Ganglion Cells In Patients Research Articles

Ramsay Hunt Syndrome with Numerous Cranial Nerve Damage Revealing An HIV Infection: A Case Report

Ramsay-Hunt syndrome corresponds to a Varicella zoster infection recurrence in the facial nerve’s sensory ganglion cells in patients with primary varicella infection. It manifests by coetaneous lesions of the sensitive Ramsay-Hunt zone, peripheral facial paralysis, and audiovestibular signs. The prognosis is usually good with well-conducted anti-viral treatment. We report the case of a 49-year-old lady who presented with several cranial nerve injuries, including impairment to cranial nerves V, VII, VIII, IX, and X, along with left facial paralysis, vesicles, discomfort in the left ear, dysphagia, and dysphonia. Her work-up in the search for an immune deficiency objected to an HIV infection. This case study aims to raise awareness among doctors to evaluate Ramsay Hunt syndrome in a patient with HIV who presents ear discomfort and insists on a diversified approach to patient care.

Anti-vascular endothelial growth factor drugs combined with laser photocoagulation maintain retinal ganglion cell integrity in patients with diabetic macular edema: study protocol for a prospective, non-randomized, controlled clinical trial.

The integrity of retinal ganglion cells is tightly associated with diabetic macular degeneration that leads to damage and death of retinal ganglion cells, affecting vision. The major clinical treatments for diabetic macular edema are anti-vascular endothelial growth factor drugs and laser photocoagulation. However, although the macular thickness can be normalized with each of these two therapies used alone, the vision does not improve in many patients. This might result from the incomplete recovery of retinal ganglion cell injury. Therefore, a prospective, non-randomized, controlled clinical trial was designed to investigate the effect of anti-vascular endothelial growth factor drugs combined with laser photocoagulation on the integrity of retinal ganglion cells in patients with diabetic macular edema and its relationship with vision recovery. In this trial, 150 patients with diabetic macular edema will be equally divided into three groups according to therapeutic methods, followed by treatment with anti-vascular endothelial growth factor drugs, laser photocoagulation therapy, and their combination. All patients will be followed up for 12 months. The primary outcome measure is retinal ganglion cell-inner plexiform layer thickness at 12 months after treatment. The secondary outcome measures include retinal ganglion cell-inner plexiform layer thickness before and 1, 3, 6, and 9 months after treatment, retinal nerve fiber layer thickness, best-corrected visual acuity, macular area thickness, and choroidal thickness before and 1, 3, 6, 9, and 12 months after treatment. Safety measure is the incidence of adverse events at 1, 3, 6, 9, and 12 months after treatment. The study protocol hopes to validate the better efficacy and safety of the combined treatment in patients with diabetic macula compared with the other two monotherapies alone during the 12-month follow-up period. The trial is designed to focus on clarifying the time-effect relationship between imaging measures related to the integrity of retinal ganglion cells and best-corrected visual acuity. The trial protocol was approved by the Medical Ethics Committee of the Affiliated Hospital of Beihua University with approval No. (2023)(26) on April 25, 2023, and was registered with the Chinese Clinical Trial Registry (registration number: ChiCTR2300072478, June 14, 2023, protocol version: 2.0).

Implementation of convolutional neural network for predicting glaucoma from fundus images

The contributions of this paper are two-fold. First, it uses machine learning tools to detect and monitor glaucoma. Second, it provides insight into medicine, which can assist healthcare professionals in improving disease diagnostic accuracy and help to reduce the progression and degeneration of retinal ganglion cells in patients. Glaucoma is a group of eye diseases that damage the optic nerve, which can cause vision loss and blindness at any age. The main symptom of glaucoma in the early stages is high internal eye pressure. It is still unknown what causes these diseases to develop, but if not treated, they result in optic nerve atrophy. For this reason, in this paper we propose a novel deep learning system for the automatic diagnosis of glaucoma using a convolutional neural network for classification, which demonstrates improved performance and records computation time for fundus images. The results showed an accuracy of 94 % and a loss value of only 0.27. The model we have created to investigate with Keras helped us achieve good results in our training and testing process. These study results demonstrate the ability of a deep learning model to identify glaucoma from fundus images. Increasing the filter size and training the model resulted in a higher accuracy rate. A population survey that was conducted in 2019 shows that most patients with glaucoma become aware of their disease late, after the disease causes a high level of optic nerve damage and a high percentage of vision loss. Early diagnosis and detection of glaucoma using optic nerve imaging technology have gained wide clinical interest in stopping or slowing the progression of the disease, allowing the development of new algorithms to automate the diagnosis of eye diseases

Optic Nerve Head and Retinal Abnormalities Associated with Congenital Fibrosis of the Extraocular Muscles.

