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Cerebellum (London, England) Jun 2024Oculomotor deficits are common in hereditary ataxia, but disproportionally neglected in clinical ataxia scales and as outcome measures for interventional trials....
Oculomotor deficits are common in hereditary ataxia, but disproportionally neglected in clinical ataxia scales and as outcome measures for interventional trials. Quantitative assessment of oculomotor function has become increasingly available and thus applicable in multicenter trials and offers the opportunity to capture severity and progression of oculomotor impairment in a sensitive and reliable manner. In this consensus paper of the Ataxia Global Initiative Working Group On Digital Oculomotor Biomarkers, based on a systematic literature review, we propose harmonized methodology and measurement parameters for the quantitative assessment of oculomotor function in natural-history studies and clinical trials in hereditary ataxia. MEDLINE was searched for articles reporting on oculomotor/vestibular properties in ataxia patients and a study-tailored quality-assessment was performed. One-hundred-and-seventeen articles reporting on subjects with genetically confirmed (n=1134) or suspected hereditary ataxia (n=198), and degenerative ataxias with sporadic presentation (n=480) were included and subject to data extraction. Based on robust discrimination from controls, correlation with disease-severity, sensitivity to change, and feasibility in international multicenter settings as prerequisite for clinical trials, we prioritize a core-set of five eye-movement types: (i) pursuit eye movements, (ii) saccadic eye movements, (iii) fixation, (iv) eccentric gaze holding, and (v) rotational vestibulo-ocular reflex. We provide detailed guidelines for their acquisition, and recommendations on the quantitative parameters to extract. Limitations include low study quality, heterogeneity in patient populations, and lack of longitudinal studies. Standardization of quantitative oculomotor assessments will facilitate their implementation, interpretation, and validation in clinical trials, and ultimately advance our understanding of the evolution of oculomotor network dysfunction in hereditary ataxias.
Topics: Humans; Consensus; Ocular Motility Disorders; Eye Movements; Biomarkers
PubMed: 37117990
DOI: 10.1007/s12311-023-01559-9 -
International Journal of Environmental... Jul 2020Facial palsy is often associated with impaired facial function and altered appearance. However, the literature with regards to the psychological adjustment of children...
INTRODUCTION
Facial palsy is often associated with impaired facial function and altered appearance. However, the literature with regards to the psychological adjustment of children and adolescents with facial palsy has not been systematically reviewed to date. This paper aimed to review all published research with regards to psychosocial adjustment for children and adolescents with facial palsy.
METHODS
MEDLINE, CINAHL, Embase, PsychInfo and AMED databases were searched and data was extracted with regards to participant characteristics, study methodology, outcome measures used, psychosocial adjustment and study quality.
RESULTS
Five studies were eligible for inclusion, all of which investigated psychosocial adjustment in participants with Moebius syndrome, a form of congenital facial palsy. Many parents reported their children to have greater social difficulties than general population norms, with difficulties potentially increasing with age. Other areas of psychosocial adjustment, including behaviour, anxiety and depression, were found to be more comparable to the general population.
DISCUSSION
Children and adolescents with Moebius syndrome may experience social difficulties. However, they also demonstrate areas of resilience. Further research including individuals with facial palsy of other aetiologies is required in order to determine the psychosocial adjustment of children and adolescents with facial palsy.
Topics: Adolescent; Anxiety; Anxiety Disorders; Child; Cross-Sectional Studies; Emotional Adjustment; Facial Paralysis; Female; Humans; Male; Mobius Syndrome
PubMed: 32751746
DOI: 10.3390/ijerph17155528 -
Journal of Sport Rehabilitation Nov 2020Convergence dysfunction following concussion is common. Near point of convergence (NPC) is a quick and easy assessment that may detect oculomotor dysfunction such as...
CONTEXT
Convergence dysfunction following concussion is common. Near point of convergence (NPC) is a quick and easy assessment that may detect oculomotor dysfunction such as convergence insufficiency (CI), but NPC measurements are rarely reported. Convergence dysfunction is treatable in otherwise healthy patients; the effectiveness of oculomotor therapy following concussion is unclear.
OBJECTIVES
The purpose of this article was to systematically review the literature and answer the following clinical questions: (1) Is performance on NPC negatively affected in patients diagnosed with a concussion compared with pre-injury levels or healthy controls? (2) In patients diagnosed with concussion, what is the effect of oculomotor/vision therapy on NPC break measurements?
EVIDENCE ACQUISITION
The search was conducted in CINAHL, SPORTDiscus, MEDLINE, and PubMed using terms related to concussion, mild traumatic brain injury, convergence, vision, and rehabilitation. Literature considered for review included original research publications that collected measures of NPC break in concussion patients, with a pretest-posttest comparison or comparison with a healthy control group. A literature review was completed; 242 relevant articles were reviewed, with 18 articles meeting criteria for inclusion in the review.
