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BMC Ophthalmology Nov 2023V pattern identification is essential for proper strabismus management. Graded recession is a tailored approach to treat inferior oblique overaction (IOOA). The aim is...
BACKGROUND
V pattern identification is essential for proper strabismus management. Graded recession is a tailored approach to treat inferior oblique overaction (IOOA). The aim is to evaluate the efficacy of graded recession of inferior oblique muscle for correction of different grades of V pattern.
METHODS
Forty patients from 3 to 18 years old with V pattern strabismus and primary IOOA were evaluated by prism cover test to assess the grade of IOOA and amplitude of V-pattern. Graded recession of IO muscle depends on the amplitude of the V-pattern and degree of IOOA. Eight mm recession for amplitude 15 PD to 20 PD and mild IOOA (10 PD-15 PD or + 1) ,10 mm recession for amplitude 20-30 PD and moderate IOOA (15-25 PD or + 2) and maximum recession for amplitude more than 30 PD and marked IOOA (≥ 25 PD or + 3). Simultaneous correction of the horizontal deviation was performed. Follow up after I week,1 month ,3 month and 6-month. Trial Registration Number (TRN) (NCT05786053) on 23/3/2023.
RESULTS
The mean age of the study patients was 9 ± 4.261. Twenty patients (50%) had V-pattern esotropia, 12 (30%) exotropia, 4 (10%) orthotropic and four (10%) had Dissociated vertical deviation (DVD). Four cases 10% were of grade 1, 20 cases (50%) grade 2 and 16 cases (40%) were of grade 3. Of eighty eyes, 66 eyes (82.5%) were fully corrected with no residual IOOA, and 14 eyes (17.5%) were under corrected. V-pattern was corrected in 28 cases 70% and only 12cases (30%) had residual V-pattern grade 1.
CONCLUSIONS
Graded recession is an effective procedure for correction of V pattern strabismus with various grades of primary inferior oblique overaction. It can be tailored according to the the degree of IO overaction which is significantly related to the grade of V pattern. The 8 mm recession for IO was significantly related to recurrence or inadequate break of the V pattern in our studied cases. The grade of IOOA correlates with the amplitude of V-pattern. The amount of recession was planned according to preoperative IOOA and grade of V-pattern with frequent undercorrections obtained by the standard 8 mm recession. A + 2 overaction merits a 10-mm recession of the inferior oblique. A + 3 or + 4 overaction merits a 14-mm maximal recession.
Topics: Humans; Child, Preschool; Child; Adolescent; Oculomotor Muscles; Treatment Outcome; Ophthalmologic Surgical Procedures; Vision, Binocular; Strabismus; Ocular Motility Disorders; Orbital Diseases; Retrospective Studies
PubMed: 37974078
DOI: 10.1186/s12886-023-03210-x -
Translational Psychiatry Oct 2023Progressive supranuclear palsy (PSP) is a pure tauopathy, implicating davunetide, enhancing Tau-microtubule interaction, as an ideal drug candidate. However, pooling...
Progressive supranuclear palsy (PSP) is a pure tauopathy, implicating davunetide, enhancing Tau-microtubule interaction, as an ideal drug candidate. However, pooling patient data irrespective of sex concluded no efficacy. Here, analyzing sex-dependency in a 52 week-long- PSP clinical trial (involving over 200 patients) demonstrated clear baseline differences in brain ventricular volumes, a secondary endpoint. Dramatic baseline ventricular volume-dependent/volume increase correlations were observed in 52-week-placebo-treated females (r = 0.74, P = 2.36), whereas davunetide-treated females (like males) revealed no such effects. Assessment of primary endpoints, by the PSP Rating Scale (PSPRS) and markedly more so by the Schwab and England Activities of Daily Living (SEADL) scale, showed significantly faster deterioration in females, starting at trial week 13 (P = 0.01, and correlating with most other endpoints by week 52). Twice daily davunetide treatments slowed female disease progression and revealed significant protection according to the SEADL scale as early as at 39 weeks (P = 0.008), as well as protection of the bulbar and limb motor domains considered by the PSPRS, including speaking and swallowing difficulties caused by brain damage, and deterioration of fine motor skills, respectably (P = 0.01), at 52 weeks. Furthermore, at 52 weeks of trial, the exploratory Geriatric Depression Scale (GDS) significantly correlated with the SEADL scale deterioration in the female placebo group and demonstrated davunetide-mediated protection of females. Female-specific davunetide-mediated protection of ventricular volume corresponded to clinical efficacy. Together with the significantly slower disease progression seen in men, the results reveal sex-based drug efficacy differences, demonstrating the neuroprotective and disease-modifying impact of davunetide treatment for female PSP patients.
