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Journal of Neurology Jan 2023The degree of involvement of the autonomic nervous system in progressive supranuclear palsy (PSP) has been investigated in several studies, often providing conflicting... (Review)
Review
BACKGROUND
The degree of involvement of the autonomic nervous system in progressive supranuclear palsy (PSP) has been investigated in several studies, often providing conflicting results. There is a need for a better characterization of autonomic dysfunction in PSP, to enhance our understanding of this highly disabling neurodegenerative disease including patients' needs and possibly be of value for clinicians in the differential diagnosis among Parkinsonian syndromes.
METHODS
We applied a systematic methodology to review existing literature on Pubmed regarding autonomic nervous system involvement in PSP.
RESULTS
PSP reported quite frequently symptoms suggestive of autonomic dysfunction in all domains. Cardiovascular autonomic testing showed in some cases a certain degree of impairment (never severe). There was some evidence suggesting bladder dysfunction particularly in the storage phase. Dysphagia and constipation were the most common gastrointestinal symptoms. Instrumental tests seemed to confirm sudomotor and pupillomotor disturbances.
CONCLUSIONS
PSP patients frequently reported visceral symptoms, however objective testing showed that not always these reflected actual autonomic impairment. Further studies are needed to better delineate autonomic profile and its prognostic role in PSP.
Topics: Humans; Supranuclear Palsy, Progressive; Neurodegenerative Diseases; Primary Dysautonomias; Parkinsonian Disorders; Autonomic Nervous System
PubMed: 36042018
DOI: 10.1007/s00415-022-11347-w -
Journal of Optometry 2020
Topics: Evidence-Based Practice; Humans; Ocular Motility Disorders; Optometry
PubMed: 32553271
DOI: 10.1016/j.optom.2020.06.001 -
The Turkish Journal of Pediatrics 2022Pediatricians are regularly involved in the initial examination of children presenting with strabismus, a common ocular condition occurring in 3% of children. The... (Review)
Review
BACKGROUND
Pediatricians are regularly involved in the initial examination of children presenting with strabismus, a common ocular condition occurring in 3% of children. The objective of this review was to gain insight into pediatric residents, fellows and attendings` understanding of strabismus, and to propose an etiology-based strabismus classification scheme to aid this understanding.
METHODS
A survey was conducted in a single Department of Pediatrics in a university academic institution in order to assess the degree of understanding of the classification, etiology and nomenclature of strabismus. A targeted literature review, pertinent to our classification scheme for strabismus in the pediatric age group, is provided to clarify the various underlying etiological conditions for pediatricians.
RESULTS
The surveyed cohort (n=26) consisted of 10 (38.5%) attendings and 16 (61.5%) pediatricians-in-training. Although 69% of survey participants felt comfortable performing an ocular motility evaluation, only 19% had a clear understanding of the underlying etiology of strabismus, 8% had a clear understanding of strabismus nomenclature and none of the participants had clear knowledge of a classification scheme of strabismus. We propose an etiologic-based strabismus classification scheme with streamlined nomenclature geared towards Pediatricians to facilitate the management of pediatric patients with various ocular misalignments. Eight major categories of this classification scheme include (1) physiologic, (2) comitant, (3) paralytic, (4) sensory, (5) syndromic, (6) orbital, (7) supranuclear and (8) pseudostrabismus.
CONCLUSIONS
Pediatricians at all levels of professional experience have a limited command of strabismus. An etiology-based classification scheme of strabismus may assist in understanding the underlying causes and facilitate the management of strabismus in the pediatrician`s office.
Topics: Child; Cohort Studies; Humans; Pediatricians; Strabismus; Surveys and Questionnaires
PubMed: 35611422
DOI: 10.24953/turkjped.2020.2372 -
Cell Reports Aug 2023Neuroblastoma is a lethal childhood solid tumor of developing peripheral nerves. Two percent of children with neuroblastoma develop opsoclonus myoclonus ataxia syndrome...
