Failed remyelination of the nonhuman primate optic nerve leads to axon degeneration, retinal damages, and visual dysfunction.

White matter disorders of the central nervous system (CNS), such as multiple sclerosis (MS), lead to failure of nerve conduction and long-lasting neurological disabilities affecting a variety of sensory and motor systems, including vision. While most disease-modifying therapies target the immune and inflammatory response, the promotion of remyelination has become a new therapeutic avenue to prevent neuronal degeneration and promote recovery. Most of these strategies have been developed in short-lived rodent models of demyelination, which spontaneously repair and do not reflect the size, organization, and biology of the human CNS. Thus, well-defined nonhuman primate models are required to efficiently advance therapeutic approaches for patients. Here, we followed the consequence of long-term toxin-induced demyelination of the macaque optic nerve on remyelination and axon preservation, as well as its impact on visual functions. Findings from oculomotor behavior, ophthalmic examination, electrophysiology, and retinal imaging indicate visual impairment involving the optic nerve and retina. These visual dysfunctions fully correlated at the anatomical level, with sustained optic nerve demyelination, axonal degeneration, and alterations of the inner retinal layers. This nonhuman primate model of chronic optic nerve demyelination associated with axonal degeneration and visual dysfunction, recapitulates several key features of MS lesions and should be instrumental in providing the missing link to translate emerging repair promyelinating/neuroprotective therapies to the clinic for myelin disorders, such as MS.

Visual symptoms in postural tachycardia syndrome: An investigation of position-dependent visual exploration.

BACKGROUND AND PURPOSE: Patients with postural tachycardia syndrome report position-dependent visual symptoms. Despite their impact on daily life, these symptoms have remained largely unexplored in research. The aim of this study was to investigate the nature of visual symptoms in postural tachycardia syndrome and possible underlying pathophysiological mechanisms. METHODS: Fifteen patients with postural tachycardia syndrome and 15 healthy controls were included in the study. Through a comprehensive array of measurements, including haemodynamics, subjective symptom assessments, eye movement tracking and pupil diameter analysis, participants were assessed during free image exploration in both supine and 60° head-up tilt positions. RESULTS: During head-up tilt, patients showed a decreased number and duration of fixations, as well as a decreased number, peak velocity and amplitude of saccades compared to the supine position and the control group. This reduction in visual exploration occurred primarily in the peripheral field of view and coincided with the occurrence of subjective visual symptoms. No significant differences in the saccade main sequence were observed between the two groups in either body position. CONCLUSIONS: Patients with postural tachycardia syndrome have a reduced exploration of the peripheral field of view when in an upright body position, potentially leading to tunnel vision. Since the normality of the saccade main sequence in patients combined with the focus on the centre of the field of view and the lower saccade amplitudes points to an intact brainstem function, the decrease in peripheral visual exploration may be attributed to a position-dependent dysfunction of the frontal eye field.

Inhibition of complement C3 signaling ameliorates locomotor and visual dysfunction in autoimmune inflammatory diseases.

Neuromyelitis optica (NMO) is an autoimmune inflammatory disease of the central nervous system (CNS) characterized by transverse myelitis and optic neuritis. The pathogenic serum IgG antibody against the aquaporin-4 (AQP4) on astrocytes triggers the activation of the complement cascade, causing astrocyte injury, followed by oligodendrocyte injury, demyelination, and neuronal loss. Complement C3 is positioned as a central player that relays upstream initiation signals to activate downstream effectors, potentially stimulating and amplifying host immune and inflammatory responses. However, whether targeting the inhibition of C3 signaling could ameliorate tissue injury, locomotor defects, and visual impairments in NMO remains to be investigated. In this study, using the targeted C3 inhibitor CR2-Crry led to a significant decrease in complement deposition and demyelination in both slice cultures and focal intracerebral injection models. Moreover, the treatment downregulated the expression of inflammatory cytokines and improved motor dysfunction in a systemic NMO mouse model. Similarly, employing serotype 2/9 adeno-associated virus (AAV2/9) to induce permanent expression of CR2-Crry resulted in a reduction in visual dysfunction by attenuating NMO-like lesions. Our findings reveal the therapeutic value of inhibiting the complement C3 signaling pathway in NMO.

