Visual Deficits Research Articles

MAIA and Humphrey Perimetry Differ in Their Estimation of Homonymous Visual Field Defects.

To assess whether the Humphrey Visual Field Analyzer (HFA) and the Macular Integrity Assessment instrument (MAIA) provide equivalent estimates of visual deficit size, severity, and progression in cortically blinded participants. Reliable, monocular 10-2 HFA and MAIA fields were collected at baseline, and after a blind-field training intervention (n = 54) or no intervention (n = 6) in adult participants with occipital strokes. Binocular HFA and MAIA mean sensitivities (MS) were first computed, before creating binocular maps of visual sensitivity for each perimetry system to calculate deficit areas, using a unitary, published, less than 10 dB criterion to define blindness. We contrasted HFA/MAIA MS and deficit area at individual study visits, together with change in these measures between visits. At individual visits, MS and deficit areas were well-correlated, but there were systematic differences between machines, greater for the intact than impaired hemifields, with the MAIA overestimating areas of visual deficit relative to the HFA. Between visits, the two perimeters' assessment of change in MS was correlated, but change in the deficit area was not, despite good test reliability. Reliable HFA and MAIA tests produce well-correlated but systematically offset estimates of visual sensitivity and deficit area in patients with homonymous field defects, which can impact assessment of progression. Cortically blinded patients exhibited comparable changes in sensitivity (spontaneous and training induced) when assessed using Humphrey and fundus-controlled MAIA perimetry. However, the photopic vs. mesopic nature of these tests impacted participant performance and suggests that custom, machine-specific criteria are needed to comparably define blindness on both systems.

NCMP-03. ASSOCIATION OF PROGESTERONE CONTRACEPTION WITH TUMOR-RELATED VISUAL IMPAIRMENT IN PREMENOPAUSAL WOMEN WITH MENINGIOMA REFERRED FOR RADIATION

Abstract BACKGROUND Meningiomas express ER/PR, but hormonal risk is poorly characterized and not always considered. We hypothesized that hormonal contraceptives lead to tumor-related visual deficits in premenopausal women referred for radiation. METHODS We conducted a retrospective case-control study with primary endpoint visual deficit prior to radiation for meningioma in women age ≤55. Key risk factors determined included age, grade, skull base location, hormonal supplementation / contraception, and neurofibromatosis / prior radiation (NF/priorRT). Univariate and multivariate (including factors with p<0.05 on univariate) logistic regression was performed to analyze associations with visual deficit. RESULTS 64 women (median age 46) receiving RT from 2012-2024 at a single academic institution were included. 45 (70%) had skull base tumors, 33 (52%) used estrogen or progesterone, and 23 (36%) used unopposed progesterone only, 15 specifically medroxyprogesterone acetate (Provera). Visual deficit was present in 35 patients (55%) and was independently associated with both skull base location (p=0.002, OR 11.5) and unopposed progesterone only use (p=0.008, OR 12) but not estrogen or progesterone use on multivariate analysis. Proportion with progesterone only use was 57% vs. 11% in patients with vs. without visual deficit (chi-sq p<0.001). In 5 with ER/PR testing, 3 were PR+ (all with visual deficits and history of medroxyprogesterone acetate) and 2 were PR- (neither with visual deficits nor hormone use). 13 women (20%) were actively using progesterone during RT. 17 women have been contacted at time of analysis, and 4 discontinued progesterone. CONCLUSIONS Progesterone increases risk for meningioma-related visual deficits in premenopausal women referred for radiotherapy, with a disproportionate number on Provera specifically. These data highlight the importance of age-appropriate counseling, consistent pathologic assessment of ER/PR status, and greater awareness of this issue amongst neurosurgeons and radiation oncologists.