Topics: Male; Humans; Female; Aged; Supranuclear Palsy, Progressive; Activities of Daily Living; Sex Factors; Disease Progression
PubMed: 37845254
DOI: 10.1038/s41398-023-02618-9 -
Brain Sciences Nov 2023The clinical category of immune-mediated cerebellar ataxias (IMCAs) has been established after 3 decades of clinical and experimental research. The cerebellum is...
The clinical category of immune-mediated cerebellar ataxias (IMCAs) has been established after 3 decades of clinical and experimental research. The cerebellum is particularly enriched in antigens (ion channels and related proteins, synaptic adhesion/organizing proteins, transmitter receptors, glial cells) and is vulnerable to immune attacks. IMCAs include various disorders, including gluten ataxia (GA), post-infectious cerebellitis (PIC), Miller Fisher syndrome (MFS), paraneoplastic cerebellar degeneration (PCD), opsoclonus myoclonus syndrome (OMS), and anti-GAD ataxia. Other disorders such as multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), Behçet disease, and collagen vascular disorders may also present with cerebellar symptoms when lesions are localized to cerebellar pathways. The triggers of autoimmunity are established in GA (gluten sensitivity), PIC and MFS (infections), PCD (malignancy), and OMS (infections or malignant tumors). Patients whose clinical profiles do not match those of classic types of IMCAs are now included in the spectrum of primary autoimmune cerebellar ataxia (PACA). Recent remarkable progress has clarified various characteristics of these etiologies and therapeutic strategies in terms of immunotherapies. However, it still remains to be elucidated as to how immune tolerance is broken, leading to autoimmune insults of the cerebellum, and the consecutive sequence of events occurring during cerebellar damage caused by antibody- or cell-mediated mechanisms. Antibodies may specifically target the cerebellar circuitry and impair synaptic mechanisms (synaptopathies). The present Special Issue aims to illuminate what is solved and what is unsolved in clinical practice and the pathophysiology of IMCAs. Immune ataxias now represent a genuine category of immune insults to the central nervous system (CNS).
PubMed: 38137074
DOI: 10.3390/brainsci13121626 -
Survey of Ophthalmology 2023One of the most common untoward occurrences during strabismus surgery at all ages is the oculocardiac reflex. Although typically easily treated, the sudden bradycardia... (Review)
Review
One of the most common untoward occurrences during strabismus surgery at all ages is the oculocardiac reflex. Although typically easily treated, the sudden bradycardia or cardiac arrest may add a few gray hairs to ophthalmologists and anesthesiologists alike as it can be potentially fatal. This updated review of the literature and novel detailed treatment algorithm may prevent patient morbidity and mortality through proper recognition of at-risk patients and rapid treatment through proper communication between surgical and anesthesia physicians/providers.
Topics: Child; Humans; Adult; Reflex, Oculocardiac; Bradycardia; Anesthetics; Strabismus
PubMed: 37116545
DOI: 10.1016/j.survophthal.2023.04.004 -
Progress in Retinal and Eye Research Jul 2023Congenital PAX6-aniridia, initially characterized by the absence of the iris, has progressively been shown to be associated with other developmental ocular abnormalities... (Review)
Review
Congenital PAX6-aniridia, initially characterized by the absence of the iris, has progressively been shown to be associated with other developmental ocular abnormalities and systemic features making congenital aniridia a complex syndromic disorder rather than a simple isolated disease of the iris. Moreover, foveal hypoplasia is now recognized as a more frequent feature than complete iris hypoplasia and a major visual prognosis determinant, reversing the classical clinical picture of this disease. Conversely, iris malformation is also a feature of various anterior segment dysgenesis disorders caused by PAX6-related developmental genes, adding a level of genetic complexity for accurate molecular diagnosis of aniridia. Therefore, the clinical recognition and differential genetic diagnosis of PAX6-related aniridia has been revealed to be much more challenging than initially thought, and still remains under-investigated. Here, we update specific clinical features of aniridia, with emphasis on their genotype correlations, as well as provide new knowledge regarding the PAX6 gene and its mutational spectrum, and highlight the beneficial utility of clinically implementing targeted Next-Generation Sequencing combined with Whole-Genome Sequencing to increase the genetic diagnostic yield of aniridia. We also present new molecular mechanisms underlying aniridia and aniridia-like phenotypes. Finally, we discuss the appropriate medical and surgical management of aniridic eyes, as well as innovative therapeutic options. Altogether, these combined clinical-genetic approaches will help to accelerate time to diagnosis, provide better determination of the disease prognosis and management, and confirm eligibility for future clinical trials or genetic-specific therapies.