Temporal trends in paediatric hydrocephalus - Rising prematurity and persistent ophthalmological challenges.

AIM: To study changes in aetiology, prematurity, comorbidity and ophthalmological outcomes in children with surgically treated hydrocephalus to provide information needed to maintain the best possible healthcare for a fragile and changing population. METHODS: Two population-based cohorts, born two decades apart in Region Västra Götaland Sweden, surgically treated for hydrocephalus at Sahlgrenska University Hospital in Gothenburg were recruited at approximately 10 years of age. The participants were examined according to an ophthalmological protocol, including history taking regarding perceptual visual dysfunction (PVD). Gestational age, aetiology and comorbidities were registered. RESULTS: The 1989-1993 group, comprised 52 children (48% girls; mean age 10, range 7.7-12.8 years), was compared with 24 children, born in 2007-2012 (29% girls; mean age 10, range 7.0-13.8 years). Extreme prematurity (gestational age ≤ 28 weeks) increased over time (p = 0.001). The vast majority of the children showed ophthalmological abnormalities, although motility defects and nystagmus decreased in the latter population. Subnormal visual acuity was associated with extreme prematurity (RR = 4.69; p = 0.030), and PVD with learning disability (RR = 2.44; p = 0.032). CONCLUSION: Paediatric hydrocephalus populations may change with improved healthcare. Since a high percentage shows ophthalmological abnormalities and more children are born extremely preterm, the entirety needs consideration both neurologically and ophthalmologically.

Quality of life and functional vision in adolescents with surgically treated hydrocephalus in infancy.

AIM: To evaluate health- and vision-related quality of life (HR- and VR-QoL) and perceptual visual dysfunction (PVD) in adolescents with hydrocephalus surgically treated in infancy. METHODS: In total, 23 adolescents (15 males and 8 females; median age 14.9 years) with hydrocephalus and 31 controls were evaluated using validated instruments to measure HR-QoL and VR-QoL. PVDs were reported by history taking in five areas: recognition, orientation, depth, movement and simultaneous perception. RESULTS: Adolescents with hydrocephalus and the parent proxy reports showed lower mean total Paediatric Quality of Life Inventory 4.0 scores (75.8 and 63.7, respectively) compared with controls (87.6 and 91.5), p = 0.016 and p < 0.0001. Parent-reported scores were lower than self-reported scores (p = 0.001). Adolescents with myelomeningocele (n = 10) showed lower physical health scores (p = 0.001). No VR-QoL difference was found between groups. PVDs were reported in ≥1 area by 14/23 hydrocephalus participants and 2/31 controls (p < 0.0001). Associations were found in the hydrocephalus group between VR-QoL and HR-QoL (rs = 0.47, p = 0.026) and number of PVD areas (rs = -0.6, p = 0.003). CONCLUSION: Adolescents with hydrocephalus and their parents reported lower HR-QoL and more PVDs. These problems indicate the need for not only ophthalmological follow-ups but also evaluation of QoL and PVDs in individuals with infantile hydrocephalus.

A spectrum of cognitive-behavioral-movement disorders in adrenoleukodystrophy: A case series from a tertiary care centre in the eastern part of India.

