-
Journal of Integrative Neuroscience Apr 2024The clinical category of immune-mediated cerebellar ataxias (IMCAs) is now recognized after 3 decades of clinical and experimental research. The cerebellum gathers about...
The clinical category of immune-mediated cerebellar ataxias (IMCAs) is now recognized after 3 decades of clinical and experimental research. The cerebellum gathers about 60% of neurons in the brain, is enriched in numerous plasticity mechanisms, and presents a large variety of antigens at the neuroglial level: ion channels and related proteins, synaptic adhesion/organizing proteins, transmitter receptors, and glial cells. Cerebellar circuitry is especially vulnerable to immune attacks. After the loss of immune tolerance, IMCAs present in an acute or subacute manner with various combinations of a vestibulocerebellar syndrome (VCS), a cerebellar motor syndrome (CMS), and a cerebellar cognitive affective syndrome/Schmahmann's syndrome (CCAS/SS). IMCAs include gluten ataxia (GA), post-infectious cerebellitis (PIC), Miller Fisher syndrome (MFS), paraneoplastic cerebellar degeneration (PCD), opsoclonus myoclonus syndrome (OMS), anti-glutamic acid decarboxylase (anti-GAD) ataxia, and glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A). In addition, multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), Behçet disease, and collagen-vascular disorders may also present with cerebellar symptoms when lesions involve cerebellar afferences/efferences. Patients whose clinical profiles do not fit with IMCAs are now gathered in the group of primary autoimmune cerebellar ataxias (PACAs). Latent auto-immune cerebellar ataxia (LACA) refers to a clinical stage with a slow progressive course and a lack of obvious auto-immune background. At a pre-symptomatic stage, patients remain asymptomatic, whereas at the prodromal stage aspecific symptoms occur, announcing the symptomatic neuronal loss. LACA corresponds to a time-window where an intervention could lead to preservation of plasticity mechanisms. Patients may evolve from LACA to PACA and typical IMCAs, highlighting a continuum. Immune ataxias represent a model to elucidate the sequence of events leading to destruction of cerebellar neuronal reserve and develop novel strategies aiming to restore plasticity mechanisms.
Topics: Humans; Ataxia; Autoimmune Diseases of the Nervous System; Cerebellar Ataxia
PubMed: 38682229
DOI: 10.31083/j.jin2304079 -
Journal of Optometry 2024This study aimed to compare the preoperative clinical features of patients with sensory esotropia (ET) and sensory exotropia (XT). (Comparative Study)
Comparative Study
PURPOSE
This study aimed to compare the preoperative clinical features of patients with sensory esotropia (ET) and sensory exotropia (XT).
METHODS
In a retrospective study, the medical records of 13,252 patients who underwent strabismus surgery were reviewed at the Farabi Eye Hospital, Iran, from 2012 to March 2022. There were 1017 patients with sensory horizontal strabismus whose, in their worse eye, had corrected distance visual acuity (CDVA) equal to or <20/160 tested with the Snellen chart.
RESULTS
The mean age of patients was 29.0 ± 12.4 years [574 (56.4%) males and 443 (43.6%) females]. Sensory XT and ET were observed in 717 (70.5%) and 300 (29.5%) patients, respectively (P<.001). The mean CDVA in the strabismic and non-strabismic eyes was 1.40 ± 0.75 and 0.05 ± 0.13, respectively (P<.001). Also, the CDVA in the strabismic eyes was significantly worse in the patients with sensory XT than in the patients with sensory ET (P<.001). Sphere and spherical equivalent (SE) components were more hyperopic in both eyes of patients with sensory ET than sensory XT (P<.001). In sensory ET group, the mean horizontal deviation at far and near was significantly higher than the sensory XT group (both P<.001). The prevalence of moderate and severe amblyopia among all patients with sensory strabismus was 274 (26.9%) and 727 (71.5%), respectively (P<.001). There were 398 (39.1%) patients who needed more than one surgery.
CONCLUSION
The frequency of sensory XT was about 2.5 times more than the sensory ET. Most patients with sensory ET were operated at a younger age, had better CDVA, more hyperopic spherical and SE, and higher angle of deviation compared with patients with sensory XT. The chance of reoperation in patients with sensory strabismus was about 40%.
