Cerebral Visual Impairment Research Articles

Cerebral visual impairment is permanent but not unchanging.

Cerebral visual impairment is permanent but not unchanging.

An evaluation of health-related quality of life and its relation with functional vision in children with cerebral visual impairment

BackgroundHealth-related Quality of Life (HRQOL) and its relation with functional vision is understudied in cerebral visual impairment (CVI). AimsCharacterising HRQOL, comparing child self- and parent proxy-reports, and exploring relations with functional vision. Methods and proceduresSeventy-three children with CVI (n females = 33; n males = 40; Mean performance age = 7y2m) were included. HRQOL was measured with Pediatric Quality of Life Inventory (PedsQL) child self- and parent proxy-reports and compared using Wilcoxon signed-rank tests. Risk for impaired HRQOL was evaluated using cut-off scores. Parents scored functional vision using the Insight Questions Inventory and the Flemish CVI Questionnaire. Outcomes and results61 % (self-reported) or 66 % of children (proxy-reported) were at-risk for impaired total HRQOL. Ratings were correlated (rs = 0.305; p = 0.013); however, children rated higher total HRQOL compared to parent-proxy (r = 0.382; p = 0.002). The Insight Questions Inventory and the PedsQL proxy-report were correlated (rp = −0.454; p < 0.001), the Flemish CVI Questionnaire was negligibly correlated (rp = −0.244; p = 0.041). Conclusions and implicationsHRQOL is reduced in CVI, and both child and parent perspectives are crucial. HRQOL and functional vision are intricately related, and impairment in one negatively affects the other.

Natural history of cerebral visual impairment in children with cerebral palsy.

Natural history of cerebral visual impairment in children with cerebral palsy.

Knowledge of Cerebral Visual Impairment (CVI) in Children among Eye Care Professionals in Selangor and Kuala Lumpur, Malaysia

Objective: Cerebral Visual Impairment (CVI), a brain-based visual disorder that affects neural networks that process incoming visual information throughout the brain, poses challenges in diagnosis and managing visual impairment in children. With the prevalence rise in CVI cases among children, there is a growing demand for ophthalmologists and optometrists to address these conditions, emphasising the importance of improving knowledge and awareness for better visual rehabilitation. This study intends to quantify the level of understanding of CVI in children among eye care professionals in Selangor and Kuala Lumpur, Malaysia.Material and Methods: Two hundred thirty registered eye care professionals, including ophthalmologists and optometrists across Selangor and Kuala Lumpur, Malaysia, participated in this cross-sectional web-based survey. A standardised questionnaire was created through Google Forms and distributed via social media platforms between April and June 2022.Results: The finding showed that the eye care professionals have significantly basic knowledge regarding CVI in children (x2=369; df=2; p-value≤0.01). Most respondents correctly identified variable loss of vision, visual field defects, and abnormal behaviour as clinical features of CVI in children. Additionally, over half of the respondents selected magnetic resonance imaging (MRI) as the appropriate investigation choice.Conclusion: These findings indicate that eye care professionals have basic knowledge and awareness of CVI in children. More research is needed to enhance knowledge and raise awareness about CVI in children among eye-care professionals.

Global certification of visual impairment registries: A scoping review.

Visual impairment is a global problem which is predicted to rise in the coming years. Some of the biggest causes of visual impairment globally include uncorrected refractive error, cataract and age-related macular degeneration. People with a visual impairment often require support and so many countries hold registers of visual impairment. These registers can sit at a national, regional or local level. This scoping review aims to identify which countries hold visual impairment registries and have published data from them. Medline All, Embase and EBSCOHost were searched using several search terms after consulting an information specialist. All papers after the year 2000 were included in the scoping review. All results are shown using a PRISMA diagram and presented narratively. The total number of articles and papers identified was 1266; after screening and review, 57 articles were included in the review from 2000 to 2024. These articles came from 19 different countries and encompassed national, regional and local visual impairment databases. Many countries cited age-related macular degeneration as the major cause of blindness with diabetic retinopathy and glaucoma following. In less economically developed countries, refractive error was the main cause of sight loss. There were papers which focused on specific eye conditions such as glaucoma and diabetic retinopathy or on specific cohorts including working-age population and children. The leading causes of blindness in children appeared to be inherited retinal diseases, albinism and cerebral visual impairment. Certification of visual impairment is held differently across the world. There is commonality among different countries regarding the major causes of visual impairment in both adults and children. The importance of holding visual impairment registers to support people with a visual impairment and to plan services is essential.