Background: Adrenoleukodystrophy (ALD) is an intriguing disease with a heterogeneous clinico-radiological profile. Behavioral and cognitive impairments are often the initial and predominant manifestations, yet their patterns are frequently overlooked. This study aims to elaborate on the patterns of cognitive dysfunction, behavioral changes, and movement disorders in ALD to facilitate its earlier diagnosis. Methods: In this case series, 12 cases of ALD were assessed and evaluated for cognitive, behavioral, and movement abnormalities to identify patterns of involvement. Results: All patients were male, with an age range of 5-46 years. 75% presented with cerebral ALD (CALD), and 25% had an adrenomyeloneuropathy phenotype. Cognitive dysfunction, behavioral changes, and seizures were observed in 75%, 66.7%, and 33.3% of ALD patients. An initial posterior to anterior pattern of progression of cognitive impairment dominated by higher-order visual dysfunction and language regression was observed in 66.7% of CALD patients, while a frontal pattern was noted in 22.2% of CALD patients. While cognitive impairment typically indicated dysfunction of occipito-parieto-temporal networks, behavioral changes predominantly suggested dysfunctional fronto-temporal-subcortical connections. A novel observation was the occurrence of tics and stereotypies in 33.3% of ALD patients. Conclusion: This study describes the patterns of cognitive, behavioral, and movement abnormalities in ALD and highlights the contributory role of dysfunctional white matter networks. Cognitive patterns predominantly reflect a posterior-to-anterior gradient of impairment of white matter connections, while behavioral markers indicate involvement of fronto-temporal-subcortical networks. Adding to this spectrum, the occurrence of tics and stereotypies is a unique observation in ALD.

Prevalence of visual dysfunction and ocular motility disorders in developmentally delayed patients.

Objective: To evaluate the prevalence of reduced visual acuity (VA), refractive errors (RE), reduced contrast sensitivity and strabismus in developmentally delayed (DD) patients. Methods: This descriptive cross sectional study was carried out in Ophthalmology Departments of Mayo Hospital, Lahore, The Children's Hospital, Lahore and The Children's Hospital, Multan from June 2019 to December 2019. We recruited 257 patients of either gender, between the ages of 06-16 years having intelligence quotient (IQ) ≤ 80 by Wechsler Intelligence Scale for Children (WISC) from the out-patient departments. Detailed systemic and ophthalmic history was taken and through anterior and posterior segment examination was carried out. VA was assessed with age matched VA charts. Cycloplegic refraction with 1% cyclopentolate was carried out. Contrast sensitivity was measured with hiding Heidi charts. Strabismus was assessed with Hirschberg and covers /uncover tests. Results: The mean age of the patients was 8.88 years with standard deviation (SD) of ± 2.70. The prevalence of reduced VA, RE, strabismus and reduced contrast sensitivity in these children were 43.58%, 52.92%, 52.14% and 32.7% respectively. Out of these 52.92% RE, 56 (21.79%) were myopic, 66 (25.68%) were hyperopic and 14 (05.45%) were astigmatic. The percentage of esotropia was 72 (28.02%) and exotropia was 62 (24.12%). Conclusion: The results of our study in DD children have shown that a significant number of children have reduced VA, RE, strabismus and reduced contrast sensitivity. Apart from general management of DD children by a pediatrician, the ophthalmic management of these problems must be carried out by a pediatric ophthalmologist to improve their quality of life.

The clinical approach to the identification of higher-order visual dysfunction in neurodegenerative disease.

PURPOSE OF REVIEW: This review is intended to assist the reader in gaining the knowledge and skills necessary for the recognition and assessment of higher-order visual dysfunction due to neurodegenerative diseases including Alzheimer's disease, dementia with Lewy bodies, Parkinson's dementia, corticobasal degeneration, Creutzfeldt-Jakob disease, and the posterior cortical atrophy syndrome. Clinical problem-solving and pattern recognition must be developed and practiced to accurately diagnosis disturbances of higher-order visual function, and knowledge of higher-order visual brain regions and their visual syndromes forms the foundation for deciphering symptoms presented by patients and/or their care partners. Tests of higher-order visual dysfunction must be assembled by the clinician and assessment can take time and effort. The use of screening tests, follow-up visits, and formal neuropsychological referrals are critical components for accurate diagnosis and these principles are reviewed here. RECENT FINDINGS: A recent survey of neuro-ophthalmologists revealed that over half of the respondents report that 5-10% of their new patient referrals carry a diagnosis of neurodegenerative disease and many patients were referred for visual symptoms of unknown cause. Despite over a century of discovery related to higher-order visual functions of the human brain, translation of discovery to the clinical assessment of patients has been slow or absent. As with the approach to translational medicine in general, to see meaningful progress, an interdisciplinary approach is indispensable. The first step involves the application of discoveries from the field visual neuroscience by clinicians from the fields of ophthalmology, neurology, and neuropsychology, and from the disciplines of neuro-ophthalmology and behavioral neurology. The unmet need for recognition, assessment, and management of higher-order visual dysfunction in neurodegeneration is evident and clinicians can contribute to closing the gap by using the approach and the tools outlined in the review.