Neuroblastoma is a lethal childhood solid tumor of developing peripheral nerves. Two percent of children with neuroblastoma develop opsoclonus myoclonus ataxia syndrome (OMAS), a paraneoplastic disease characterized by cerebellar and brainstem-directed autoimmunity but typically with outstanding cancer-related outcomes. We compared tumor transcriptomes and tumor-infiltrating T and B cell repertoires from 38 OMAS subjects with neuroblastoma to 26 non-OMAS-associated neuroblastomas. We found greater B and T cell infiltration in OMAS-associated tumors compared to controls and showed that both were polyclonal expansions. Tertiary lymphoid structures (TLSs) were enriched in OMAS-associated tumors. We identified significant enrichment of the major histocompatibility complex (MHC) class II allele HLA-DOB01:01 in OMAS patients. OMAS severity scores were associated with the expression of several candidate autoimmune genes. We propose a model in which polyclonal auto-reactive B lymphocytes act as antigen-presenting cells and drive TLS formation, thereby supporting both sustained polyclonal T cell-mediated anti-tumor immunity and paraneoplastic OMAS neuropathology.
Topics: Child; Humans; Autoimmunity; Neuroblastoma; Opsoclonus-Myoclonus Syndrome; Autoantibodies; Genes, MHC Class II; Ataxia
PubMed: 37537844
DOI: 10.1016/j.celrep.2023.112879 -
Journal of Clinical Neurophysiology :... Nov 2019The cerebellum works as a network hub for optimizing eye movements through its mutual connections with the brainstem and beyond. Here, we review three key areas in the... (Review)
Review
The cerebellum works as a network hub for optimizing eye movements through its mutual connections with the brainstem and beyond. Here, we review three key areas in the cerebellum that are related to the control of eye movements: (1) the flocculus/paraflocculus (tonsil) complex, primarily for high-frequency, transient vestibular responses, and also for smooth pursuit maintenance and steady gaze holding; (2) the nodulus/ventral uvula, primarily for low-frequency, sustained vestibular responses; and (3) the dorsal vermis/posterior fastigial nucleus, primarily for the accuracy of saccades. Although there is no absolute compartmentalization of function within the three major ocular motor areas in the cerebellum, the structural-functional approach provides a framework for assessing ocular motor performance in patients with disease that involves the cerebellum or the brainstem.
Topics: Animals; Cerebellum; Eye Movements; Humans; Ocular Motility Disorders
PubMed: 31688323
DOI: 10.1097/WNP.0000000000000579 -
Journal of Binocular Vision and Ocular... 2022Heavy eye syndrome is a condition that results in an acquired, progressive strabismus that is due to extreme myopia and long axial length. The underlying pathogenesis of... (Review)
Review
Heavy eye syndrome is a condition that results in an acquired, progressive strabismus that is due to extreme myopia and long axial length. The underlying pathogenesis of the condition is due to prolapse of the supero-temporal aspect of the myopic globe causing displacement of the lateral and superior rectus muscles inferiorly and nasally, respectively. Treatments for heavy eye syndrome often target this anatomical defect and seek to re-place the globe within the extraocular muscle cone. This review will discuss current theories of pathogenesis and treatment of heavy eye syndrome.
Topics: Adult; Humans; Esotropia; Diplopia; Magnetic Resonance Imaging; Oculomotor Muscles; Strabismus; Ocular Motility Disorders; Syndrome
PubMed: 36279483
DOI: No ID Found -
Ugeskrift For Laeger Sep 2022This is a case report of a 48-year-old female with no medical conditions, with a one-day history of diplopia, mild headache, vertigo and generalized paraesthesia. The...
This is a case report of a 48-year-old female with no medical conditions, with a one-day history of diplopia, mild headache, vertigo and generalized paraesthesia. The neurological examination revealed ophthalmoplegia, ataxia and areflexia leading to a diagnosis of Miller Fisher syndrome, a rare variant of Guillain-Barré syndrome. This highlights the importance of considering the rarer variants of Guillain-Barré syndrome in the differential diagnosis of patients who present with this triad.
Topics: Diagnosis, Differential; Female; Guillain-Barre Syndrome; Headache; Humans; Middle Aged; Miller Fisher Syndrome; Ophthalmoplegia
PubMed: 36205161
DOI: No ID Found -
Tidsskrift For Den Norske Laegeforening... Aug 2019Multiple sclerosis can give rise to signs and symptoms from the entire nervous system, including visual impairments. Visual impairments often go unreported because they... (Review)
Review
Multiple sclerosis can give rise to signs and symptoms from the entire nervous system, including visual impairments. Visual impairments often go unreported because they are not obvious to patients, which means that doctors must ask about them specifically. Regular monitoring of vision is important, however, to provide personalised rehabilitation and assistive technologies, and thereby improve patients’ functioning and quality of life.