Natural history of cerebral visual impairment in children with cerebral palsy.

To longitudinally evaluate the natural history of cerebral visual impairment (CVI) in children with cerebral palsy (CP) and identify which early visual signs or symptoms are associated with cognitive visual disorders (CVDs) at school age. Fifty-one individuals with CP and CVI underwent an ophthalmological, oculomotor, and basic visual function evaluation at three time points: T0 (6-35 months old); T1 (3-5 years old); and T2 (≥6 years old). We also performed a cognitive visual evaluation at T2. Logistic regression fitted using a generalized estimation equation (binary) and cumulative link models (ordinal) were used to model the outcomes of interest. Ophthalmological deficits were stable over time, except for ocular fundus abnormalities (T1-T0, p = 0.01; T2-T1, p = 0.02; T2-T0, p < 0.01) and strabismus, whose frequency increased with age (T2-T0, p= 0.02 with T2-T0, p = 0.05). Conversely, fixation (T1-T0, T2-T0, p < 0.01), smooth pursuit (T2-T1, T2-T0, p < 0.01), saccades (T1-T0, T2-T1, T2-T0, p < 0.01), as well as visual acuity, contrast sensitivity, and visual field (T1-T0, T2-T0, p < 0.01) all improved over time. Early oculomotor dysfunction was associated with CVD at T2. Although a diagnosis of CVI was confirmed in all children at each time point, several visual signs and symptoms improved over time; in some cases, they reached complete recovery at T1 and T2. These results emphasize the 'permanent' but 'not unchanging' nature of the CVI associated with CP during development.

Assessing Visuomotor Abilities in Cerebral Visual Impairment with Eye and Hand Tracking

Assessing Visuomotor Abilities in Cerebral Visual Impairment with Eye and Hand Tracking

Effect of Cerebral Visual Impairment on Functional-Structural Coupling of the Attention Networks

Effect of Cerebral Visual Impairment on Functional-Structural Coupling of the Attention Networks

Correlation of optic nerve on magnetic resonance imaging with visual acuity in children with cerebral visual impairment

Abstract: AIM: The aim of the study was to correlate the optic nerve dimensions at various locations on magnetic resonance imaging (MRI) with the visual acuity of children with cerebral visual impairment (CVI). MATERIALS AND METHODS: A 1-year cross-sectional study was conducted in a tertiary care hospital involving 60 children aged 1 to 12 years diagnosed with CVI but without ocular pathologies. Visual acuity was evaluated using teller acuity cards (TACs). MRI of the brain and orbit was performed using three-dimensional high-resolution, volumetric heavily T2-weighted sequences to screen for brain pathologies and measure optic nerve dimensions at two locations. The correlation between visual acuity and optic nerve dimensions was analyzed. The Chi-square test was employed to assess significance using SPSS software. RESULTS: In our study, 64% (n = 38) of the participants were children under 3 years of age. The severity and characteristics of CVI influenced visual functioning at different ages. As children with CVI grew older and moved from Phase I to Phase III, the proportion with very low visual acuity significantly decreased from 85.2% in Phase I to 22.2% in Phase III and from 63.2% in the &lt;3-year age group to 18.2% in more than 5-year age group. No direct correlation was found between optic nerve dimensions and visual acuity but optic disc changes such as optic disc pallor significantly correlated with low visual acuity. CONCLUSION: Our findings suggest that there may not be a direct correlation between optic nerve dimensions and visual acuity in children with CVI. However, optic disc changes do correlate with visual acuity, but optic disc changes alone are not a criterion for assessing visual function. Additionally, our study highlights the significance of a combined assessment by a neuro- ophthalmologist and neuro-pedaitrician in children with CVI.