A Review of Sturge–Weber Syndrome Brain Involvement, Cannabidiol Treatment and Molecular Pathways

Sturge–Weber syndrome (SWS) is a rare congenital neurocutaneous disorder typically caused by a somatic mosaic mutation in R183Q GNAQ. At-risk children present at birth with a capillary malformation port-wine birthmark. The primary diagnostic characteristic of the disorder includes leptomeningeal enhancement of the brain, which demonstrates abnormal blood vessels and results in impaired venous drainage and impaired local cerebral perfusion. Impaired cerebral blood flow is complicated by seizures resulting in strokes, hemiparesis and visual field deficits, hormonal deficiencies, behavioral impairments, and intellectual disability. Therefore, anti-seizure medication in combination with low-dose aspirin is a common therapeutic treatment strategy. Recently published data indicate that the underlying mutation in endothelial cells results in the hyperactivation of downstream pathways and impairment of the blood–brain barrier. Cannabidiol (CBD) has been used to treat medically refractory seizures in SWS due to its anti-seizure, anti-inflammatory, and neuroprotective properties. Pilot research suggests that CBD improves cognitive impairment, emotional regulation, and quality of life in patients with SWS. Recent preclinical studies also suggest overlapping molecular pathways in SWS and in CBD, suggesting that CBD may be uniquely effective for SWS brain involvement. This review aims to summarize early data on CBD’s efficacy for preventing and treating epilepsy and neuro-cognitive impairments in patients with SWS, likely molecular pathways impacted, and provide insights for future translational research to improve clinical treatment for patients with SWS.

Lack of retinal degeneration in a Dram2 knockout mouse model

Damage-regulated autophagy modulator 2 (DRAM2) is a homologue of the DRAM family protein, which can induce autophagy process. In the retina, DRAM2 is located to the inner segment of photoreceptors, the apical surface of retinal pigment epithelial (RPE) cells, and the lysosome. Pathogenic variants of DRAM2 lead to autosomal recessive Cone-rod dystrophy 21 (CORD21). Cone-rod dystrophy is characterised by primary cone involvement, or sometimes simultaneous cone and rod loss, thus leading to decreased visual acuity, colour vision deficits, photophobia, and decreased sensitivity of the central visual field. However, the mechanisms underlying DRAM2 related retinal diseases remained unclear. To further explore the role of Dram2 in the retina, we generated Dram2 knockout mice (KO) by CRISPR/Cas-9 technology and demonstrated that expression of DRAM2 was abolished in KO retinas. Dram2 ablation failed to manifest any retinal degenerative phenotypes. Dram2 KO did not exhibit visible defect in photo response and the overt structure of the retinas. Immunostaing analysis using antibodies against cone opsins revealed no detectable loss of cone cells. Moreover, no visible change was observed in the expression and localisation of rhodopsin and other membrane disc proteins in Dram2 KO retinas and no gliosis and apoptosis were detected in KO mice. In summary, these data revealed lack of overt retinal degeneration in Dram2 KO model and emphasized the importance of further investigation of the mechanisms underlying Cone-rod dystrophy 21.

The role of intervention timing and treatment modality in visual recovery following pituitary apoplexy: a systematic review and meta-analysis.

Pituitary apoplexy has historically been considered an emergent condition that necessitates surgical intervention when there is acute symptomatic onset. This potentially serious condition often occurs in the setting of an underlying adenoma, cystic lesion, or other sellar mass. When these mass lesions hemorrhage within the confined space of the sella turcica, the pituitary gland is subjected to hemorrhagic ischemia. Furthermore, critical neurovasculature in close proximity to the sella can sustain collateral damage. In the present study, we investigate whether early versus delayed surgical intervention (in terms of three timelines: before versus after 48h, 72h, and 7days, respectively) results in differences in visual outcomes for patients experiencing pituitary apoplexy with acute onset neurological and/or neuro-opthalmic symptoms. Furthermore, we compare the efficacy of surgical decompression versus expectant management of this condition. Accordingly, we queried the PubMed, Scopus, and Embase databases in adherence to PRISMA guidelines. Quantitative meta-analysis was performed according to the Mantel-Haenszel method and forest plots were generated using Review Manager v5.4. P-values < 0.05 were defined as the threshold for statistical significance. Twenty-nine studies remained eligible for review following initial search and screen, including 16 studies describing the role of intervention timing and 15 studies comparing intervention modality. Most patients presented with a visual deficit, and all patients underwent surgery - most commonly via the endoscopic endonasal (EEA) approach. Two hundred and twenty patients were included in the sub-analysis for the 7-day cutoff point. Furthermore, 81 patients underwent surgical decompression of the sella prior to 48h, and 32 patients underwent surgical decompression between 48-72h following presentation. Almost all patients exhibited improved vision post-decompression, including 19/19 patients (100%) in the post-72-h cohort. On meta-analysis using the Mantel-Haenszel method, there was a significant difference in vision outcomes in favor of patients who underwent surgical decompression before 7days as compared to after seven days (OR 5.88, 95% CI [1.77, 19.60], I2 = 0%, p < 0.01). In a separate sub-analysis, there was a total of 288 patients across 15 studies comparing surgical versus conservative management of pituitary apoplexy. These management options proved equivocal on meta-analysis (p > 0.05). In the present study, timing of surgical intervention for pituitary apoplexy was predictive of visual function recovery only at the 7-day timepoint, as has been reported by previous studies. Ultimately, this suggests that pituitary apoplexy involving severe visual deficits or altered mental status is best addressed within the first seven days post-presentation, and that both surgery and conservative management can offer similar outcomes. When apoplexy is suspected, IV corticosteroids should be administered independent of acuity or severity to prevent secondary adrenal crisis. Subsequently, for patients presenting without severe visual or other neurological deficits, expectant management is recommended. Management should be patient-specific and dependent upon the severity of symptoms present at onset.