EVIDENCE SYNTHESIS
Articles were categorized according to the clinical question they addressed. The patient or participant sample (number, sex, age, and health status), study design, instrumentation, or intervention used, and main results were extracted from each article.
CONCLUSIONS
The authors' main findings suggest that there is a moderate level of evidence that patients have impaired NPC up to several months postconcussion, and a low level of evidence that impairments can be successfully treated with oculomotor therapy. These findings should be cautiously evaluated; the studies are limited by weak/moderate quality, small sample sizes, varied methodology, and nonrandomized treatment groups. Future research should explore factors affecting convergence postconcussion and include randomized, controlled studies to determine if performing vision therapy improves visual measures and promotes recovery.
Topics: Brain Concussion; Humans; Ocular Motility Disorders; Physical Therapy Modalities
PubMed: 32131046
DOI: 10.1123/jsr.2019-0428 -
BMC Neurology Nov 2019Progressive Supranuclear Palsy (PSP) is the most frequent form of atypical Parkinsonism. Although there is preliminary evidence for the benefits of gait rehabilitation,...
BACKGROUND
Progressive Supranuclear Palsy (PSP) is the most frequent form of atypical Parkinsonism. Although there is preliminary evidence for the benefits of gait rehabilitation, balance training and oculomotor exercises in PSP, the quality of reporting of exercise therapies appears mixed. The current investigation aims to evaluate the comprehensiveness of reporting of exercise and physical activity interventions in the PSP literature.
METHODS
Two independent reviewers used the Consensus on Exercise Reporting Template (CERT) to extract all exercise intervention data from 11 studies included in a systematic review. CERT items covered: 'what' (materials), 'who' (instructor qualifications), 'how' (delivery), 'where' (location), 'when', 'how much' (dosage), 'tailoring' (what, how), and 'how well' (fidelity) exercise delivery complied with the protocol. Each exercise item was scored '1' (adequately reported) or '0' (not adequately reported or unclear). The CERT score was calculated, as well as the percentage of studies that reported each CERT item.
RESULTS
The CERT scores ranged from 3 to 12 out of 19. No PSP studies adequately described exercise elements that would allow exact replication of the interventions. Well-described items included exercise equipment, exercise settings, exercise therapy scheduling, frequency and duration. Poorly described items included decision rules for exercise progression, instructor qualifications, exercise adherence, motivation strategies, safety and adverse events associated with exercise therapies.
DISCUSSION
The results revealed variability in the reporting of physical therapies for people living with PSP. Future exercise trials need to more comprehensively describe equipment, instructor qualifications, exercise and physical activity type, dosage, setting, individual tailoring of exercises, supervision, adherence, motivation strategies, progression decisions, safety and adverse events.
CONCLUSION
Although beneficial for people living with PSP, exercise and physical therapy interventions have been inadequately reported. It is recommended that evidence-based reporting templates be utilised to comprehensively document therapeutic exercise design, delivery and evaluation.
Topics: Consensus; Exercise Therapy; Gait; Humans; Motivation; Physical Therapy Modalities; Supranuclear Palsy, Progressive
PubMed: 31783740
DOI: 10.1186/s12883-019-1539-4 -
International Journal of Environmental... Sep 2020The Coronavirus Disease 2019 (COVID-19) outbreak has shocked the whole world with its unexpected rapid spread. The virus responsible for the disease, the Severe Acute...
The Coronavirus Disease 2019 (COVID-19) outbreak has shocked the whole world with its unexpected rapid spread. The virus responsible for the disease, the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), enters host cells by means of the envelope spike protein, which binds to angiotensin-converting enzyme 2 receptors. These receptors are highly expressed in heart, lungs, respiratory tract epithelium, endothelial cells and brain. Since an increasing body of significant evidence is highlighting a possible neuroinvasion related to SARS-CoV-2, a state of the art on the neurological complications is needed. To identify suitable publications, our systematic review was carried out by searching relevant studies on PubMed and Scopus databases. We included studies investigating neurologic manifestations of SARS-CoV-2 in patients over 18. According to the analyzed studies, the most frequent disorders affecting central nervous system (CNS) seem to be the following: olfactory and taste disorders, ischemic/hemorrhagic stroke, meningoencephalitis and encephalopathy, including acute necrotizing encephalopathy, a rare type of encephalopathy. As regards the peripheral nervous system (PNS), Guillain-Barré and Miller Fisher syndromes are the most frequent manifestations reported in the literature. Important clinical information on the neurological manifestations of SARS-CoV-2 would help clinicians raise awareness and simultaneously improve the prognosis of critically ill patients.