Topics: Humans; PAX6 Transcription Factor; Aniridia; Eye Abnormalities; Mutation; Phenotype; Eye Proteins
PubMed: 36280537
DOI: 10.1016/j.preteyeres.2022.101133 -
BMC Ophthalmology Jun 2024The long-term visual outcomes in spasmus nutans patients is largely unknown. The purpose of this study was to characterize visual outcomes and identify comorbid...
BACKGROUND
The long-term visual outcomes in spasmus nutans patients is largely unknown. The purpose of this study was to characterize visual outcomes and identify comorbid ophthalmic conditions in patients with spasmus nutans.
METHODS
We retrospectively reviewed the charts of consecutive patients diagnosed with spasmus nutans between 2000 and 2020. Demographic information, ophthalmic characteristics, and neuroimaging results were assessed over time.
RESULTS
Of the 32 patients included in the study, 13 (41%) were female. Underlying medical conditions included a diagnosis of Trisomy 21 in 6 (19%) and prematurity in 8 (25%). Twenty-one patients (66%) self-reported as a race other than Caucasian. 18 patients (56%) had non-private health insurance and 1 (3%) was uninsured. Mean age at diagnosis and resolution were 16 months (range 45 months) and 48 months (range 114 months), respectively. All 32 patients had nystagmus, 31 (97%) had head nodding and 16 (50%) had ocular torticollis. Mean follow-up was 66 months (range 185 months). On initial presentation, 6/32 (19%) had an amblyogenic refractive error and mean best-corrected visual acuity (BCVA) in the better-seeing eye was 0.78 Logarithm of the Minimum Angle of Resolution (LogMAR) (range 1.24). In a sub-analysis that included patients with > 1 exam (n = 23), 17/20 (85%) had an amblyogenic refractive error and mean BCVA in the better-seeing eye was 0.48 LogMAR (range 1.70). At the final exam, 12 patients had measurable stereopsis, eight had strabismus, and three had undergone strabismus surgery. Eight patients required treatment for amblyopia.
CONCLUSIONS
We found a high prevalence of amblyogenic refractive error, strabismus and amblyopia among patients with spasmus nutans. Children with spasmus nutans benefit from ongoing ophthalmic follow-up until they are past the amblyopic age range, even after resolution of nystagmus.
Topics: Humans; Female; Male; Retrospective Studies; Visual Acuity; Child, Preschool; Child; Infant; Follow-Up Studies; Nystagmus, Pathologic; Amblyopia; Strabismus
PubMed: 38867186
DOI: 10.1186/s12886-024-03494-7 -
Ophthalmic & Physiological Optics : the... Jul 2023Individuals with different types of intermittent exotropia (IXT) may use neurally coupled accommodation and vergence responses differently from those without exotropia...
PURPOSE
Individuals with different types of intermittent exotropia (IXT) may use neurally coupled accommodation and vergence responses differently from those without exotropia to achieve eye alignment. This study examined the relationship between simultaneously recorded accommodation and vergence responses in children and young adults with a range of types of IXT while aligned and deviated.
METHODS
Responses of 29 participants with IXT (4-31 years) and 24 age-matched controls were recorded using simultaneous eye-tracking and eccentric photorefraction while they watched a movie in binocular or monocular viewing at varying viewing distances. Gradient response AC/A ratios and fusional vergence ranges were also assessed. Eight participants had divergence or pseudo-divergence excess type IXT, 5 had convergence insufficiency and 16 had basic IXT.
RESULTS
Control and IXT participants accommodated similarly both in monocular and binocular-aligned conditions to visual targets at 80 and 33 cm. When deviated in binocular viewing, most participants with IXT exhibited changes in accommodation <0.5D relative to alignment. Gradient response AC/A ratios were similar for control [0.56 MA/D (IQR: 0.51 MA/D)] and IXT participants [0.42 MA/D (0.54 MA/D); p = 0.60]. IXT participants showed larger vergence to accommodation ratios with changes from distance to near fixation [1.19 MA/D (1.45 MA/D)] than control participants [0.78 MA/D (0.60 MA/D); p = 0.02], especially among IXT participants with divergence or pseudo-divergence excess. Participants with IXT exhibited typical fusional divergence ranges beyond their dissociated position [8.86 Δ (7.10 Δ)] and typical fusional convergence ranges from alignment [18 Δ (15.75 Δ)].