Enhancing Late Retinopathy of Prematurity Outcomes with Fresh Bone Marrow Mononuclear Cells and Melatonin Combination Therapy.

Retinopathy of prematurity (ROP) is a vasoproliferative disease affecting premature neonates with life-lasting impacts. This study aims to investigate the long-term functional outcomes and alterations in neural retina architecture following the intravitreal transplantation of bone marrow mononuclear cells (BMMNC) in the rat models of ROP, and to evaluate the effect of adjunctive therapy with melatonin. 32 neonate rats were employed. The ROP model was developed in 10 neonatal rats, and two were assigned as control. The ROP models received BMMNC suspension, containing 1.2 × 105cells, in their right eye, and normal saline in left at p12. Five ROP rats received 12.5 mg/kg melatonin orally for five days (p12 to p17). Optical coherence tomography (OCT) and electroretinography (ERG) were performed on p47. Eyes were then harvested on p47, and after six months for histology, immunofluorescence (anti-calbindin, anti-PKC, and anti-Brn3), and immunohistochemistry (synaptophysin). Cell therapy alone and with melatonin increased retinal thickness, and improved oscillatory potentials on ERG. Combination therapy increased horizontal and retinal ganglion cell populations. All treatments improved synaptic maturity in the inner plexiform layer, but only combination therapy was effective on the outer plexiform layer. Melatonin and BMMNCs combination therapyeffectively ameliorates retinal structural and functional deficits at later ROP stages, without causing severe adverse effects.It significantly increases the survival of post-receptor retinal neurons and preserves retinal synaptic structures in the long term, highlighting the promising potential of this novel combination therapy approach to minimize visual deficits in ROP patients.

Network-based hub biomarker discovery for glaucoma

ObjectiveGlaucoma is an optic neuropathy and the leading cause of irreversible blindness worldwide. However, the early detection of glaucoma remains challenging, as chronic forms of glaucoma remain largely asymptomatic until...

Long-lasting pure topographical disorientation due to heading disorientation following left retrosplenial infarction: A report of two cases

Topographical disorientation is linked to lesions in the right hemisphere and typically resolves within a few months post-stroke. Persistent topographical disorientation is uncommon and frequently accompanied by impairments in visual memory, complicating the analysis of the underlying mechanisms. Herein, we report two cases of sustained pure topographical disorientation following cerebral hemorrhages in the left retrosplenial region. The patients exhibited disorientation in both familiar and unfamiliar settings, attributable to heading disorientation, a deficit in determining the directional relationship between one’s current position and a target location or external frames. The patients struggled with reconstructing large-scale spatial frameworks and integrating new egocentric and allocentric perspectives upon changes in body orientation. There were no landmark agnosia, egocentric disorientation, or anterograde disorientation. Although mild verbal memory deficits were observed, no other cognitive impairments, including visual memory deficits, were detected. Our findings imply that lesions confined to the left retrosplenial region can induce enduring heading disorientation and suggest a significant role for this area in processing and integrating spatial information necessary for large-scale navigation. Clarifying the features of topographical disorientation will significantly impact the therapeutic approaches, enhancing the quality of life for affected patients by restoring their independence and mobility.