Electroencephalographic-Based Functional Connectivity Networks of Visual Hallucinations and Visuospatial Dysfunctions in Parkinson's Disease.

Visual dysfunction is an important nonmotor symptom of Parkinson's disease (PD). Visual hallucinations (VHs) and visuospatial dysfunctions (VSDs) are common visual dysfunctions in PD; however, the underlying mechanisms remain unclear. Our study aimed to evaluate neuronal synchronization between patients with PD with and without VH or VSD using electroencephalographic (EEG) coherence analysis. Twenty-four patients with sporadic PD were evaluated for the presence of VH and VSD, and were divided into VH-negative and VH-positive groups, and these groups were further subdivided by VSD status. Coherence analysis was performed on EEG data. Whole-brain and regional coherences were calculated and compared between the groups. There was a significant difference in frontal-frontal coherence between the VH+ VSD- and VH+ VSD+ groups (p = 0.026). Our findings suggest that reduced EEG coherence in frontal regions might be involved in VSD in patients with PD. Reduced neuronal synchronization between the frontal lobes may contribute to the disruption of visual processing in PD.

Ambient particulate matter exposure causes visual dysfunction and retinal neuronal degeneration.

PM2.5 pollution is related to neurotoxic and vascular effects in eye diseases such as glaucoma. This study investigates the adverse effects of PM2.5 exposure on visual function and retinal neurons. A versatile aerosol concentration enrichment system was used to expose mice to either control air or PM2.5 polluted air. Six months after PM2.5 exposure, visual function was measured by electroretinography (ERG). Hematoxylin and eosin staining and immunofluorescence staining were used for histopathological analysis. Protein markers of apoptosis, astrocytic reactivity, inflammatory cytokines, lipid peroxidation, protein nitration and DNA damage response were quantified with ELISA, western blot or detected using immunofluorescence and immunohistochemistry. After six months of exposure, PM2.5-exposed mice responded poorly to light stimuli compared with those exposed to the control air. PM2.5 exposure caused retinal thinning and reduction in the expression of retinal ganglion cell-selective marker RNA-binding protein with multiple splicing (RBPMS). Further, positive TUNEL staining was observed in the inner nucleus and outer nuclear layers of the retinae after exposure to PM2.5, which was accompanied by the activation of apoptosis signaling molecules p53, caspase-3 and Bax. PM2.5 induced the release of inflammatory cytokines including tumor necrosis factor-α and cleaved interleukin-1ß. Furthermore, increased levels of 8-OHdG and γ-H2AX in the mouse retinea were indicative of DNA single and double strand breaks by PM2.5 exposure, which activated PARP-1 mediated DNA damage and repair. In conclusion, this study demonstrates sub-chronic systemic exposure to concentrated PM2.5 causes visual dysfunction and retinal neuronal degeneration. DATA AVAILABILITY: The datasets during and/or analyzed during the current study available from the corresponding author on reasonable request.

Characteristics of Visual Evoked Potential in Different Parts of Visual Impairment. / 不同部位损伤致视力障碍的视觉诱发电位特征.

