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Translational Cancer Research Oct 2022Neuroblastoma is the most common malignancy in children younger than seven years of age and is the most frequent extracranial solid tumor that occurs in childhood. While...
BACKGROUND
Neuroblastoma is the most common malignancy in children younger than seven years of age and is the most frequent extracranial solid tumor that occurs in childhood. While opsoclonus-myoclonus syndrome (OMS), a paraneoplastic neurologic syndrome, affects 2-3% of children with neuroblastoma, and the percentage of mediastinal localization of the tumor is 49%. The objective of this study was to identify and characterize features of the OMS syndrome and treatments of mediastinal and non-mediastinal neuroblastoma associated with OMS.
METHODS
A systematic review of the literature was performed using PubMed, Medline, Web of Science, Embase and Cochrane. The search has no limit on date with the last search done on Dec 31, 2020. There is no publication restrictions or study design filters applied in the search.
RESULTS
Fifty-five out of 242 papers were identified and met our study eligibility. There were 77 cases found (28 cases had Mediastinal neuroblastoma, and 49 cases had non-mediastinal neuroblastoma). Data from trials showed that cases with mediastinal neuroblastoma who seemed to have undergone less treatment for OMS [rate ratio (RR) 0.41 (95% CI: 0.22-0.76)] had resulted in decreasing persistent neurologic symptoms [RR 0.31 (95% CI: 0.10-0.96)].
CONCLUSIONS
Children who have OMS and mediastinal neuroblastoma may be associated with more favorable clinical and biological characteristics and better outcomes than children who have OMS and non-mediastinal neuroblastoma, and they are more likely to present with a single neurological symptom at first. The OMS in mediastinal neuroblastoma might also be treated effectively through resection of the tumor followed by appropriate radiotherapy and chemotherapy, and no long-term treatments of OMS is indicated.
PubMed: 36388023
DOI: 10.21037/tcr-22-1120 -
Neurology(R) Neuroimmunology &... May 2020To describe the main syndrome and clinical course in a large cohort of patients with anti-Ri-associated paraneoplastic neurologic syndrome (Ri-PNS).
OBJECTIVE
To describe the main syndrome and clinical course in a large cohort of patients with anti-Ri-associated paraneoplastic neurologic syndrome (Ri-PNS).
METHODS
Twenty-year retrospective nationwide study and systematic review of the literature.
RESULTS
Thirty-six patients with complete clinical information were identified (median age 66 years, range: 47-87 years). In this French cohort, the majority were women (78%). At onset, 4 main patterns were observed: cerebellar syndrome (39%), isolated tremor (24%), oculomotor disturbances (17%), and other symptoms (19%). Course was multistep for 78% of cases. At the time the disease reached the plateau phase (median 12 weeks, range: 1-64 weeks; 28% >3 months), 24 (67%) showed an overt cerebellar syndrome, which was isolated in 3 patients, and was most frequently (21/24 cases) part of a multisystem neurologic disease. Patients manifested a variety of movement disorders, including myoclonus (33%), dystonia (17%), either cervical or oromandibular, and parkinsonism (17%). Most patients had cancer (92%), mainly breast cancer (n = 22). Misdiagnoses concerned 22% of patients (n = 8) and included atypical parkinsonism (n = 2), MS (n = 2), Bickerstaff encephalitis (n = 1), hyperekplexia (n = 1), vestibular neuritis (n = 1), and functional neurologic disorder (n = 1). Survival at 12 months was 73% (95% CI [0.54-0.85]), at 24 months 62% (95% CI [0.41-0.78]), and at 36 months 47% (95% CI [0.25-0.65]). There was no major clinical difference between cases retrieved from the systematic review of the literature (n = 55) and the French cohort.
CONCLUSIONS
Ri-PNS is a multisystem neurologic syndrome with prominent cerebellum/brainstem involvement. Opsoclonus-myoclonus is less common than expected, and the disorder can mimic neurodegenerative diseases.
Topics: Aged; Aged, 80 and over; Female; France; Humans; Male; Middle Aged; Movement Disorders; Nerve Tissue Proteins; Neuro-Oncological Ventral Antigen; Paraneoplastic Cerebellar Degeneration; Paraneoplastic Syndromes, Nervous System; RNA-Binding Proteins; Retrospective Studies
PubMed: 32170042
DOI: 10.1212/NXI.0000000000000699 -
Clinical Neurology and Neurosurgery Feb 2023This study aims to describe the clinical characteristics of patients with isolated oculomotor nerve palsy from COVID-19 infection, and provide guidance on their...