Persistent visual field deficit after half-fluence photodynamic therapy for chronic central serous chorioretinopathy.

To report a rare case of a persistent paracentral scotoma after half-fluence photodynamic therapy for chronic central serous chorioretinopathy. A 65-year-old man with a history of chronic central serous chorioretinopathy presented with a temporal paracentral visual distortion one week after undergoing repeat off-label treatment with half-fluence verteporfin photodynamic therapy for recurrent intraretinal fluid exudation in the right eye. Fundus photography, fundus autofluorescence, optical coherence tomography, fluorescein angiography, indocyanine green angiography, and automatic visual field testing were obtained. Humphrey visual field 10-2 demonstrated a focal temporal paracentral area of reduced sensitivity, which remained persistent on repeat testing up to 9 months. OCT macula and fluorescein angiography demonstrated subtle corresponding changes in the nasal macula, while indocyanine green angiography demonstrated no visible alteration in choroidal perfusion. Symptomatic scotoma in the location of laser treatment may be a rare complication of verteporfin photodynamic therapy even at reduced fluence, in the setting of repeat treatment in the same location, and may be identified and monitored with automated 10-2 visual field testing.

Influence of Subjective Postural Vertical with Closed and Open Eyes in Patients with Hemiplegic and Pusher Behavior with Unilateral Spatial Neglect After Stroke: A Cross-Sectional Study

Background: When integrating visual and somatosensory processing into the subjective postural vertical using the Romberg test, patients with hemiplegic can be sorted into either post-stroke or pushers with unilateral spatial neglect (USN). This study aimed to clarify the characteristics of the integrated processing of the integrated subjective postural vertical (ISPV) with open or closed eyes in patients with hemiplegic and/or pusher with USN. Methods: This cross-sectional study included 91 patients with hemiplegic and 45 with pusher and USN. The outcomes included the ratio and sum of SPV with the eyes closed and open. Statistical analyses were performed using the parametric and/or non-parametric Wilcoxon rank-sum test, Mann–Whitney U test, or chi-square test after the Shapiro–Wilk test. Results: The outcomes in the 91 patients with hemiplegic were as follows: moderate-to-severe ISPV with ratio, 1.64°; ISPV sum (ISPVS), 9.41°. The outcomes in the 45 patients with pusher and USN were as follows: moderate-to-severe, ISPV: 1.35°, and ISPVS: 13.96°. No significant differences were observed between the two groups in terms of demographic data or ISPV. However, the number of patients with pusher syndrome was significantly higher in the ISPVS group than in stroke patients with hemiplegic. Conclusions: Adaptation occurs by integrating sensory modalities, and the pusher behavior in patients with USN is characterized by the specific pathophysiology of a two-modality disorder with visual and somatosensory deficits. This study provides key insights into the pathophysiological characteristics of patients with pusher syndrome and USN.

Combination Resective or Ablative Epilepsy Surgery with Neurostimulation for Complex Epilepsy Networks: A Case Series

Introduction: Complex epilepsy networks with multifocal onset zones that overlap with eloquent cortex may benefit from combined surgical approaches. However, limited data exist on outcomes associated with performing these therapies in tandem. In this case series, we report on 6 patients who underwent combination surgery with either resection or laser interstitial thermal therapy (LITT) and neuromodulation with responsive neurostimulation (RNS) or deep brain stimulation (DBS). Methods: We performed a retrospective review of adult patients with medically refractory epilepsy who underwent staged combination epilepsy surgeries during the same admission at our institution. Six cases treated between 2019 and 2023 were identified. All patients underwent a presurgical work-up including invasive intracranial monitoring and underwent a combined approach with either surgical resection, LITT, RNS, or DBS. We extracted data on demographic, clinical, and surgical characteristics. The primary outcome was change in seizure frequency from baseline. Results: The mean age was 42.7 years old (4 female). All patients had at least one epileptogenic zone in the temporal lobe, two in extratemporal neocortex, two in periventricular nodular heterotopia. For the staged combination approach, 3 patients underwent LITT followed by RNS, two underwent resection and RNS, and one received LITT and DBS. The mean reduction in seizure frequency per month at last follow-up was 90%. Postoperatively, 1 patient experienced superior visual field deficits related to LITT, and another had postoperative deep vein thrombosis. Conclusion: All patients experienced at least an 83% reduction in seizures. This case series demonstrates the potential benefits of a combined surgical approach in patients with multifocal seizures and at least one lesion that can be safely resected or ablated. Future prospective studies are warranted.