CONCLUSIONS
This study suggests that control of IXT is typically neither driven by accommodative convergence alone nor associated with over-accommodation secondary to fusional convergence efforts. These simultaneous measurements confirmed that proximal vergence contributed significantly to IXT control, particularly for divergence or pseudo-divergence excess type IXT. For IXT participants in this study, achieving eye alignment did not conflict with having clear vision.
Topics: Child; Young Adult; Humans; Exotropia; Vision, Binocular; Convergence, Ocular; Accommodation, Ocular; Strabismus; Chronic Disease
PubMed: 36692334
DOI: 10.1111/opo.13093 -
Graefe's Archive For Clinical and... Jul 2023Strabismus is not a condition in itself but the consequence of an underlying problem. Eye misalignment can be caused by disease, injury, and/or abnormalities in any of... (Review)
Review
Strabismus is not a condition in itself but the consequence of an underlying problem. Eye misalignment can be caused by disease, injury, and/or abnormalities in any of the structures and processes involved in visual perception and oculomotor control, from the extraocular muscles and their innervations to the oculomotor and visual processing areas in the brain. A small percentage of all strabismus cases are the consequence of well-described genetic syndromes, acquired insult, or disease affecting the extraocular muscles (EOMs) or their innervations. We will refer to them as strabismus of peripheral origin since their etiology lies in the peripheral nervous system. However, in most strabismus cases, that is comitant, non-restrictive, non-paralytic strabismus, the EOMs and their innervations function properly. These cases are not related to specific syndromes and their precise causes remain poorly understood. They are generally believed to be caused by deficits in the central neural pathways involved in visual perception and oculomotor control. Therefore, we will refer to them as central strabismus. The goal of this narrative review is to discuss the possible causes behind this particular type of eye misalignment and to raise awareness among eyecare professionals about the important role the central nervous system plays in strabismus etiology, and the subsequent implications regarding its treatment. A non-systematic search was conducted using PubMed, Medline, Cochrane, and Google Scholar databases with the keywords "origins," "causes," and "etiology" combined with "strabismus." A snowball approach was also used to find relevant references. In the following article, we will first describe EOM integrity in central strabismus; next, we will address numerous reasons that support the idea of central nervous system (CNS) involvement in the origin of the deviation, followed by listing several possible central causes of the ocular misalignment. Finally, we will discuss the implications CNS etiology has on strabismus treatment.
Topics: Humans; Oculomotor Muscles; Syndrome; Strabismus; Eye Movements; Central Nervous System
PubMed: 36680614
DOI: 10.1007/s00417-022-05935-9 -
Science Progress 2023Original texts and expert translations from various ancient cultures covering a time span from the 2 millennium BC to the ninth century AD were searched for historical... (Review)
Review
Original texts and expert translations from various ancient cultures covering a time span from the 2 millennium BC to the ninth century AD were searched for historical observations of involuntary eye movements. Abnormal, spontaneous eye movements are an easily recognisable neuro-ophthalmological symptom that can be both congenital and acquired. Ocular oscillations termed 'hippos' by Hippocrates (460-370BC) and Galenos (129-216AD) are synonymous with nystagmus, a term first introduced in the eighteenth century. The original description of hippos suggests an innate onset, which retrospectively can be related to either congenital (infantile) nystagmus or continuous involuntary eye movements of the blind. Other descriptions of abnormal involuntary eye movements with different beating directions, possibly associated with vertigo, seizures or ear symptoms and their impact on patients' quality of life (e.g. oscillopsia, blurred vision) are preserved in many fragmentary ancient documents including papyrus scrolls and stone tablets from Egypt, Mesopotamia, India, China, Greece, Rome and the Middle East. Although the sparse original descriptions of the direction and type of eye movements may inspire daring medical interpretations, caution is required when attempting to assign them to distinct nystagmus forms according to our current clinical classification of ocular motor disorders.
Topics: Humans; Eye Movements; Quality of Life; Retrospective Studies; Nystagmus, Pathologic; Seizures
PubMed: 37642983
DOI: 10.1177/00368504231191986 -
Journal of Pediatric Ophthalmology and... 2023
Topics: Humans; Adult; Telemedicine; Strabismus
PubMed: 38019942
DOI: 10.3928/01913913-20231025-01