Congenital fibrosis of the extraocular muscles (CFEOM) is a congenital cranial dysinnervation disorder caused by developmental abnormalities affecting cranial nerves/nuclei innervating the extraocular muscles. Autosomal dominant CFEOM arises from heterozygous missense mutations of KIF21A or TUBB3. Although spatiotemporal expression studies have shown KIF21A and TUBB3 expression in developing retinal ganglion cells, it is unclear whether dysinnervation extends beyond the oculomotor system. We aimed to investigate whether dysinnervation extends to the visual system by performing high-resolution optical coherence tomography (OCT) scans characterizing retinal ganglion cells within the optic nerve head and retina. Sixteen patients with CFEOM were screened for mutations in KIF21A, TUBB3, and TUBB2B. Six patients had apparent optic nerve hypoplasia. OCT showed neuro-retinal rim loss. Disc diameter, rim width, rim area, and peripapillary nerve fiber layer thickness were significantly reduced in CFEOM patients compared to controls (p < 0.005). Situs inversus of retinal vessels was seen in five patients. Our study provides evidence of structural optic nerve and retinal changes in CFEOM. We show for the first time that there are widespread retinal changes beyond the retinal ganglion cells in patients with CFEOM. This study shows that the phenotype in CFEOM extends beyond the motor nerves.

Alterations of Retinal Pigment Epithelium-Photoreceptor Complex in Patients with Type 2 Diabetes Mellitus without Diabetic Retinopathy: A Cross-Sectional Study.

Aim A cross-sectional study was performed to examine the alterations of the retinal pigment epithelium– (RPE–) photoreceptor complex layer in type 2 diabetes mellitus (DM) without diabetic retinopathy (DR), using spectral-domain optical coherence tomography (SD-OCT). Methods Patients with type 2 DM without DR and healthy controls without DM were recruited. All participants underwent examinations including SD-OCT. The thickness measurements of the retinal neural layers were calculated after automatic segmentation. An independent-sample t-test was used to compare the means of the thickness of retinal neural layers in patients with DM and healthy controls. Results Sixty-seven eyes from 67 patients with DM and 30 eyes from 30 healthy controls were included in this study. No significant differences were found in age (P = 0.601), gender (P = 0.601), gender (P = 0.601), gender (P = 0.601), gender (P = 0.601), gender (P = 0.601), gender (P = 0.601), gender (P = 0.601), gender (Conclusion Lesions in the RPE–photoreceptor complex are present without vascular abnormalities, which may precede the alterations of ganglion cells in patients with type 2 DM.

Disinhibition of intrinsic photosensitive retinal ganglion cells in patients with X-linked congenital stationary night blindness.

To assess the pupil light response (PLR) to chromatic stimulation in patients with different types of X-linked congenital stationary night blindness (CSNB). Eight patients with CSNB due to CACNA1F and NYX mutations were exposed to blue and red light stimuli, and PLR was evaluated using infrared video pupillography. Pupil responses were compared between CSNB patients and healthy subjects (n = 34) at baseline, at maximum of constriction, for post-illumination pupil responses (PIPR) and the slope of redilation using Cohen's d. A subgroup comparison was performed descriptively between CACNA1F and NYX associated CSNB patients using the same parameters. In CSNB, smaller baseline pupil diameters compared to healthy subjects were measured both before blue and red light stimulation (d = 1.44-1.625). The maximum constriction to blue light stimuli was smaller for the CSNB group compared to healthy subjects (d = 1.251) but not for red light stimuli (d = 0.449). Pupil response latencies were prolonged in CSNB for both light stimuli (d = -1.53 for blue and d = -1.011 for red stimulation). No relevant differences were found between the CSNB group and healthy subjects for PIPR (d = 0.01), but the slope of redilation was smaller for CSNB patients (d = 2.12). Paradoxical pupil constriction at light offset was not seen in our patients. A reduced redilation and smaller baseline pupil diameters for patients with CSNB indicate a disinhibition of intrinsically photosensitive retinal ganglion cells due to affected post-photoreceptor transduction via bipolar cells and can explain the pupillary behavior in our patient group.

Normal Visual Recovery after Optic Neuritis Despite Significant Loss of Retinal Ganglion Cells in Patients with Multiple Sclerosis.

Optic neuritis (ON) is a frequent manifestation of demyelinating attack in multiple sclerosis (MS). Initial visual loss can vary from minimal to complete. Visual improvement occurs in about 95% of patients, some of them recovering to normal [visual acuity (VA), color vision, visual field (VF)]. We analyzed retinal ganglion cell layer (RGCL) thickness in MS patients who recovered their normal vision after ON to determine whether a relative preservation of RGCL existed in these patients. We conducted a retrospective study of all patients with MS and ON examined by one of us (F. X. B.) between 2013 and 2018. Inclusion criteria were strictly unilateral ON, full recovery of vision, computerized visual field, and OCT examinations. Full recovery of vision was defined as VA ≥ 10/10, Ishihara ≥ 11/13, and VF mean defect (MD) ≤ 2.6 dB. Evaluation of RGCL was obtained with spectral domain optical coherence tomography (SD-OCT). The normal fellow eye of all patients served as the control group. Relative thinning of RGCL, expressed as percentage, was calculated by comparing results from the affected eye to the fellow eye of the same patient. Twenty-one patients (21 affected eyes - Group 1, 21 normal fellow eyes - Group 2) satisfying the inclusion criteria were retrieved from our database. All patients exhibited the relapsing-remitting form of MS. There were 16 women and 5men. Mean age was 39.3 years old. There were no statistically significant differences between Group 1 and Group 2 for either VA (p = 0.3934) or Ishihara (p = 0.140), but a significant difference was found for VF MD (p = 0.0405). A markedly significant difference for RGCL thickness (p = 0.0001) was found, without any correlation with the degree of visual recovery. A subgroup of patients (n = 14) was examined at the time of initial visual loss. We correlated their results of visual function to the final RGCL thickness, and a correlation was found between either the initial VA loss or the initial VF loss and the final loss of RGCL (R2 = 0.4075 and R2 = 0.00739, respectively). In our study, all patients with ON lost a significant amount of RGCL despite a full recovery of vision, as defined by our criteria. The percentage of RGCL loss varied from 5 - 27% and could not be correlated with any final visual indices. However, a correlation was found with the degree of initial visual loss. Despite sometimes marked RGCL loss after ON, patients with MS can recover normal visual function, according to standard clinical tests.