Topics: Humans; Female; Male; Retrospective Studies; Adult; Esotropia; Visual Acuity; Young Adult; Exotropia; Adolescent; Middle Aged; Child; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Child, Preschool; Iran; Vision, Binocular; Aged
PubMed: 38663271
DOI: 10.1016/j.optom.2024.100516 -
Neurology(R) Neuroimmunology &... May 2024Paraneoplastic neurologic syndromes (PNSs) are remote neurologic immune-related effects of tumors. The clinical characteristics of pediatric PNSs remain unclear. We...
BACKGROUND AND OBJECTIVES
Paraneoplastic neurologic syndromes (PNSs) are remote neurologic immune-related effects of tumors. The clinical characteristics of pediatric PNSs remain unclear. We retrospectively examined the clinical characteristics of cases of pediatric PNSs and assessed the performance of the 2021 diagnostic criteria in children.
METHODS
Patients hospitalized in the Beijing Children's Hospital between June 2015 and June 2023 and fulfilling the description of by 2004 diagnostic criteria of PNSs were included. A retrospective analysis of clinical characteristics was conducted, and the 2021 diagnostic criteria were applied to rediagnostic stratification.
RESULTS
Among the 42 patients included, the most common neurologic syndrome was opsoclonus-myoclonus syndrome (OMS) (62%), followed by rapidly progressive cerebellar syndrome (26%). Most tumors were neuroblastomas (88%), with few being ovarian teratomas (10%). Approximately 71% (30/42) of patients were classified as definite and 24% (10/42) as according to the 2021 criteria. All cases judged as probable exhibited rapidly progressive cerebellar ataxia with neuroblastoma. For OMS, chemotherapy was administered based on the tumor's risk stage, accompanied by regular infusion of IV gamma globulin and oral steroids following tumor diagnosis. Twenty-one patients underwent regular follow-ups over 4.92 (0.58-7.58) years. The initial hospitalization recorded a median score of 12 (7-14) on the Mitchell and Pike OMS rating scale, decreasing to 0 (0-5) at the final follow-up. In cases of rapidly progressive cerebellar syndrome, a similar therapeutic regimen was used. Nine patients underwent regular follow-ups over 4.42 (1.17-7.50) years. The mean modified Rankin scale score at first hospitalization was 4 (3-4), reducing to 1 (0-4) at the final follow-up. Only 17% (5/30) of patients across both groups exhibited poor response to this regimen. Among these 5 patients, 4 belonged to the low-risk group (without chemotherapy).
DISCUSSION
OMS followed by rapidly progressive cerebellar ataxia are the most common forms of PNSs in children and are associated with neuroblastoma. An aggressive approach with multiple immunotherapies may improve the prognosis of neuroblastoma-associated PNSs. The 2021 criteria perform well in pediatric PNSs. However, we propose upgrading the classification of antibody-negative rapidly progressive cerebellar ataxia with neuroblastoma to definite diagnosis. This adjustment aims to further improve the diagnostic efficacy of this diagnostic criterion in childhood.
Topics: Humans; Female; Male; Retrospective Studies; Child, Preschool; Child; Paraneoplastic Syndromes, Nervous System; Infant; Opsoclonus-Myoclonus Syndrome; Adolescent; Neuroblastoma
PubMed: 38657195
DOI: 10.1212/NXI.0000000000200242 -
Investigative Ophthalmology & Visual... Apr 2024Individuals with amblyopia experience central vision deficits, including loss of visual acuity, binocular vision, and stereopsis. In this study, we examine the...
PURPOSE
Individuals with amblyopia experience central vision deficits, including loss of visual acuity, binocular vision, and stereopsis. In this study, we examine the differences in peripheral binocular imbalance in children with anisometropic amblyopia, strabismic amblyopia, and typical binocular vision to determine if there are systematic patterns of deficits across the visual field.
METHODS
This prospective cohort study recruited 12 participants with anisometropic amblyopia, 10 with strabismic amblyopia, and 10 typically sighted controls (age range, 5-18 years). Binocular imbalance was tested at 0°, 4°, and 8° eccentricities (4 angular locations each) using band-pass filtered Auckland optotypes (5 cycles per optotype) dichoptically presented with differing contrast to each eye. The interocular contrast ratio was adjusted until the participant reported each optotype with equal frequency.
RESULTS
Participants with anisometropic and strabismic amblyopia had a more balanced contrast ratio, or decreased binocular imbalance, at 4° and 8° eccentricities as compared with central vision. Participants with strabismic amblyopia had significantly more binocular imbalance in the periphery as compared with individuals with anisometropic amblyopia or controls. A linear mixed effects model showed a main effect for strabismic amblyopia and eccentricity on binocular imbalance across the visual field.