OBJECTIVES: To study the quantitative and qualitative differences of visual evoked potential (VEP) in monocular visual impairment after different parts of visual pathway injury. METHODS: A total of 91 subjects with monocular visual impairment caused by trauma were selected and divided into intraocular refractive media-injury group (eyeball injury group for short), optic nerve injury group, central nervous system injury and intracranial combined injury group according to the injury cause and anatomical segment. Pattern Reversal visual evoked potential (PR-VEP) P100 peak time and amplitude, Flash visual evoked potential (F-VEP) P2 peak time and amplitude were recorded respectively. SPSS 26.0 software was used to analyze the differences of quantitative (peak time and amplitude) and qualitative indexes (spatial frequency sweep-VEP acuity threshold, and abnormal waveform category and frequency) of the four groups. RESULTS: Compared with healthy eyes, the PR-VEP P100 waveforms of the intraocular eyeball injury group and the F-VEP P2 waveforms of the optic nerve group showed significant differences in prolonged peak time and decreased amplitude in injured eyes (P<0.05). The PR-VEP amplitudes of healthy eyes were lower than those of injured eyes at multiple spatial frequencies in central nervous system injury group and intracranial combined injury group (P<0.05).The amplitude of PR-VEP in patients with visual impairment involving central injury was lower than that in patients with eye injury at multiple spatial frequencies. The frequency of VEP P waveforms reaching the threshold of the intraocular injury group and the optic nerve injury group were siginificantly different from the intracranial combined injury group, respectively(P<0.008 3), and the frequency of abnormal reduction of VEP amplitude of threshold were significantly different from the central nervous system injury group, respectively(P<0.008 3). CONCLUSIONS: VEP can distinguish central injury from peripheral injury, eyeball injury from nerve injury in peripheral injury, but cannot distinguish simple intracranial injury from complex injury, which provides basic data and basis for further research on the location of visual impairment injury.

Self-reported visual dysfunction in Parkinson disease: the Survey of Health, Ageing and Retirement in Europe.

BACKGROUND AND PURPOSE: Visual dysfunction is a non-motor symptom of Parkinson disease (PD), but its prevalence is unknown as population-based data on the epidemiology of visual symptoms in PD are lacking. The objective was to determine the prevalence of visual dysfunction in PD. METHODS: This was a cross-sectional analysis of data from adults ≥50 years old in the Survey of Health, Ageing and Retirement in Europe (SHARE), a multinational population-based health survey of adults living in one of 27 European countries and Israel. PD diagnosis was self-reported. Impairment in overall, distance or near eyesight was defined as a score of 4 or 5 on a 1-5 scale. Adjusted logistic regression was used to determine the association between PD and self-reported vision. RESULTS: There were 115 240 age-eligible participants in the SHARE study (mean age 64.3 years, 54% female), of whom 1438 (1.25%) reported a diagnosis of PD. In adjusted logistic regression models, PD was associated with increased odds of impaired overall [odds ratio (OR) 2.67, 95% confidence interval (CI) 1.91-3.72], distance (OR 2.55, 95% CI 2.04-3.19) and near (OR 2.07, 95% CI 1.69-2.55) eyesight. Individuals with PD were also less likely to report having an eye examination within the previous 2 years (OR 0.59, 95% CI 0.38-0.92), but this did not remain statistically significant after adjusting for confounders (OR 0.76, 95% CI 0.47-1.24). CONCLUSIONS: Visual dysfunction is significantly more common in PD than in the general adult population. Visual symptoms are a potentially under-recognized and under-treated source of reduced quality of life in PD patients that require further attention and study.

Parafoveal thinning of inner retina is associated with visual dysfunction in Lewy body diseases.