Elucidating the clinical and genetic spectrum of inositol polyphosphate phosphatase INPP4A-related neurodevelopmental disorder

PurposeBiallelic INPP4A variants have recently been associated with severe neurodevelopmental disease in single-case reports. Here, we expand and elucidate the clinical-genetic spectrum and provide a pathomechanistic explanation for genotype-phenotype correlations. MethodsClinical and genomic investigations of 30 individuals were undertaken alongside molecular and in silico modelling and translation reinitiation studies. ResultsWe characterize a clinically variable disorder with cardinal features, including global developmental delay, severe-profound intellectual disability, microcephaly, limb weakness, cerebellar signs, and short stature. A more severe presentation associated with biallelic INPP4A variants downstream of exon 4 has additional features of (ponto)cerebellar hypoplasia, reduced cerebral volume, peripheral spasticity, contractures, intractable seizures, and cortical visual impairment. Our studies identify the likely pathomechanism of this genotype-phenotype correlation entailing translational reinitiation in exon 4 resulting in an N-terminal truncated INPP4A protein retaining partial functionality, associated with less severe disease. We also identified identical reinitiation site conservation in Inpp4a−/− mouse models displaying similar genotype-phenotype correlation. Additionally, we show fibroblasts from a single affected individual exhibit disrupted endocytic trafficking pathways, indicating the potential biological basis of the condition. ConclusionOur studies comprehensively characterize INPP4A-related neurodevelopmental disorder and suggest genotype-specific clinical assessment guidelines. We propose that the potential mechanistic basis of observed genotype-phenotype correlations entails exon 4 translation reinitiation.

Prevalence of cerebral visual impairment in developmental and Epileptic Encephalopathies: a systematic review protocol

BackgroundDevelopmental and Epileptic Encephalopathies (DEEs) are defined by drug-resistant seizures and neurodevelopmental disorders. Over 50% of patients have a genetic cause. Studies have shown that patients with DEEs, regardless of genetic diagnosis, experience a central visual function disorder known as Cerebral (cortical) Visual Impairment (CVI). The prevalence of CVI in DEE patients is currently unknown. A quantitative synthesis of existing data on the prevalence rates of this condition would aid in understanding the magnitude of the problem, outlining future research, and suggesting the need for therapeutic strategies for early identification and prevention of the disorder.MethodsThe protocol followed the PRISMA-P statement for systematic review and meta-analysis protocols. The review will adhere to the JBI Manual for Evidence Synthesis (Systematic Reviews of Prevalence and Incidence) and use the CoCoPop framework to establish eligibility criteria. We will conduct a comprehensive search of several databases, including MEDLINE, EMBASE, Science Direct, Scopus, PsychINFO, Wiley, Highwire Press, and Cochrane Library of Systematic Reviews. Our primary focus will be determining the prevalence of cerebral visual impairments (Condition) in patients with developmental and epileptic encephalopathy (Population). To ensure clarity, we will provide a narrative summary of the risk of bias in the studies we include. The Cochrane Q statistic will be used to assess heterogeneity between studies. If the quantitative synthesis includes more than 10 studies, potential sources of heterogeneity will be investigated through subgroup and meta-regression analyses. Meta(bias)es analysis will also be performed. The quality of evidence for all outcomes will be evaluated using the Grading of Recommendations Assessment Development and Evaluation (GRADE) working group methodology.DiscussionThis protocol outlines a systematic review and meta-analysis to identify, collect, evaluate, and integrate epidemiological knowledge related to the prevalence of CVI in patients with DEEs. To the best of our knowledge, no other systematic review and meta-analysis has addressed this specific issue. The results will provide useful information for understanding the extent of the problem, outlining future research, and suggesting the need for early identification strategies.Systematic review registrationsThis Systematic Review Protocol was registered in PROSPERO (CRD42023448910).

Virtual Reality With Eye Tracking for Pediatric Ophthalmology: A Systematic Review.

Virtual reality presents an efficient and engaging approach to ophthalmological diagnosis and treatment in children. This systematic review investigates the current state of eye tracking technology integrated into virtual reality for the spectrum of pediatric ophthalmology. The search encompassed the MEDLINE database via PubMed, without imposing any time restrictions. A total of 20 relevant studies that met our inclusion criteria were incorporated and categorized into the following domains: diagnosis, examination, treatment, and rehabilitation use of virtual reality devices within the realm of pediatric ophthalmology. Strabismus examinations and postoperative monitoring, inherited retinal degeneration examination and visual function testing, therapy of amblyopia, glaucoma visual field testing, cerebral visual impairment rehabilitation, and neuro-ophthalmic disease examination were included in the final analysis. Pediatric ophthalmology offers a promising landscape for the integration of eye tracking technology within virtual reality, with accelerated, quantifiable, and objective examination and diagnosis, and precise, real-time measurements that are crucial in children. Virtual reality is an engaging experience, easily applied in a pediatric setting and facilitating compliance during examination and adherence to therapy. Although our systematic review provides insights into the current state of research, it is anticipated that further exploration is required for the widespread utilization of eye tracking in virtual reality within pediatric ophthalmology. [J Pediatr Ophthalmol Strabismus. 20XX;X(X):XX-XX.].