CONCLUSIONS
There is evidence of decreased binocularity deficits, or interocular suppression, in the periphery in anisometropic and strabismic amblyopia as compared with controls. Notably, those with strabismic amblyopia exhibited more significant peripheral binocular imbalance. These variations in binocularity across the visual field among different amblyopia subtypes may necessitate tailored approaches for dichoptic treatment.
Topics: Humans; Amblyopia; Vision, Binocular; Male; Female; Child; Prospective Studies; Adolescent; Strabismus; Visual Acuity; Visual Fields; Child, Preschool; Anisometropia; Depth Perception
PubMed: 38652649
DOI: 10.1167/iovs.65.4.36 -
Indian Journal of Ophthalmology May 2024Mutations of G protein-coupled receptor 143 (GPR143) and FERM domain containing 7 (FRMD7) may result in congenital nystagmus (CN) in the first 6 months of life. We aimed...
PURPOSE
Mutations of G protein-coupled receptor 143 (GPR143) and FERM domain containing 7 (FRMD7) may result in congenital nystagmus (CN) in the first 6 months of life. We aimed to compare the differences in ocular oscillations between patients with these two gene mutations as well as the functional and structural changes in their retinas and visual pathways.
METHODS
Medical records were retrospectively reviewed to identify patients of congenital nystagmus with confirmed mutations in either GPR143 or FMRD7 genes from January 2018 to May 2023. The parameters of the ocular oscillations were recorded using Eyelink 1000 Plus. The retinal structure and function were evaluated using optical coherence tomography and multi-focal electroretinography (mERG). The visual pathway and optical nerve projection were evaluated using visual evoked potentials. The next-generation sequencing technique was used to identify the pathogenic variations in the disease-causing genes for CN.
RESULTS
Twenty nystagmus patients of GPR143 and 21 patients of FMRD7 who had been confirmed by molecular testing between January 2018 and May 2023 were included. Foveal hypoplasia was detected only in patients with the GPR143 pathogenic variant. mERG examination showed a flat response topography in the GPR143 group compared to the FRMD7 group. VEP showed that bilateral amplitude inconsistency was detected only in the patients with GPR143 gene mutation. The amplitude and frequency of the ocular oscillations were not found to differ between patients with two different genetic mutations.
CONCLUSIONS
Although the etiology and molecular mechanisms are completely different between CN patients, they may have similar ocular oscillations. A careful clinical examination and electrophysiological test will be helpful in making a differential diagnosis. Our novel identified variants will further expand the spectrum of the GPR143 and FRMD7 variants.
Topics: Female; Humans; Male; Cytoskeletal Proteins; DNA; DNA Mutational Analysis; Electroretinography; Evoked Potentials, Visual; Eye Movements; Eye Proteins; Membrane Glycoproteins; Membrane Proteins; Mutation; Nystagmus, Congenital; Retina; Retrospective Studies; Tomography, Optical Coherence
PubMed: 38648460
DOI: 10.4103/IJO.IJO_3141_23 -
Indian Journal of Ophthalmology May 2024
Topics: Humans; Strabismus; Ophthalmology; Ophthalmologic Surgical Procedures; Models, Anatomic; Oculomotor Muscles; Education, Medical, Graduate
PubMed: 38648445
DOI: 10.4103/IJO.IJO_2141_23 -
Indian Journal of Ophthalmology May 2024
Topics: Humans; Retinal Ganglion Cells; Tomography, Optical Coherence; Macula Lutea; Oculomotor Muscles; Male; Female; Nerve Fibers; Follow-Up Studies; Ophthalmologic Surgical Procedures; Adult; Strabismus
PubMed: 38648439
DOI: 10.4103/IJO.IJO_2353_23 -
Medicine Apr 2024Isolated ischemic oculomotor nerve palsy as a type of ophthalmic disease is rarely observed in clinical practice. Quality of life is frequently impacted by isolated...
BACKGROUND
Isolated ischemic oculomotor nerve palsy as a type of ophthalmic disease is rarely observed in clinical practice. Quality of life is frequently impacted by isolated ischemic oculomotor nerve palsy due to its lack of treatment options and long-term visual impairment. We describe an acupuncture-treated instance of isolated ischemic oculomotor paralysis.