BACKGROUND: Retinal optical coherence tomography findings in Lewy body diseases and their implications for visual outcomes remain controversial. We investigated whether region-specific thickness analysis of retinal layers could improve the detection of macular atrophy and unravel its association with visual disability in Parkinson's disease. METHODS: Patients with idiopathic Parkinson's disease (n = 63), dementia with Lewy bodies (n = 8), and E46K mutation carriers in the α-synuclein gene (E46K-SNCA) (n = 4) and 34 controls underwent Spectralis optical coherence tomography macular scans and a comprehensive battery of visual function and cognition tests. We computed mean retinal layer thicknesses of both eyes within 1-, 2-, 3-, and 6-mm diameter macular discs and in concentric parafoveal (1- to 2-mm, 2- to 3-mm, 1- to 3-mm) and perifoveal (3- to 6-mm) rings. Group differences in imaging parameters and their relationship with visual outcomes were analyzed. A multivariate logistic model was developed to predict visual impairment from optical coherence tomography measurements in Parkinson's disease, and cutoff values were determined with receiver operating characteristic analysis. RESULTS: When compared with controls, patients with dementia with Lewy bodies had significant thinning of the ganglion cell-inner plexiform layer complex within the central 3-mm disc mainly because of differences in 1- to 3-mm parafoveal thickness. This parameter was strongly correlated in patients, but not in controls, with low contrast visual acuity and visual cognition outcomes (P < .05, False Discovery Rate), achieving 88% of accuracy in predicting visual impairment in Parkinson's disease. CONCLUSION: Our findings support that parafoveal thinning of ganglion cell-inner plexiform complex is a sensitive and clinically relevant imaging biomarker for Lewy body diseases, specifically for Parkinson's disease. © 2019 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.

Sensory symptoms in Parkinson's disease: Clinical features, pathophysiology, and treatment.

Parkinson's disease (PD) is one of the most common forms of neurodegenerative disease in the elderly population and is typically manifested by motor symptoms and nonmotor symptoms and signs. Nonmotor symptoms, such as sensory symptoms, have been regarded as the significant features of this disease. These symptoms often occur in early stages of PD and influence quality of life. However, researchers suggest that the sensory symptoms of PD are frequently unrecognized by clinicians and remain untreated. The disorders include pain, olfactory disturbance, and visual dysfunction input on the underlying sensory abnormality. This Review focuses on the clinical features, pathophysiological mechanisms, and treatment strategies for sensory symptoms of PD from both clinical studies and basic research, providing a comprehensive overview of the sensory symptoms in PD. © 2016 Wiley Periodicals, Inc.

Delayed saccade to perceptually demanding locations in Parkinson's disease: analysis from the perspective of the speed-accuracy trade-off.

Parkinson's disease (PD) patients reportedly have shortened, normal, or prolonged latency of visually guided saccades (VGSs). This inconsistency seems to be partly derived from differences in experimental conditions, such as target eccentricity and direction. Another etiology may be a physiological saccade property, the speed-accuracy trade-off. VGS latency tends to increase along with its gain in certain conditions; however, this relationship has not been addressed in PD saccade studies. In this study, we measured VGS latency and gain in 47 PD patients and 48 normal controls (NCs). VGS was evoked by a target, which was presented at the central position initially and pseudo-randomly jumped to the horizontal (10° or 20° eccentricity) or vertical (10° or 15°) meridian. For each target location, the logarithm of the latency (log-latency) was modeled with subject type (PD or NC), age, and gain in the linear-mixed regression analysis. Subsequently, for target locations where PD patients showed an abnormality, the log-latency was similarly modeled with additional clinical variables measured by the mini-mental state examination (MMSE) and unified Parkinson's disease rating scale Part III. PD saccade latency was prolonged and influenced by the MMSE score when targets were presented at the 20° horizontal and upper vertical meridians. Furthermore, gain was a consistently significant variable in all models. The target locations of the delayed saccade corresponded to perceptually demanding locations, indicating that PD subclinical visual dysfunction prolonged the latency. The influence of the MMSE score supports this reasoning. Moreover, the speed-accuracy trade-off appeared to contribute to the accurate saccade analysis.

Oculo-visual changes and clinical considerations affecting older patients with dementia.