Motor-Based Application Process for Cerebral Visual Impairment-Related Questionnaires for School-Age Children with Cerebral Palsy

This study aims to examine the psychometric properties of questionnaires related to cerebral visual impairment (CVI) in school-age children with cerebral palsy (CP). Additionally, it proposes an application process based on motor functionality for occupational therapists. A total of 288 children with CP were recruited for the study and administered the CVI Motor Questionnaire (CVI-MQ). The children’s daily visual performance was assessed using the CVI Inventory and the Functional Vision Questionnaire (FVQ) considering the children’s ambulatory status. The FVQ and the two factors extracted from the CVI Inventory significantly predicted visual functioning. Specific questionnaires related to CVI, applied with gross motor function in mind, can be valuable tools for occupational therapists to assess daily visual performance.

The Impact of Cerebral Visual Impairment on Social Skills and Sensory Processing.

Limited evidence exists on the impact of cerebral visual impairment (CVI) on developmental domains in toddlers. This study investigated whether CVI influences social skills and sensory processing in toddlers with cerebral palsy (CP). The study included toddlers with CP, with or without CVI, and typically developing (TD) toddlers. All parents completed the demographic information form. Social skills were assessed by the Brief Infant-Toddler Social and Emotional Assessment (BITSEA). Sensory processing was evaluated by the Infant/Toddler Sensory Profile (ITSP). Analysis of covariance was employed to assess the disparity in BITSEA and ITSP scores. Toddlers with CP and CVI group demonstrated lower social competence than the other groups (p < .05). Having CVI did not cause a statistically significant difference in sensory processing among toddlers with CP (p > .05). CVI significantly affects social competence in toddlers with CP but does not affect sensory processing.

Eyes on CVI: Eye movements unveil distinct visual search patterns in Cerebral Visual Impairment compared to ADHD, dyslexia, and neurotypical children

Visual search problems are often reported in children with Cerebral Visual Impairment (CVI). To tackle the clinical challenge of objectively differentiating CVI from other neurodevelopmental disorders, we developed a novel test battery. Visual search tasks were coupled with verbal and gaze-based measurements. Two search tasks were performed by children with CVI (n: 22; mean age (SD): 9.63 (.46) years) ADHD (n: 32; mean age (SD): 10.51 (.25) years), dyslexia (n: 28; mean age (SD): 10.29 (.20) years) and neurotypical development (n: 44; mean age (SD): 9.30 (.30) years). Children with CVI had more impaired search performance compared to all other groups, especially in crowded and unstructured displays and even when they had normal visual acuity. In-depth gaze-based analyses revealed that this group searched in overall larger areas and needed more time to recognize a target, particularly after their initial fixation on the target. Our gaze-based approach to visual search offers new insights into the distinct search patterns and behaviours of children with CVI. Their tendency to overlook targets whilst fixating on it, point towards higher-order visual function (HOVF) deficits. The novel method is feasible, valid, and promising for clinical differential-diagnostic evaluation between CVI, ADHD and dyslexia, and for informing individualized training.

A deep learning model for brain segmentation across pediatric and adult populations.