AIM
This study aims to describe the clinical characteristics of patients with isolated oculomotor nerve palsy from COVID-19 infection, and provide guidance on their treatment and management.
METHODS
We performed a systematic review and retrospective analysis on the clinical features and outcomes of patients with isolated oculomotor nerve palsy from COVID-19 reported in literature over the past three years.
RESULTS
We analyzed a total of 11 cases; 9 identified in literature from January 2020 to September 2022, together with our two patients. Their median age was 46 years (range 2-65), and three were children. More than half (6/11, 55 %) were without medical history. Oculomotor nerve palsies tended to occur early (longest interval of 16 days), but they can also occur concurrently (2/11, 18 %) or before the appearance of COVID-19 symptoms (1/11, 9 %). COVID-19 symptoms tended to be mild (8/11, 73 %). Oculomotor nerve palsies, however, displayed neither a clear gender predilection, nor consistent clinical features in terms of the severity of extraocular weakness and the involvement of pupillary light responses. Nearly two-thirds (7/11, 64 %) received no pharmacological treatment. Regardless, recovery was complete in nearly all (9/10, 90 %), with most occurring within a month (8/9, 89 %) CONCLUSION: Isolated oculomotor nerve palsies are early but uncommon complications of COVID-19. They affect patients with mild infections, and can be the first symptom. Prognosis is excellent, with recovery being often complete and early. Early discharge and outpatient clinical review, with or without short courses of oral steroids, are reasonable treatment measures.
Topics: Child; Humans; Child, Preschool; Adolescent; Young Adult; Adult; Middle Aged; Aged; Retrospective Studies; COVID-19; Oculomotor Nerve Diseases; Prognosis; Oculomotor Nerve
PubMed: 36696848
DOI: 10.1016/j.clineuro.2023.107601 -
Eye (London, England) Mar 2021Amblyopia screening can target reduced visual acuity (VA), its refractive risk factors, or both. VA testing is imprecise under 4 years of age, so automated risk-factor... (Review)
Review
BACKGROUND
Amblyopia screening can target reduced visual acuity (VA), its refractive risk factors, or both. VA testing is imprecise under 4 years of age, so automated risk-factor photoscreening appears an attractive option. This review considers photoscreening used in community services, focusing on costs, cost-effectiveness and scope of use, compared with EUSCREEN project Country Reports describing how photo- and automated screening is used internationally.
METHODS
A systematic narrative review was carried out of all English language photoscreening literature to September 10th 2018, using publicly available search terms. Where costs were considered, a CASP economic evaluation checklist was used to assess data quality.
RESULTS
Of 370 abstracts reviewed, 55 reported large-scale community photoscreening projects. Five addressed cost-effectiveness specifically, without original data. Photoscreening was a stand-alone, single, test event in 71% of projects. In contrast, 25 of 45 EUSCREEN Country Reports showed that if adopted, photoscreening often supplements other tests in established programmes and is rarely used as a stand-alone test. Reported costs varied widely and evidence of cost-effectiveness was sparse in the literature, or in international practice. Only eight (13%) papers compared the diagnostic accuracy or cost-effectiveness of photoscreening and VA testing, and when they did, cost-effectiveness of photoscreening compared unfavourably.
DISCUSSION
Evidence that photoscreening reduces amblyopia or strabismus prevalence or improves overall outcomes is weak, as is evidence of cost-effectiveness, compared to later VA screening. Currently, the most cost-effective option seems to be a later, expert VA screening with the opportunity for a re-test before referral.
Topics: Amblyopia; Child; Cost-Benefit Analysis; Humans; Strabismus; Vision Disorders; Vision Screening
PubMed: 33257800
DOI: 10.1038/s41433-020-01261-8 -
Journal of Neural Transmission (Vienna,... Oct 2021Differential diagnosis of parkinsonian syndromes is considered one of the most challenging in neurology. Quantitative MR planimetric measurements were reported to... (Meta-Analysis)
Meta-Analysis Review
Differential diagnosis of parkinsonian syndromes is considered one of the most challenging in neurology. Quantitative MR planimetric measurements were reported to discriminate between progressive supranuclear palsy (PSP) and non-PSP-parkinsonism. Several studies have used midbrain to pons ratio (M/P) and the Magnetic Resonance Parkinsonism Index (MRPI) in distinguishing PSP patients from those with Parkinson's disease. The current meta-analysis aimed to compare the performance of these measures in discriminating PSP from multiple system atrophy (MSA). A systematic MEDLINE review identified 59 out of 2984 studies allowing a calculation of sensitivity and specificity using the MRPI or M/P. Meta-analyses of results were carried out using random effects modelling. To assess study quality and risk of bias, the QUADAS-2 tool was used. Eight studies were suitable for analysis. The meta-analysis showed a pooled sensitivity and specificity for the MRPI of PSP versus MSA of 79.2% (95% CI 72.7-84.4%) and 91.2% (95% CI 79.5-96.5%), and 84.1% (95% CI 77.2-89.2%) and 89.2% (95% CI 81.8-93.8%), respectively, for the M/P. The QUADAS-2 toolbox revealed a high risk of bias regarding the methodological quality of patient selection and index test, as all patients were seen in a specialized outpatient department without avoiding case control design and no predefined threshold was given regarding MRPI or M/P cut-offs. Planimetric brainstem measurements, in special the MRPI and M/P, yield high diagnostic accuracy for the discrimination of PSP from MSA. However, there is an urgent need for well-designed, prospective validation studies to ameliorate the concerns regarding the risk of bias.