Topics: Betacoronavirus; COVID-19; Central Nervous System Diseases; Coronavirus Infections; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome; Pandemics; Pneumonia, Viral; SARS-CoV-2
PubMed: 32937949
DOI: 10.3390/ijerph17186688 -
Medicine Dec 2022Covid-19 has serious sequelae that may be poorly understood, underreported, and, as a result, not diagnosed promptly, such as variations in clinical manifestations of...
BACKGROUND
Covid-19 has serious sequelae that may be poorly understood, underreported, and, as a result, not diagnosed promptly, such as variations in clinical manifestations of hyperinflammation among people infected with SARS-CoV-2. ophthalmoplegia can be one of these manifestations.
METHODS
We are reporting a 55-year-old male patient with unilateral diplopia considering it as a case of multisystem inflammatory syndrome in adults. We also reviewed the literature systematically for the previously reported studies/cases with third, fourth and sixth cranial nerve palsies due to or after Covid-19.
RESULTS
The literature search yielded 17 studies reporting 29 patients. 71.4% of the patients were males with a mean age of 42.23 years. Ophthalmological symptoms took 9.7 days to appear after the respiratory involvement. All patients had diplopia as part of their visual symptoms. 41.4% of the patients had unilateral sixth nerve palsy, 24% had bilateral sixth nerve involvement, 17% had fourth nerve involvement, and 27.6% had third nerve involvement.
CONCLUSION
Ophthalmoplegia is considered presenting symptom of Covid-19. Further research is needed to detect all neuro-ophthalmological manifestations of Covid-19.
Topics: Adult; Female; Humans; Male; Middle Aged; Abducens Nerve Diseases; COVID-19; Cranial Nerve Diseases; Diplopia; Oculomotor Nerve Diseases; Ophthalmoplegia; SARS-CoV-2
PubMed: 36626529
DOI: 10.1097/MD.0000000000032023 -
Indian Journal of Ophthalmology Nov 2019Multiple transposition procedures have been described for management of lateral rectus palsy. However, relative effect and indications of each procedure are unclear....
Multiple transposition procedures have been described for management of lateral rectus palsy. However, relative effect and indications of each procedure are unclear. This systematic review was planned to evaluate functional and anatomical outcomes of vertical rectus transposition (VRT) surgery in patients with lateral rectus palsy. We searched databases in English language, namely, MEDLINE, PubMed Central, EMBASE, Google Scholar, Scopus, and Index Copernicus without any date restrictions in electronic searches, using the search words 'vertical rectus transposition for lateral rectus palsy," "vertical rectus transposition for abducens palsy," "superior rectus transposition," "inferior rectus transposition," and "Hummelsheim procedure." References of the selected publications were also searched to find any relevant studies. We searched for studies that provided data on single VRT and double VRT surgeries for lateral rectus palsies. Three authors independently assessed the related studies gathered from electronic and manual searches. We found 27 studies which were relevant to the review question. As there were no randomized control trials (RCTs) available related to our study question, nonrandomized studies were used to arrive at summarization of outcomes of different transposition procedures. There is a need for prospective RCTs to investigate the different types of transposition procedures for lateral rectus palsy.
Topics: Abducens Nerve Diseases; Esotropia; Eye Movements; Humans; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Vision, Binocular
PubMed: 31638036
DOI: 10.4103/ijo.IJO_1841_18 -
Medicine May 2021Intravenous dexmedetomidine (DEX) has been used to prevent emergence agitation (EA) in children. The aim of this meta-analysis was to evaluate whether DEX decreases EA... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Intravenous dexmedetomidine (DEX) has been used to prevent emergence agitation (EA) in children. The aim of this meta-analysis was to evaluate whether DEX decreases EA incidence without augmenting oculocardiac reflex (OCR) in pediatric patients undergoing strabismus surgery.
METHODS
We searched PubMed, EMBASE, Chinese National Knowledge Infrastructure (CNKI), Wan Fang, and the Cochrane Library to collect the randomized controlled trials (RCTs) investigating the effects of intraoperative DEX in children undergoing strabismus surgery from inception to October 2019. Postoperative Pediatric Agitation and Emergence Delirium (PAED) score, postoperative EA, extubation or laryngeal mask airway (LMA) removal time, postanesthetic care unit (PACU) stay time, OCR, and postoperative vomiting (POV) were evaluated.
RESULTS
11 RCTs including 801 patients were included in this study. Compared with control group, intravenous DEX significantly reduced postoperative PAED score (WMD, 3.05; 95% CI: -3.82 to -2.27, P = .017) and incidences of postoperative EA 69% (RR, 0.31; 95% CI: 0.17 to 0.55, P < .00) and POV (RR, 0.28; 95% CI: 0.13 to 0.61, P = .001). Furthermore, the use of DEX significantly delayed extubation or LMA removal time (WMD, 2.11; 95% CI: 0.25 to 3.97, P < .001). No significant difference was found in the incidence of ORC and PACU stay time.