METHODS
Acupoints including Jingming (BL 1), Chengqi (ST 1), Cuanzhu (BL 2), and Sizhukong (TE 23) on the right side, and bilateral Fengchi (GB 20), Waiguan (TE 5), Hegu (LI 4), and Zulinqi (GB 41) were selected for needling. Each treatment lasted for 30 minutes, once every other day. Acupuncture treatment was administered for a total of 11 times.
RESULTS
Acupuncture is a promising treatment option for isolated ischemic oculomotor nerve palsy.
CONCLUSIONS
Ischemic oculomotor nerve paralysis can affect the quality of life of patients. Acupuncture intervention can promote the recovery of the disease is a very effective treatment measure.
Topics: Humans; Quality of Life; Acupuncture Therapy; Acupuncture Points; Oculomotor Nerve Diseases; Acupuncture
PubMed: 38640311
DOI: 10.1097/MD.0000000000037850 -
BMC Ophthalmology Apr 2024Convergence insufficiency is a common issue in the field of binocular vision. Various treatment options have been suggested for managing this condition, but their...
BACKGROUND
Convergence insufficiency is a common issue in the field of binocular vision. Various treatment options have been suggested for managing this condition, but their efficacy in individuals with presbyopia remains unclear. The objective of this study is to compare the effectiveness of home-based vision therapy and prism prescription, in presbyopic patients with convergence insufficiency.
METHODS/DESIGN
It is a randomized, prospective, double-blind clinical trial, with total of 150 participants randomly assigned to the three groups. The Control Group will receive a new near glasses as a conventional prescription, along with aimless and random eye movement exercises that do not have any convergence or accommodation effects. The Home Vision Therapy Group will receive new near glasses with accommodative and convergence eye exercises. The Prism Group will receive a near prismatic glasses prescribed using the Sheard's criterion. All treatments will be administered for a period of 2 months, and measurements of the modified convergence insufficiency symptoms survey (CISS), near point convergence, near phoria, and positive fusional vergence will be taken at baseline, one month later, and at the end of the treatment.
DISCUSSION
We aim to identify which component - either the prism prescription or the home vision therapy - is more effective in improving binocular abilities and reducing patients' symptom scores.
TRIAL REGISTRATION
ClinicalTrials.gov NCT05311917 with last update on 04/22/2023.
Topics: Humans; Ocular Motility Disorders; Prospective Studies; Strabismus; Eye Movements; Orthoptics; Vision, Binocular; Accommodation, Ocular; Convergence, Ocular; Randomized Controlled Trials as Topic
PubMed: 38622543
DOI: 10.1186/s12886-024-03411-y -
Romanian Journal of Ophthalmology 2024To quantify variation between surgeons in reoperation rates after horizontal strabismus surgery, and to explore associations of reoperation rate with surgical...
Variation between surgeons in rate of reoperation after horizontal strabismus surgery among Medicare beneficiaries: associations with patient and surgeon characteristics and adjustable sutures.
To quantify variation between surgeons in reoperation rates after horizontal strabismus surgery, and to explore associations of reoperation rate with surgical techniques, patient characteristics, and practice type and volume. Fee-for-service payments in a national database to providers for Medicare beneficiaries having strabismus surgery on horizontal muscles between 2012 and 2020 were analyzed retrospectively to identify same calendar year reoperations. Multivariable linear regression was used to determine predictors of each surgeon's reoperation rate. The reoperation rate for 1-horizontal muscle surgery varied between 0.0% and 30.8% among 141 surgeons. Just 7.8% of surgeons contributed over half of the reoperation events for 1-horizontal muscle surgery, due to the presence of high-volume surgeons with high reoperation rates. Surgeon seniority, gender, surgery volume, and use of adjustable sutures were not independently associated with surgeon reoperation rate. We explored associations of reoperation with patient characteristics, such as age and poverty. Surgeons in the South tended to have a higher reoperation rate (p=0.03) in a multivariable model. However, the multivariable model could only explain 16.3% of the inter-surgeon variation in reoperation rate for 1-horizontal muscle surgery. Strabismus surgery is similar to other areas of medicine, in which large variations in outcomes between surgeons are observed. Future work can be directed towards explaining this variation. Patient-level analyses that fail to consider variation between surgeons will be dominated by a small number of high-reoperation, high-volume surgeons. Order-of-magnitude variations exist in reoperation rates among strabismus surgeons, the cause of which is largely unexplained.
Topics: United States; Aged; Humans; Reoperation; Retrospective Studies; Medicare; Surgeons; Sutures; Strabismus
PubMed: 38617723
DOI: 10.22336/rjo.2024.03