Topics: Humans; Magnetic Resonance Imaging; Multiple System Atrophy; Parkinson Disease; Parkinsonian Disorders; Supranuclear Palsy, Progressive
PubMed: 34105000
DOI: 10.1007/s00702-021-02362-8 -
Environmental Science and Pollution... May 2021Recently, during the pandemic infection of the novel SARS-CoV-2, some cases of Miller Fisher syndrome (MFS) have been reported. We want to summarize the main features of... (Review)
Review
Recently, during the pandemic infection of the novel SARS-CoV-2, some cases of Miller Fisher syndrome (MFS) have been reported. We want to summarize the main features of patients with MFS and COVID-19. A PubMed search was performed on 8 October to identify references reporting cases with MFS associated with COVID-19 from the first report of COVID-19 to 8 October 2020 using the following keywords: "Miller Fisher syndrome" AND "COVID-19" OR "SARS-CoV-2". A systematic review from the first report of coronavirus disease 2019 (COVID-19) to 8 October 2020 revealed 7 cases with Miller Fisher syndrome (MFS) associated with COVID-19. The 7 cases came from 5 countries but most of these patients were from Europe (85.7%), especially Spain. There are 5 cases of MFS diagnosed after the laboratory confirmation of SARS-CoV-2 infection. The mean onset time of MFS-associated neurological symptoms was 14.75 days after the diagnosis of COVID-19. However, the two remaining cases presented initially with MFS-associated neurological symptoms followed by the diagnosis of COVID-19. The most common symptoms of COVID-19-associated MFS were perioral paresthesias (57.1%), ataxia (57.1%), blurred vision (42.9), ophthalmoplegia (42.9), and generalized areflexia (42.9). However, more cohort and case-control studies are required to establish the epidemiological linkage.
Topics: COVID-19; Europe; Humans; Miller Fisher Syndrome; SARS-CoV-2; Spain
PubMed: 33677662
DOI: 10.1007/s11356-021-13233-w -
BMC Neurology Sep 2023Miller Fisher syndrome (MFS) is a subtype of Guillain-Barré syndrome (GBS) which is characterized by the three components of ophthalmoplegia, ataxia, and areflexia....
BACKGROUND
Miller Fisher syndrome (MFS) is a subtype of Guillain-Barré syndrome (GBS) which is characterized by the three components of ophthalmoplegia, ataxia, and areflexia. Some studies reported MFS as an adverse effect of the COVID-19 vaccination. We aimed to have a detailed evaluation on demographic, clinical, and para-clinical characteristics of subjects with MFS after receiving COVID-19 vaccines.
MATERIALS AND METHODS
A thorough search strategy was designed, and PubMed, Web of Science, and Embase were searched to find relevant articles. Each screening step was done by twice, and in case of disagreement, another author was consulted. Data on different characteristics of the patients and types of the vaccines were extracted. The risk of bias of the studies was assessed using Joanna Briggs Institute (JBI) tools.
RESULTS
In this study, 15 patients were identified from 15 case studies. The median age of the patients was 64, ranging from 24 to 84 years. Ten patients (66.6%) were men and Pfizer made up 46.7% of the injected vaccines. The median time from vaccination to symptoms onset was 14 days and varied from 7 to 35 days. Furthermore,14 patients had ocular signs, and 78.3% (11/14) of ocular manifestations were bilateral. Among neurological conditions, other than MFS triad, facial weakness or facial nerve palsy was the most frequently reported side effect that was in seven (46.7%) subjects. Intravenous immunoglobulin (IVIg) was the most frequently used treatment (13/15, 86.7%). Six patients received 0.4 g/kg and the four had 2 g/kg. Patients stayed at the hospital from five to 51 days. No fatal outcomes were reported. Finally, 40.0% (4/15) of patients completely recovered, and the rest experienced improvement.