PURPOSE: Dementia is associated with various alterations of the eye and visual function. Over 60% of cases are attributable to Alzheimer's disease, a significant proportion of the remainder to vascular dementia or dementia with Lewy bodies, while frontotemporal dementia, and Parkinson's disease dementia are less common. This review describes the oculo-visual problems of these five dementias and the pathological changes which may explain these symptoms. It further discusses clinical considerations to help the clinician care for older patients affected by dementia. RECENT FINDINGS: Visual problems in dementia include loss of visual acuity, defects in colour vision and visual masking tests, changes in pupillary response to mydriatics, defects in fixation and smooth and saccadic eye movements, changes in contrast sensitivity function and visual evoked potentials, and disturbance of complex visual functions such as in reading ability, visuospatial function, and the naming and identification of objects. Pathological changes have also been reported affecting the crystalline lens, retina, optic nerve, and visual cortex. Clinically, issues such as cataract surgery, correcting the refractive error, quality of life, falls, visual impairment and eye care for dementia have been addressed. SUMMARY: Many visual changes occur across dementias, are controversial, often based on limited patient numbers, and no single feature can be regarded as diagnostic of any specific dementia. Nevertheless, visual hallucinations may be more characteristic of dementia with Lewy bodies and Parkinson's disease dementia than Alzheimer's disease or frontotemporal dementia. Differences in saccadic eye movement dysfunction may also help to distinguish Alzheimer's disease from frontotemporal dementia and Parkinson's disease dementia from dementia with Lewy bodies. Eye care professionals need to keep informed of the growing literature in vision/dementia, be attentive to signs and symptoms suggestive of cognitive impairment, and be able to adapt their practice and clinical interventions to best serve patients with dementia.

Carotid-ophthalmic aneurysms-Our results and treatment strategy.

OBJECTIVE: Ophthalmic aneurysms comprise 1.3-5% of all intracranial aneurysms and are the least likely to rupture. On the other hand, they can cause symptoms (visual dysfunction and eye movement palsy) in 18-35% of cases even when unruptured. In our article, we review all the cases of ophthalmic aneurysms treated in our department, discuss treatment methods, and compare our results with those reported in the literature. MATERIAL AND METHODS: In the period 1998-2010, we operated on 37 patients and treated 21 endovascularly. Out of these surgically/endovascularly treated, there were 11/7 asymptomatic, 3/2 unruptured symptomatic, and 23/12 ruptured casess. RESULTS: Surgically treated: Asymptomatic aneurysms were operated on with 9% (1 patient) mortality (due to delayed infection); 87% of patients with ruptured aneurysms improved, 9% remained unchanged, and 4% got worse. Visual dysfunction was restored in 66% of unruptured symptomatic cases (2 patients operated on within 1 month of the start of symptoms). Endovascularly treated. Asymptomatic aneurysms were coiled with 9% (1 patient) mortality and 9% (1 patient) morbidity. Patients with ruptured aneurysms improved in 50% of cases, 25% of patients did not change, 1 patient got worse (12.5%), and 1 patient died (12.5%). Only 1 of 3 coiled patients showed improved 3rd nerve palsy after coiling of an unruptured aneurysm. CONCLUSION: The mortality and morbidity of aneurysms treated in our department, both unruptured and ruptured, were relatively low and comparable with the results presented in the literature. The number of unruptured aneurysms with visual dysfunction was not as high as reported in the literature, but our results correlate with the main conclusion of those studies: to treat those aneurysms surgically when possible and within three months from the start of symptoms.

Photoreceptor layer thinning in idiopathic Parkinson's disease.

This study was undertaken to quantify retinal and intra-retinal layer thicknesses in Parkinson's disease (PD), and to evaluate whether retinal structural changes may be related to altered discrimination of color vision and to severity and duration of PD disease. We examined 97 PD patients and 32 healthy controls (HC) with spectral-domain optical coherence tomography (OCT), including intra-retinal layer segmentation. In total, we compared 111 retinal nerve fiber layer (RNFL)-scans and 114 macula scans from 68 PD patients with 62 RNFL and 63 macula scans from 32 HC. For clinical evaluation of disease severity, we used the Unified Parkinson's Disease Rating Scale (UPDRS) motor examination. To determine color discrimination, we performed the Farnsworth Munsell 100 Hue Test (FMT) in a subgroup of PD patients. We found significant combined outer nuclear and photoreceptor layer thinning in PD versus HC (118.6 vs. 123.5 µm, P = 0.001). Differences in RNFL, total macular volume, or the other retinal layer thicknesses were not detected. The OCT measures were not associated with disease severity, duration, or color vision. By showing photoreceptor cell layer thinning, our findings support previous in vivo and autopsy studies demonstrating retinal alterations in PD. Optical coherence tomography may help to assess morphological retinal changes in PD patients; however, the utility of OCT in routine clinical practice may be limited because many PD patients have difficulties complying with OCT investigation because of disease-related symptoms such as tremor, axial rigidity, or cognitive impairment.