Topics: Cranial Nerve Diseases; Humans; Multiple Sclerosis; Nystagmus, Pathologic; Ocular Motility Disorders; Optic Neuritis; Quality of Life; Vision Disorders
PubMed: 31429247
DOI: 10.4045/tidsskr.18.0786 -
The Cochrane Database of Systematic... Jan 2023Infantile esotropia (IE) is the inward deviation of the eye. Various aspects of the clinical management of IE are unclear; mainly, the most effective type of... (Review)
Review
BACKGROUND
Infantile esotropia (IE) is the inward deviation of the eye. Various aspects of the clinical management of IE are unclear; mainly, the most effective type of intervention and the age at intervention.
OBJECTIVES
To examine the effectiveness and optimal timing of surgical and non-surgical treatment options for IE to improve ocular alignment and achieve or allow the development of binocular single vision.
SEARCH METHODS
We searched CENTRAL, MEDLINE, Embase, one other database, and three trials registers (November 2021). We did not use any date or language restrictions in the electronic searches for trials. SELECTION CRITERIA: We included randomized trials and quasi-randomized trials comparing any surgical or non-surgical intervention for IE.
DATA COLLECTION AND ANALYSIS
We used standard Cochrane methodology and graded the certainty of the body of evidence for six outcomes using the GRADE classification.
MAIN RESULTS
We included two studies with 234 children with IE. The first study enrolled 110 children (mean age 26.9 ± 14.5 months) with an onset of esotropia before six months of age, and large-angle IE defined as esotropia of ≥ 40 prism diopters. It was conducted between 2015 and 2018 in a tertiary care hospital in South Africa. It compared a maximum of three botulinum toxin injections with surgical intervention of bimedial rectus muscle recession, and children were followed for six months. There were limitations in study design and implementation; the risk of bias was high, or we had some concerns for most domains. Surgery may increase the incidence of treatment success, defined as orthophoria or residual esotropia of ≤ 10 prism diopters, compared with botulinum toxin injections, but the evidence was very uncertain (risk ratio (RR) of treatment success 1.88, 95% confidence interval (CI) 1.27 to 2.77; 1 study, 101 participants; very low-certainty evidence). The results should be read with caution because 23 children with > 60 prism diopters at baseline in the surgery arm also received botulinum toxin at the time of surgery to augment the recessions. There was no evidence of an important difference between surgery and botulinum toxin injections for over-correction (> 10 prism diopters) of deviation (RR 0.29, 95% CI 0.06 to 1.37; 1 study, 101 participants; very low-certainty evidence), or additional interventions required (RR 0.66, 95% CI 0.36 to 1.19; 1 study, 101 participants; very low-certainty evidence). No major complications of surgery were observed in the surgery arm, while children experienced various complications in the botulinum toxin arm, including partial transient ptosis in 9 (16.7%) children, transient vertical deviation in 3 (5.6%) children, and consecutive exotropia in 13 (24.1%) children. No other outcome data for our prespecified outcomes were reported. The second study enrolled 124 children with onset of esotropia before one year of age in 12 university hospitals in Germany and the Netherlands. It compared bilateral recession with unilateral recession surgeries, and followed children for three months postoperatively. Very low-certainty evidence suggested that there was no evidence of an important difference between bilateral and unilateral surgeries in the presence of binocular vision (numbers with event unclear, P = 0.35), and over-correction (RR of having exotropia 1.09, 95% CI 0.45 to 2.63; 1 study, 118 participants). Dissociated vertical deviation, latent nystagmus, or both were observed in 8% to 21% of participants.
AUTHORS' CONCLUSIONS
Medial rectus recessions may increase the incidence of treatment success compared with botulinum toxin injections alone, but the evidence was very uncertain. No evidence of important difference was found between bilateral surgery and unilateral surgery. Due to insufficient evidence, it was not possible to resolve the controversies regarding type of surgery, non-surgical intervention, or age of intervention in this review. There is clearly a need to conduct good quality trials in these areas to improve the evidence base for the management of IE.
Topics: Child, Preschool; Humans; Infant; Botulinum Toxins; Esotropia; Exotropia; Strabismus; Treatment Outcome; Ophthalmologic Surgical Procedures
PubMed: 36645238
DOI: 10.1002/14651858.CD004917.pub4 -
The Cochrane Database of Systematic... Mar 2023The use of botulinum toxin as an investigative and treatment modality for strabismus is well reported in the medical literature. However, it is unclear how effective it... (Review)
Review
BACKGROUND
The use of botulinum toxin as an investigative and treatment modality for strabismus is well reported in the medical literature. However, it is unclear how effective it is in comparison with other treatment options for strabismus.