CONCLUSION
MFS after COVID-19 immunization has favorable outcomes and good prognosis. However, long interval from disease presentation to treatment in some studies indicates that more attention should be paid to MFS as the adverse effect of the vaccination. Due to the challenging diagnosis, MFS must be considered in list of the differential diagnosis in patients with a history of recent COVID-19 vaccination and any of the ocular complaints, ataxia, or loss of reflexes, specially for male patients in their 60s and 70s.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Young Adult; Ataxia; COVID-19; COVID-19 Vaccines; Drug-Related Side Effects and Adverse Reactions; Facial Paralysis; Miller Fisher Syndrome; Prognosis; Vaccination
PubMed: 37735648
DOI: 10.1186/s12883-023-03375-4 -
Journal of Neurology Apr 2024Parkinsonian disorders, including Parkinson's disease (PD), multiple system atrophy (MSA), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP), and... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND AND OBJECTIVE
Parkinsonian disorders, including Parkinson's disease (PD), multiple system atrophy (MSA), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS), exhibit overlapping early-stage symptoms, complicating definitive diagnosis despite heterogeneous cellular and regional pathophysiology. Additionally, the progression and the eventual conversion of prodromal conditions such as REM behavior disorder (RBD) to PD, MSA, or DLB remain challenging to predict. Extracellular vesicles (EVs) are small, membrane-enclosed structures released by cells, playing a vital role in communicating cell-state-specific messages. Due to their ability to cross the blood-brain barrier into the peripheral circulation, measuring biomarkers in blood-isolated speculative CNS enriched EVs has become a popular diagnostic approach. However, replication and independent validation remain challenging in this field. Here, we aimed to evaluate the diagnostic accuracy of speculative CNS-enriched EVs for parkinsonian disorders.
METHODS
We conducted a PRISMA-guided systematic review and meta-analysis, covering 18 studies with a total of 1695 patients with PD, 253 with MSA, 21 with DLB, 172 with PSP, 152 with CBS, 189 with RBD, and 1288 HCs, employing either hierarchical bivariate models or univariate models based on study size.
RESULTS
Diagnostic accuracy was moderate for differentiating patients with PD from HCs, but revealed high heterogeneity and significant publication bias, suggesting an inflation of the perceived diagnostic effectiveness. The bias observed indicates that studies with non-significant or lower effect sizes were less likely to be published. Although results for differentiating patients with PD from those with MSA or PSP and CBS appeared promising, their validity is limited due to the small number of involved studies coming from the same research group. Despite initial reports, our analyses suggest that using speculative CNS-enriched EV biomarkers may not reliably differentiate patients with MSA from HCs or patients with RBD from HCs, due to their lesser accuracy and substantial variability among the studies, further complicated by substantial publication bias.
CONCLUSION
Our findings underscore the moderate, yet unreliable diagnostic accuracy of biomarkers in speculative CNS-enriched EVs in differentiating parkinsonian disorders, highlighting the presence of substantial heterogeneity and significant publication bias. These observations reinforce the need for larger, more standardized, and unbiased studies to validate the utility of these biomarkers but also call for the development of better biomarkers for parkinsonian disorders.
Topics: Humans; Parkinsonian Disorders; Parkinson Disease; Multiple System Atrophy; Supranuclear Palsy, Progressive; REM Sleep Behavior Disorder; Biomarkers; Extracellular Vesicles; Diagnosis, Differential
PubMed: 38103086
DOI: 10.1007/s00415-023-12093-3 -
The Cochrane Database of Systematic... Nov 2017Strabismus (misalignment of the eyes) is a risk factor for impaired visual development both of visual acuity and of stereopsis. Detection of strabismus in the community... (Review)
Review
BACKGROUND
Strabismus (misalignment of the eyes) is a risk factor for impaired visual development both of visual acuity and of stereopsis. Detection of strabismus in the community by non-expert examiners may be performed using a number of different index tests that include direct measures of misalignment (corneal or fundus reflex tests), or indirect measures such as stereopsis and visual acuity. The reference test to detect strabismus by trained professionals is the cover‒uncover test.
OBJECTIVES
To assess and compare the accuracy of tests, alone or in combination, for detection of strabismus in children aged 1 to 6 years, in a community setting by non-expert screeners or primary care professionals to inform healthcare commissioners setting up childhood screening programmes.Secondary objectives were to investigate sources of heterogeneity of diagnostic accuracy.