CONCLUSION
Intravenous DEX reduced the incidences of EA without increasing OCR in pediatric patients undergoing strabismus surgery. Meanwhile, DEX infusion decreased the incidence of POV in children.
Topics: Anesthesia, General; Dexmedetomidine; Emergence Delirium; Humans; Incidence; Injections, Intravenous; Ophthalmologic Surgical Procedures; Perioperative Period; Randomized Controlled Trials as Topic; Reflex, Oculocardiac; Strabismus; Treatment Outcome
PubMed: 33950954
DOI: 10.1097/MD.0000000000025717 -
International Journal of Environmental... Jun 2020Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare, inherited disorder characterized by a congenital absence of conjugate horizontal eye movements with...
Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare, inherited disorder characterized by a congenital absence of conjugate horizontal eye movements with progressive scoliosis developing in childhood and adolescence. Mutations in the Roundabout (3) gene located on chromosome 11q23-25 are responsible for the development of horizontal gaze palsy and progressive scoliosis. However, some studies redefined the locus responsible for this pathology to a 9-cM region. This study carried out a systematic review in which 25 documents were analyzed, following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standards. The search was made in the following electronic databases from January 1995 to October 2019: PubMed, Scopus, Web of Science, PEDRO, SPORT Discus, and CINAHL. HGPPS requires a multidisciplinary diagnostic approach, in which magnetic resonance imaging might be the first technique to suggest the diagnosis, which should be verified by an analysis of the 3 gene. This is important to allow for adequate ocular follow up, apply supportive therapies to prevent the rapid progression of scoliosis, and lead to appropriate genetic counseling.
Topics: Adolescent; Child; Female; Humans; Male; Mutation; Ocular Motility Disorders; Ophthalmoplegia, Chronic Progressive External; Receptors, Cell Surface; Receptors, Immunologic; Scoliosis
PubMed: 32580277
DOI: 10.3390/ijerph17124467 -
Academic Emergency Medicine : Official... Sep 2020Dizziness is a common complaint presented in the emergency department (ED). A subset of these patients will present with acute vestibular syndrome (AVS). AVS is a... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Dizziness is a common complaint presented in the emergency department (ED). A subset of these patients will present with acute vestibular syndrome (AVS). AVS is a clinical syndrome defined by the presence of vertigo, nystagmus, head motion intolerance, ataxia, and nausea/vomiting. These symptoms are most often due to benign vestibular neuritis; however, they can be a sign of a dangerous central cause, i.e., vertebrobasilar stroke. The Head Impulse test, Nystagmus, Test of Skew (HINTS) examination has been proposed as a bedside test for frontline clinicians to rule out stroke in those presenting with AVS. Our objective was to assess the diagnostic accuracy of the HINTS examination to rule out a central cause of vertigo in an adult population presenting to the ED with AVS. Our aim was to assess the diagnostic accuracy when performed by emergency physicians versus neurologists.
METHODS
We searched PubMed, Medline, Embase, the Cochrane database, and relevant conference abstracts from 2009 to September 2019 and performed hand searches. No restrictions for language or study type were imposed. Prospective studies with patients presenting with AVS using criterion standard of computed tomography and/or magnetic resonance imaging were selected for review. Two independent reviewers extracted data from relevant studies. Studies were combined if low clinical and statistical heterogeneity was present. Study quality was assessed using the QUADAS-2 tool. Random effects meta-analysis was performed using RevMan 5 and SAS 9.3.
RESULTS
A total of five studies with 617 participants met the inclusion criteria. The mean (±SD) study length was 5.3 (±3.3) years. Prevalence of vertebrobasilar stroke ranged 9.3% to 44% (mean ± SD = 39.1% ± 17.1%). The most common diagnoses were vertebrobasilar stroke (mean ± SD = 34.8% ± 17.1%), peripheral cause (mean ± SD = 30.9% ± 16%), and intracerebral hemorrhage (mean ± SD = 2.2% ± 0.5%). The HINTS examination, when performed by neurologists, had a sensitivity of 96.7% (95% CI = 93.1% to 98.5%, I = 0%) and specificity of 94.8% (95% CI = 91% to 97.1%, I = 0%). When performed by a cohort of physicians including both emergency physicians (board certified) and neurologists (fellowship trained in neurootology or vascular neurology) the sensitivity was 83% (95% CI = 63% to 95%) and specificity was 44% (95% CI = 36% to 51%).
CONCLUSIONS
The HINTS examination, when used in isolation by emergency physicians, has not been shown to be sufficiently accurate to rule out a stroke in those presenting with AVS.
Topics: Adult; Head Impulse Test; Humans; Nystagmus, Pathologic; Physicians; Prospective Studies; Vertigo
PubMed: 32167642
DOI: 10.1111/acem.13960