Delayed-onset cord1 progressive retinal atrophy in English Springer Spaniels genetically affected with the RPGRIP1 variant.

Cone-rod dystrophy (cord1) is a form of progressive retinal atrophy. It is linked to an RPGRIP1 genetic variant which is the third most common canine disease variant thus far. While the variant affects various breeds, it is highly prevalent in English Springer Spaniels (ESSs). Yet its clinical and pathological implications remain equivocal. Herein, we study the retinal phenotype in ESSs genetically affected with the RPGRIP1 variant. Over 4 years, 494 ESSs (123 affected) were enrolled. Owner-perceived vision was collected via a questionnaire. Ophthalmic examination included fundus photography. In selected ESSs, retinal function and structure were assessed using electroretinography (ERG, 148 dogs) and optical coherence tomography (OCT, 4 dogs). Ophthalmoscopic changes included peripheral hypo-reflective lesions often with distinct borders progressing centripetally culminating in generalized retinal atrophy. Cross-sectional study revealed declining photopic ERG amplitudes with age in the affected group but not in controls. OCT indicated progressive photoreceptor loss. Despite ophthalmoscopic, ERG, or OCT abnormalities, most affected dogs were not visually impaired per their owners. In a fraction of afflicted ESSs, vision/globe-threatening complications were documented including cataracts, lens luxation, and glaucoma. In ESSs, the RPGRIP1 variant is associated with insidious pathology with delayed-onset visual defects. The subtle phenotype without apparent visual deficit until the final years of life, if at all, may have caused underdiagnosis of cord1. Still, DNA testing remains informative, and ERG and OCT indicate progressive pathology. Peripheral fundus examination and photopic ERG are particularly useful for early detection and monitoring of cord1.

POSTOPERATIVE VISUAL fiELD DEfiCITS ARE NOT ASSOCIATED WITH REDUCTION IN QUALITY OF LIFE IN GLIOBLASTOMA PATIENTS

Abstract AIMS Preservation of functional integrity is a core goal of glioblastoma surgery. Visual field deficits can significantly impact quality of life and patient function. Both tumour and surgical effects may affect visual function in these patients. The objective of this Study was to evaluate the effect of visual field deficits on HRQoL in the preoperative and early postoperative period (prior to chemoradiotherapy). METHOD 48 patients with glioblastoma received preoperative, early postoperative (withon 1 week) and delayed postoperative testing (6 weeks) for visual function usinga combination of the OCS-Bridge neurocognitive screening tool and formal visual field testing. Patients also completed the EORTC QLQ C30 with BN-20 and VFQ-25 visual function questionaires at the same timepoints. Statistical tests were performed using R Statistical Software (v4.1.2; R Core Team 2021). RESULTS The proportion of patients with visual field deficits at preoperative, early postoperative and late early postoperative timepoints were 20 (41.7%), 28 (58.3%) and 24 (50%) respectively. Most patients without a preoperative deficit did not have an early 17/20 85% or late postoperative deficit 19/24 79.1%. The proportion of patients with deficits at early and late postoperative timepoints was significantly higher in patients with preoperative deficits compared to patients without preoperative deficits, (Chi square p = 0.004) and (p = 0.008) respectively. There was no significant differences between HRQoL measures between timepoints or between patients with deficits at any timepoint compared to patients without deficits. CONCLUSION Postoperative visual field deficits are related to preoperative tumour-effects rather than surgery. Patients can be counselled that while they do not recover following surgery, it is not associated with significant reductions in HRQoL. Patients with preoperative visual field deficits may be considered for resection without significant worry about the preservation of visual field function since these deficits do not recover postoperatively.

Assessing chiasm perfusion and postoperative visual function with superior hypophyseal artery indocyanine green angiograms during endoscopic endonasal surgery.