OBJECTIVES
The primary objective was to examine the efficacy of botulinum toxin therapy in the treatment of strabismus compared with alternative conservative or surgical treatment options. This review sought to ascertain those types of strabismus that particularly benefit from the use of botulinum toxin as a treatment option (such as small angle strabismus or strabismus with binocular potential, i.e. the potential to use both eyes together as a pair). The secondary objectives were to investigate the dose effect and complication rates associated with botulinum toxin.
SEARCH METHODS
We searched CENTRAL, MEDLINE, Embase, LILACS and three trials registers on 6 July 2022, together with reference checking to identify additional studies. We did not use any date or language restrictions in the electronic searches for trials.
SELECTION CRITERIA
We planned to include randomized controlled trials (RCTs) comparing botulinum toxin with strabismus surgery, botulinum toxin alternatives (i.e. bupivacaine) and conservative therapy such as orthoptic exercises, prisms, or lens therapy for people of any age with strabismus. All relevant RCTs identified in this update compared botulinum toxin with strabismus surgery.
DATA COLLECTION AND ANALYSIS
We used standard methods expected by Cochrane and assessed the certainty of the body of evidence using GRADE.
MAIN RESULTS
We included four RCTs with 242 participants that enrolled adults with esotropia or exotropia, children with acquired esotropia, and children with infantile esotropia. The follow-up period ranged from six to 36 months. Two studies were conducted in Spain, and one each in Canada and South Africa. We judged the included studies to have a mixture of low, unclear and high risk of bias. We did not consider any of the included studies to be at low risk of bias for all domains. All four studies reported the proportion of participants who improved or corrected strabismus, defined as ≤ 10 prism diopters (PD) at six months (two studies) or ≤ 8 PD at one year (two studies). Low-certainty evidence suggested that participants treated with the surgery may be more likely to improve or correct strabismus compared with those who treated with botulinum toxin (risk ratio (RR) 0.72, 95% confidence interval (CI) 0.53 to 0.99; I² = 50%; 4 studies, 242 participants; low-certainty evidence). One study, which enrolled 110 children with infantile esotropia, suggested that surgery may reduce the incidence of additional surgical intervention required, but the evidence was very uncertain (RR 3.05, 95% CI 1.34 to 6.91; 1 study, 101 participants; very low-certainty evidence). Two studies conducted in Spain compared botulinum toxin with surgery in children who required retreatment for acquired or infantile esotropia. These two studies provided low-certainty evidence that botulinum toxin may have little to no effect on achieving sensory fusion (RR 0.88, 95% CI 0.63 to 1.23; I² = 0%; 2 studies, 102 participants) and stereopsis (RR 0.86, 95% CI 0.59 to 1.25; I² = 0%; 2 studies, 102 participants) compared with surgery. Three studies reported non-serious adverse events. Partial transient ptosis (range 16.7% to 37.0%) and transient vertical deviation (range 5.6% to 18.5%) were observed among participants treated with botulinum toxin in three studies. In one study, 44.7% participants in the surgery group experienced discomfort. No studies reported serious adverse events or postintervention quality of life.
AUTHORS' CONCLUSIONS
It remains unclear whether botulinum toxin may be an alternative to strabismus surgery as an independent treatment modality among certain types of strabismus because we found only low and very low-certainty evidence in this review update. Low-certainty evidence suggests that strabismus surgery may be preferable to botulinum toxin injection to improve or correct strabismus when types of strabismus and different age groups are combined. We found low-certainty evidence suggesting botulinum toxin may have little to no effect on achievement of binocular single vision compared with surgery in children with acquired or infantile esotropia. We did not find sufficient evidence to draw any meaningful conclusions with respect to need for additional surgery, quality of life, and serious adverse events. We identified three ongoing trials comparing botulinum toxin with conventional surgeries in the varying types of strabismus, whose results will provide relevant evidence for our stated objectives. Future trials should be rigorously designed, and investigators should analyze outcome data appropriately and report adequate information to provide evidence of high certainty. Quality of life and cost-effectiveness should be examined in addition to clinical and safety outcomes.
Topics: Adult; Child; Humans; Botulinum Toxins; Esotropia; Strabismus; Canada
PubMed: 36916692
DOI: 10.1002/14651858.CD006499.pub5