SEARCH METHODS
We searched the Cochrane Central Register of Controlled Trials (CENTRAL; 2016, Issue 12) (which contains the Cochrane Eyes and Vision Trials Register) in the Cochrane Library, the Health Technology Assessment Database (HTAD) in the Cochrane Library (2016, Issue 4), MEDLINE Ovid (1946 to 5 January 2017), Embase Ovid (1947 to 5 January 2017), CINAHL (January 1937 to 5 January 2017), Web of Science Conference Proceedings Citation Index-Science (CPCI-S) (January 1990 to 5 January 2017), BIOSIS Previews (January 1969 to 5 January 2017), MEDION (to 18 August 2014), the Aggressive Research Intelligence Facility database (ARIF) (to 5 January 2017), the ISRCTN registry (www.isrctn.com/editAdvancedSearch); searched 5 January 2017, ClinicalTrials.gov (www.clinicaltrials.gov); searched 5 January 2017 and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en); searched 5 January 2017. We did not use any date or language restrictions in the electronic searches for trials. In addition, orthoptic journals and conference proceedings without electronic listings were searched.
SELECTION CRITERIA
All prospective or retrospective population-based test accuracy studies of consecutive participants were included. Studies compared a single or combination of index tests with the reference test. Only those studies with sufficient data for analysis were included specifically to calculate sensitivity and specificity and determine diagnostic accuracy.Participants were aged 1 to 6 years. Studies reporting participants outside this range were included if subgroup data were available.Permitted settings included population-based vision screening programmes or opportunistic screening programmes, such as those performed in schools.
DATA COLLECTION AND ANALYSIS
We used standard methodological procedures expected by Cochrane. In brief, two review authors independently assessed titles and abstracts for eligibility and extracted the data, with a third senior author resolving any disagreement. We analysed data primarily for specificity and sensitivity.
MAIN RESULTS
One study from a total of 1236 papers, abstracts and trials was eligible for inclusion with a total number of participants of 335 of which 271 completed both the screening test and the gold standard test. The screening test using an automated photoscreener had a sensitivity of 0.46 (95% confidence interval (CI) 0.19 to 0.75) and specificity of 0.97 (CI 0.94 to 0.99). The overall number affected by strabismus was low at 13 (4.8%).
AUTHORS' CONCLUSIONS
There is very limited data in the literature to ascertain the accuracy of tests for detecting strabismus in the community as performed by non-expert screeners. A large prospective study to compare methods would be required to determine which tests have the greatest accuracy.
Topics: Blinking; Child; Child, Preschool; Depth Perception; Fundus Oculi; Humans; Infant; Prospective Studies; Retrospective Studies; Sensitivity and Specificity; Strabismus; Vision Screening; Visual Acuity
PubMed: 29105728
DOI: 10.1002/14651858.CD011221.pub2 -
Scientific Reports May 2018Congenital ptosis may be associated with abnormalities of visual development and function, including amblyopia, strabismus and refractive errors. However, the prevalence... (Meta-Analysis)
Meta-Analysis
Congenital ptosis may be associated with abnormalities of visual development and function, including amblyopia, strabismus and refractive errors. However, the prevalence estimates of these abnormalities vary widely. We performed a systematic review and meta-analysis to estimate the prevalence of amblyopia, strabismus and refractive errors in congenital ptosis. Cochrane, Pubmed, Medline, Embase, and Web of Science were searched by July 2017. We used random/fixed effects models based on a proportion approach to estimate the prevalence. Heterogeneity would be considered signifcant if the p values less than 0.1 and/or I greater than 50%. Subgroup analyses, meta-regression analyses and sensitivity analyses were utilized to explore the potential sources of it. A total of 24 studies selected from 3,633 references were included. The highest prevalence was revealed for myopia with 30.2% (95%CI 3.0-69.8%), followed by 22.7% (95%CI 18.5-27.8%) for amblyopia, 22.2% (95%CI 7.8-63.1%) for astigmatism, 19.6% (95%CI 16.5-23.2%) for strabismus, 17.3% (95% CI 13.1-22.9%) for anisometropia and 4.0% for hyperopia (95%CI 1.8-7.1%). Significant heterogeneity was identified across most estimates. Our findings suggest that amblyopia, strabismus and refractive errors in congenital ptosis are present in much higher percentage. This study highlights the importance of early diagnosis and timely treatment of patients with congenital ptosis.
Topics: Amblyopia; Anisometropia; Astigmatism; Blepharoptosis; Humans; Hyperopia; Myopia; Prevalence; Refractive Errors; Strabismus; Visual Acuity
PubMed: 29844360
DOI: 10.1038/s41598-018-26671-3