To date, there are no validated intraoperative tools to predict postoperative visual function following endoscopic endonasal surgery (EES). Assessment of post-surgical vision during surgery can help in postoperative planning and disposition and inform surgical decisions in real-time. The objective of this study was to evaluate the capability of intraoperative endoscopic indocyanine green (ICG) angiography to measure optic chiasm perfusion and determine its relationship with postoperative visual function. A retrospective review was performed on patients undergoing EES for sellar andsuprasellar lesions. ICG was injected prior to surgical closure at a time when the optic chiasm and anterior circulation were visible. Luminescence of the superior hypophyseal artery (SHA) branches enveloping the optic chiasm was registered 10seconds after ICG penetration into the anterior cerebral arteries (ACAs). Pre and post-operative visual acuity and field exams were used to assess visual function. Patients with and without new deficits were compared to examine statistical association with intraoperative ICG findings. Twenty patients were included (mean age 49years, 55% female). Eighteen patients displayed stable or improved vision after resectionand demonstrated luminescence of all chiasmatic branches within 10s of ACA fluorescence. Two patients experienced new postoperative visual deficits. Upon review, their ICG administrations showed no fluorescence of the SHA branches enveloping the chiasm. A lack of chiasm fluorescence within ten seconds of ACA fluorescence was associated with new postoperative vision deficits (p = 0.005). This study shows that the lack of ICG fluorescence of SHA branches at the chiasm correlates with ischemic changes and new post-operative bitemporal hemianopsia. Lack of chiasm fluorescence after 10seconds of observation reflects compromised chiasm perfusion and likely new post-surgical visual deficits.

Torsion-Induced Traumatic Optic Neuropathy (TITON): A Physiologically Relevant Animal Model of Traumatic Optic Neuropathy.

Traumatic optic neuropathy (TON) is a common cause of irreversible blindness following head injury. TON is characterized by axon damage in the optic nerve followed by retinal ganglion cell death in the days and weeks following injury. At present, no therapeutic or surgical approach has been found to offer any benefit beyond observation alone. This is due in part to the lack of translational animal models suitable for understanding mechanisms and evaluating candidate treatments. In this study, we developed a rat model of TON in which the eye is rapidly rotated, inflicting mechanical stress on the optic nerve and leading to significant visual deficits. These functional deficits were thoroughly characterized up to one week after injury using electrophysiology and immunohistochemistry. The photopic negative response (PhNR) of the light adapted full field electroretinogram (LA ffERG) was significantly altered following injury. This correlated with increased biomarkers of retinal stress, axon disruption, and ganglion cell death. Together, this evidence suggests the utility of our model for mimicking clinically relevant TON and that the PhNR may be an early diagnostic for TON. We also found indirect evidence that ketamine, which was used for anesthesia, may ameliorate TON. Future studies will utilize this animal model for evaluation of candidate treatments.

New Perspective of Multi-dimensional Approach for the Management of Attention-deficit Hyperactivity Disorder: A Review

One of the most common mental diseases in childhood, attention-deficit/hyperactivity disorder (ADHD) often lasts into adulthood for many individuals. The neurodevelopmental condition known as ADHD impacts three areas of the brain: hyperactivity, impulsivity, and attention. The visual field is where attention is most affected by ADHD. Non-strabismic binocular vision disorder (NSBVD), which impairs eye coordination and makes it challenging to focus, has been linked to ADHD. Through a critical cognitive process called visual attention, humans are able to take in and organize information from their visual environment. This greatly affects how one observes, processes, and understands visual information in day-to-day living. Vision therapy is a non-invasive therapeutic approach that aims to improve visual talents and address visual attention deficits. This study aims to provide an overview of the research on the many approaches to treating ADHD, the relationship between NSBVD and ADHD, and whether vision therapy is a viable treatment option for ADHD. After a comprehensive search of many online resources, relevant studies were found. The review's findings provide insight into the range of ADHD patients' treatment choices. In order to improve treatment outcomes, non-pharmacological treatments can be employed either alone or in conjunction with medicine. Medicine by itself is insufficient and has several severe side effects when used continuously. The efficacy of vision therapy in improving visual attention and making recommendations for potential directions for further research in this field. Multiple studies are needed to identify the most effective treatment modalities for achieving positive outcomes for ADHD patients.

Long-term sequelae and quality of life after childhood-onset craniopharyngioma: Results of a Spanish multicenter study.