Automated quantification of brain tissues on MR images has greatly contributed to the diagnosis and follow-up of neurological pathologies across various life stages. However, existing solutions are specifically designed for certain age ranges, limiting their applicability in monitoring brain development from infancy to late adulthood. This retrospective study aims to develop and validate a brain segmentation model across pediatric and adult populations. First, we trained a deep learning model to segment tissues and brain structures using T1-weighted MR images from 390 patients (age range: 2-81 years) across four different datasets. Subsequently, the model was validated on a cohort of 280 patients from six distinct test datasets (age range: 4-90 years). In the initial experiment, the proposed deep learning-based pipeline, icobrain-dl, demonstrated segmentation accuracy comparable to both pediatric and adult-specific models across diverse age groups. Subsequently, we evaluated intra- and inter-scanner variability in measurements of various tissues and structures in both pediatric and adult populations computed by icobrain-dl. Results demonstrated significantly higher reproducibility compared to similar brain quantification tools, including childmetrix, FastSurfer, and the medical device icobrain v5.9 (p-value< 0.01). Finally, we explored the potential clinical applications of icobrain-dl measurements in diagnosing pediatric patients with Cerebral Visual Impairment and adult patients with Alzheimer's Disease.

Advancements in brain-computer interfaces for the rehabilitation of unilateral spatial neglect: a concise review.

This short review examines recent advancements in neurotechnologies within the context of managing unilateral spatial neglect (USN), a common condition following stroke. Despite the success of brain-computer interfaces (BCIs) in restoring motor function, there is a notable absence of effective BCI devices for treating cerebral visual impairments, a prevalent consequence of brain lesions that significantly hinders rehabilitation. This review analyzes current non-invasive BCIs and technological solutions dedicated to cognitive rehabilitation, with a focus on visuo-attentional disorders. We emphasize the need for further research into the use of BCIs for managing cognitive impairments and propose a new potential solution for USN rehabilitation, by combining the clinical subtleties of this syndrome with the technological advancements made in the field of neurotechnologies.

Visuospatial processing in early brain-based visual impairment is associated with differential recruitment of dorsal and ventral visual streams.

Visuospatial processing impairments are prevalent in individuals with cerebral visual impairment (CVI) and are typically ascribed to "dorsal stream dysfunction" (DSD). However, the contribution of other cortical regions, including early visual cortex (EVC), frontal cortex, or the ventral visual stream, to such impairments remains unknown. Thus, here, we examined fMRI activity in these regions, while individuals with CVI (and neurotypicals) performed a visual search task within a dynamic naturalistic scene. First, behavioral performance was measured with eye tracking. Participants were instructed to search and follow a walking human target. CVI participants took significantly longer to find the target, and their eye gaze patterns were less accurate and less precise. Second, we used the same task in the MRI scanner. Along the dorsal stream, activation was reduced in CVI participants, consistent with the proposed DSD in CVI. Intriguingly, however, visual areas along the ventral stream showed the complete opposite pattern, with greater activation in CVI participants. In contrast, we found no differences in either EVC or frontal cortex between groups. These results suggest that the impaired visuospatial processing abilities in CVI are associated with differential recruitment of the dorsal and ventral visual streams, likely resulting from impaired selective attention.

Expert consensus on the definition, diagnosis, assessment and intervention of cerebral visual impairment in children(2023)

Expert consensus on the definition, diagnosis, assessment and intervention of cerebral visual impairment in children(2023)

Temporal changes in incidence, prevalence and causes of childhood visual impairment - Learnings from 45 years with the National Danish Registry of Children with Visual Impairment.

The aim of the study was to describe the temporal changes in causes and prevalence of childhood visual impairment in Denmark based on the National Danish Registry of Children with Visual Impairment (NDRCVI). Annual reports on the NDRCVI since its establishment in 1979 were reviewed and data on the number of registered children and the causes for registration with a visual impairment were evaluated. The average annual incidence of childhood visual impairment in Denmark is 2.8 per 1000 live-born children and the prevalence of childhood visual impairment is 1.6 per 1000 children <18 years. Today, fewer children are severely visually impaired (visual acuity ≤6/60) at the time of registration (31.6% since 2010 vs. 51.1% in the 1980s). Cerebral visual impairment and optic nerve atrophy have remained common causes of childhood visual impairment whereas sequelae to retinopathy of prematurity have been almost eliminated as a cause. Systemic comorbidities are more common now in children with visual impairment (seen in 63.9% in the last decades vs. 44.6%in the 1980-ties). Whereas the prevalence of visual impairment has remained relatively stable over the years, the severity of visual impairment has improved, suggesting that more children will be able to live an active life supported by aids compensating vision loss. However, more children have systemic comorbidities in combination with their visual impairment suggesting that children with visual impairment face a life not only limited by the obstacles of poor vision. This calls for multidisciplinary management and support of affected children and families.