Endoscopic Endonasal Approach for Optic Canal Dural Metastasis in a Patient with Progressive Visual Dysfunction: A Case Report.

To improve optic nerve function in a patient with progressive visual dysfunction, performing early decompressive and debulking surgery for a metastatic tumor located in the optic canal is essential. The endoscopic endonasal approach could be a practical and effective alternative for lesions in the inferomedial part of the optic canal. A 66-year-old man with a right visual eye field deficit had multiple lesions in the pineal gland, occipital lobe, and right inferomedial optic canal. The optic nerve was distorted by a tumor compressing against the falciform ligament. Although a systemic examination suggested the presence of primary lung cancer, the patient only complained of progressive visual impairment in the right eye. We planned surgery with endoscopic transethmoidal and transsphenoidal approaches to restore visual function and make a pathological diagnosis. During the procedure, we drilled the sella floor, tuberculum sellae, and optic canal and successfully removed the tumor underneath the dura mater. The patient's visual function improved rapidly following surgery, and no complications were observed, such as cerebrospinal fluid leakage. After confirming the pathological diagnosis, the patient subsequently received whole-brain radiotherapy. The endoscopic endonasal skull base approach to the optic canal region could be a practical alternative for treating symptomatic metastatic tumors.

Longitudinal follow-up of vision in a neuromyelitis optica cohort.

BACKGROUND: Neuromyelitis optica (NMO) is an inflammatory disease associated with optic neuritis and myelitis. Recently, several studies showed that optical coherence tomography (OCT) could be an interesting method for the evaluation of disease severity; however, to date there are no studies with a longitudinal follow-up of visual function in NMO. The aim of this study was to assess the ability of OCT to evaluate the progression of visual dysfunction in NMO. PATIENTS AND METHODS: A group of 30 NMO patients (thus, 60 eyes), comprised of 20 women and 10 men with a mean age of 43.7 +/- 12.3 years, were prospectively evaluated clinically and by a whole neuro-ophthalmological work-up, including: visual acuity (VA), fundoscopy, visual evoked potential (VEP), visual field (VF) and optical coherence tomography (OCT). All patients were tested at baseline (after a mean disease duration of 6.1 years) and after a mean time of follow-up of 18 months (range: 12-36 months). RESULTS: Mean VA was similar at the two evaluation times (0.77 +/- 0.36 versus 0.77 +/- 0.35). The mean VF defect decreased slightly, but the difference was not significant (-5.9 +/- 1.3 dB versus -5.3 +/- 1.3 dB). In contrast, the mean retinal thickness seen on OCT decreased from 87.4 +/- 23.3 µm to 79.7 +/- 22.4 µm (p = 0.006). These modifications were only observed in eyes with a past or a recent history of optic neuritis (-15.1 µm; p < 0.001) and not in eyes without any history of optic neuritis (-2.4 µm; not significant). Also, they occurred independently of the occurrence of relapses (n = 13) and especially optic neuritis episodes; however, the number of optic neuritis episodes was low (n = 5). CONCLUSION: OCT seems to be a more sensitive test than VA or VF for monitoring ophthalmological function in NMO and it seems to be helpful for the detection of infra-clinical episodes in patients with a past history of optic neuritis. Our results suggest that this easily performed technique should be used in the follow-up of NMO, but complementary studies are warranted to confirm its interest at an individual level.