We aim to describe the characteristics of patients with childhood-onset craniopharyngioma and to analyze factors that impair quality of life (QoL) in this population. Multicenter national study including patients treated between 2008 and 2022, from 2 to 25years of age diagnosed with craniopharyngioma. QoL was assessed once during patient's follow-up by age-adapted versions of Pediatric Quality of Life Inventory (PedsQL) questionnaire. Sixty-six patients were included. Median age at diagnosis was 5years (interquartile range [IQR]: 3-8), while median follow-up was 7.4years (IQR: 2.8-9.7). Most craniopharyngioma were suprasellar (93.9%), and 59.7% had hypothalamic involvement (HI). All patients underwent surgery, 44.4% received radiotherapy, and 23.6% intracystic therapy. Most frequent long-term complications were visual deficit (72.7%) and endocrine impairment (94.5%). Patients exhibited hypothyroidism requiring hormone replacement (92.4%), hypocortisolism (80.3%), diabetes insipidus (86.4%), and/or growth hormone therapy (50%). When parents evaluated QoL, PedsQL median score was 53.8 points out of 100 (IQR: 41-71.6). Higher scores were noted when patients assessed their own QoL (median score 64.8 [IQR: 57.3-81.8]), observing statistically significant differences (p=.019). QoL was impaired by repeated surgeries (r=-.44; p=.014), HI (median score 51.5 [IQR: 39-63.8] vs. 76.4 [59-84.8]; p=.001), radiotherapy (median score 51.9 [IQR: 38.1-61.3] vs. 63.8 [IQR: 49-82.5]; p=.02) and longer follow-up (r=-.3; p=.01). In our study, most patients had significant comorbidities and low overall QoL scores, which was mainly affected by repeated surgery, radiation, and hypothalamic involvement. This reflects the need for further research and intensified studies of systemic therapy/alternate strategies to broaden the standard-of-care options, so that treatment-related sequalae can be avoided.

Global Processing Deficit in Amnestic Mild Cognitive Impairment.

Visuo-perceptual and visuo-attentional disorders, such as global processing deficit and simultanagnosia, are not routinely investigated in prodromal forms of typical Alzheimer's disease, as amnestic mild cognitive impairment (MCI). This study evaluated global processing abilities through Navon's classical paradigm in individuals with amnestic MCI and investigated the related visuo-perceptual and attentional components involved in simultanagnosia. Sixteen consecutive patients with amnestic MCI (6 single-domain, 10 multiple-domain) and 16 matched controls were requested to identify global and local elements of hierarchical Navon letters, and to name large and small solid letters. While correctly identifying solid letters, patients with multiple-domain amnestic MCI were less accurate in processing the global level of hierarchical stimuli compared to controls. Single-case analyses suggested that global processing may also be impaired in single-domain amnestic MCI. In addition, patients with pathological performance in the Navon task showed perceptual and/or visual focal attention deficits. Early dysfunction of holistic processing can be detected in amnestic MCI. Visuo-perceptual and/or visual focal attention mechanisms, which have been shown to be damaged in Posterior Cortical Atrophy patients with simultanagnosia, may be impaired in individuals with amnestic MCI. Investigation and identification of global processing deficits in MCI could contribute to early diagnosis and longitudinal monitoring of the disease.

