24. Of 121 patients, 77 patients relapsed and 61 patients were younger than 18 years at disease onset. The overall median ARR of 63 patients in the non-IST group was 0.5, with 46.0% patients showing relapse at a median follow-up of 33.5 months. In the IST group, the ARR decreased from 1.75 pre-IST to 0.00 post-IST in 53 patients who received IST exceeding 6 months, with 20.8% patients showing relapse at a median follow-up of 23.8 months. The relapse rates of patients treated with rituximab (RTX) and mycophenolate mofetil (MMF) were not statistically different, but the rate of discontinuation was significantly lower in the RTX-treated group (18.2% vs 57.7%, p=0.0017).ConclusionThis study provides Class III evidence that both MMF and RTX may lower disease activity in patients with MOG-ON, and RTX showed better tolerability than MMF. However, observation after a single attack remains a good option because less than half of patients not on treatment suffered a relapse.

scholarly journals Patterns of pain over time among children with juvenile idiopathic arthritis

2017 ◽  
Vol 103 (5) ◽  
pp. 437-443 ◽  
Author(s):  
Amir Rashid ◽  
Lis Cordingley ◽  
Roberto Carrasco ◽  
Helen E Foster ◽  
Eileen M Baildam ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Multinomial Logistic Regression ◽  
Age At Onset ◽  
Management Strategies ◽  
Disease Onset ◽  
Common Symptom ◽  
Pain Outcomes ◽  
First Pain ◽  
Over Time

ObjectivesPain is a very common symptom of juvenile idiopathic arthritis (JIA). Disease activity alone cannot explain symptoms of pain in all children, suggesting other factors may be relevant. The objectives of this study were to describe the different patterns of pain experienced over time in children with JIA and to identify predictors of which children are likely to experience ongoing pain.MethodsThis study used longitudinal-data from patients (aged 1–16 years) with new-onset JIA. Baseline and up to 5-year follow-up pain data from the Childhood Arthritis Prospective Study (CAPS) were used. A two-step approach was adopted. First, pain trajectories were modelled using a discrete mixture model. Second, multinomial logistic regression was used to determine the association between variables and trajectories.ResultsData from 851 individuals were included (4 years, median follow-up). A three-group trajectory model was identified: consistently low pain (n=453), improved pain (n=254) and consistently high pain (n=144). Children with improved pain or consistently high pain differed on average at baseline from consistently low pain. Older age at onset, poor function/disability and longer disease duration at baseline were associated with consistently high pain compared with consistently low pain. Early increases in pain and poor function/disability were also associated with consistently high pain compared with consistently low pain.ConclusionsThis study has identified routinely collected clinical factors, which may indicate those individuals with JIA at risk of poor pain outcomes earlier in disease. Identifying those at highest risk of poor pain outcomes at disease onset may enable targeted pain management strategies to be implemented early in disease thus reducing the risk of poor pain outcomes.

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