12562 Extraocular Muscle Enlargement With Proptosis In A Patient With Non Thyroidal Disease

Abstract Disclosure: S.S. Bantu: None. P.N. Kabir: None. A. Gondhi: None. T.B. Vaughan: None. Extraocular muscle enlargement with proptosis in a patient with Nonthyroidal disease Introduction: Majority of cases with extraocular muscle enlargement (EOME) is due to Graves’ disease, commonly in about 95% cases and another 5% make up other causes including inflammatory, neoplastic, vascular, infectious and acromegaly. We present a patient with sudden loss of vision, retroorbital pain with visual field deficits found to have EOME and subsequently a pituitary macroadenoma on imaging. Case: We present a 54-year-old male with history of hypertension, obstructive sleep apnea and morbid obesity who presented to us with complains of left sided vision loss. Patient was evaluated by ophthalmology and noted to have unilateral, left-sided, change in visual acuity. Physical exam was notable for macrognathia, skin tags, enlarged hands, excessive sweating. MRI brain revealed bilateral EOME with proptosis and a pituitary macroadenoma measuring 1.5x1.7x1.3cm, abutting the pre-chiasmatic left optic nerve. On biochemical evaluation he was noted to have elevated IGF1 of 979 confirming the diagnosis of acromegaly. ((hemoglobin A1C of 5.5%, prolactin 5.1, FT4 0.89, TSH 0.692, total testosterone 61, IGF1 level was 979)). He underwent transsphenoidal resection of the pituitary macroadenoma. Pathology showed sparsely granulated somatotroph adenoma. Discussion: Acromegaly has an incidence of 3-4 cases per million per year. The characteristics of the disease are due to chronic growth hormone hypersecretion resulting in the excessive growth of tissue due to the stimulation of insulin-like growth factor 1 (IGF-1). EOME with proptosis in acromegaly is seen rarely. Visual symptoms may manifest as visual field deficits due to compression of the optic pathway in 18-25% cases, increased intraocular pressure, increase in corneal thickness and a diffuse symmetrical enlargement of the extraocular muscles which cannot be distinguished from thyroid orbitopathy on imaging. The degree of enlargement appears to correlate with the duration of the disease rather than the levels of IGF1. EOME improves with the treatment of acromegaly. Although visual symptoms may be a rare presentation of acromegaly, if the clinical suspicion exists, one may consider obtaining an IGF-1 level to complete the evaluation of EOME if the thyroid levels are normal. Presentation: 6/3/2024

8617 Gender Differences In The Course Of Giant Prolactinomas - Single-Centre Case Series

Abstract Disclosure: M.A. Komisarz-Calik: None. A. Boguslawska: None. K. Ciszek: None. G. Zieliński: None. A. Hubalewska-Dydejczyk: None. A. Gilis-Januszewska: None. Introduction: Giant prolactinomas are the specific subtype of lactotroph PitNET, and are classified, according to their maximum diameter (≥40 mm). They constitute about 3% of prolactinomas. The prevalence is more common in men, however, the differences in the course of the disease between the genders remain unclear. Aim: The purpose of our study was to analyse gender differences in the course of giant prolactinomas. Materials and Methods: We analysed the data of consecutive patients with giant prolactinomas treated in the tertiary clinical center in the years 2022-2023. Results: There were 12 patients with giant prolactinoma (10 men and 2 women).Men and women were of similar age (32 vs 36 years). The maximum dimensions of the pituitary tumour in men were 56x70x67 mm, in women 60x55x55 mm. In all cases, treatment included dopamine agonists (cabergoline in 11 patients (all male patients and one female patient) and bromocriptine in one woman. Combined treatment with temozolomide and pasireotide were implemented in two male patients. In three men (30%) a surgical treatment was performed due to the visual field disturbances and severe headaches. Multiple surgeries were performed in two patients due to recurrence of symptoms and tumour progression (surgery was performed twice, and three times). One male patient received radiotherapy.The most frequent symptoms were associated with insufficiency of the adrenal, thyroid, and gonadal axis (80% vs 50%), visual field deficits (50% vs 50%) and headache (80% vs 0%), respectively in men and women. In men, the most common symptom was hypofunction of the gonadal axis (8 cases - 4 isolated gonadal axis hypofunction and 4 cases - hypofunction of the adrenal, thyroid and gonadal axis). In one woman, hypofunction of the three pituitary axes was present. Serum prolactin was higher in men than in women (6576 - 284232 ulU/ml vs 2000 - 5659 ulU/ml). In total, after treatment, prolactin concentration decreased in 7 cases, 5 men (50%) and 2 women (100%). The decrease in prolactin levels in men was by 80,4-99,9%, while in women it was by 83,9-86,7%. Tumour shrinkage was observed in 7 cases - 6/10 men and 1/2 woman. In men, tumour size was reduced by 27,5-64,4%. Tumour progression was observed in three cases, in 1 case the dimensions were stable. In two cases due to a recent diagnosis, follow-up MRI has not been yet performed. In women, tumour reduction by 99,8% was observed; in second the size of the tumour remained stable. Conclusions: The prevalence of giant prolactinomas is higher in men. The most frequent symptoms in both genders are visual field disturbances. The prevalence of tumour shrinkage is similar in both sexes, however giant prolactinomas require surgery more often in men. Further multicenter projects are needed to study the course of giant prolactinomas and to improve the outcomes of care. Presentation: 6/3/2024