Identifying Glaucomatous Damage to the Macula.

SIGNIFICANCEMeasurements of the macula have been increasingly used to diagnose and manage patients with glaucoma. Asymmetry analysis was clinically introduced to assess damage to the macular ganglion cells in patients with glaucoma, but its effectiveness is limited by high normal between-subject variability.PURPOSEWe aimed to reduce the high normal between-subject variability and improve the potential of asymmetry analysis to identify glaucomatous damage to the macula.METHODSTwenty patients with glaucoma (aged 57 to 85 years) and 30 age-similar control subjects (aged 53 to 89 years) were recruited from a longitudinal glaucoma study. Participants were imaged with the Spectralis OCT using the posterior pole protocol; measurements of the averaged retinal thickness and ganglion cell layer (GCL) thickness were obtained. We established three zones per hemifield within the central ±9°, based on the lowest between-subject variability that we previously found and the course of retinal nerve fiber layer projections. The criteria for flagging abnormality were at least two contiguous zones when P < 5% or one zone when P < 1% with two-tailed tests.RESULTSBetween-subject variability of the asymmetry analysis for both retinal and GCL thicknesses remained lower than that of the average thickness across each zone in control subjects (F > 2.52, P < .01). Asymmetry analysis of retinal and GCL thicknesses flagged 16 and 18 of 20 patients, respectively.CONCLUSIONSBetween-subject variability was reduced in control subjects using the three zones; our criteria identified glaucomatous damage to the macula in most of the patients. We used high-density B-scans to confirm the patterns of the glaucomatous damage we found in this study.

Loss of Melanopsin-Expressing Retinal Ganglion Cells in Patients With Diabetic Retinopathy.

Photo-entrainment of the circadian clock is mediated by melanopsin-expressing retinal ganglion cells (mRGCs) located in the retina. Patients suffering from diabetic retinopathy (DR) show impairment of light regulated circadian activity such as sleep disorders, altered blood pressure, and abnormal melatonin secretion. The aim of this study was to assess the impact of DR on the expression of mRGCs in the human retina. The expression of mRGCs and RGCs was determined in eye sections containing retinal tissue from patients with DR (n = 6) and respective age-matched controls (n = 8) using immunohistochemistry by costaining with antibodies against RNA binding protein with multiple splicing (RBPMS), which identified RGCs and melanopsin which identified the mRGCs. The expression of RGCs in the retina from patients with severe DR was significantly reduced to a density of 146 ± 76 cells/mm2 compared with controls at 1280 ± 249 cells/mm2. The density of mRGCs was also significantly reduced from 3.12 ± 0.54 cells/mm2 in controls to 0.72 ± 0.18 cells/mm2 in patients with DR, with significant loss of 73.5% and 81.9% in mRGC density in the ganglion cell layer (GCL) and inner nuclear layer (INL), respectively. Our findings show that DR affects the expression of mRGCs in the human retina and could explain the abnormal circadian activity observed in patients with DR.

Comparison of Pattern Electroretinography and Optical Coherence Tomography Parameters in Patients with Primary Open-Angle Glaucoma and Ocular Hypertension

Objectives:To investigate the correlation of visual field (VF), pattern electroretinography (PERG) and Fourier domain optical coherence tomography (FD-OCT) results in patients with ocular hypertension (OHT) and early primary open-angle glaucoma (POAG).Materials and Methods:The study included 72 eyes of 37 patients with early POAG, 76 eyes of 38 patients with OHT, and 60 eyes of 30 controls. All subjects underwent full ophthalmologic examination, VF assessment with 24-2 Humphrey standard automated perimetry (Swedish Interactive Thresholding Algorithm (SITA)-Standard), retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thickness measurement with FD-OCT, and PERG P50 and N95 wave latency and amplitude measurements with electroretinography (Nihon Kohden).Results:With the exception of the nasal quadrant, all GCC parameters and RNFL results were significantly lower in the POAG group compared to the OHT and control groups. There was no statistically significant difference between the OHT and control group. PERG amplitudes were lower in the POAG and OHT groups than in the control group. Reduction in N95 amplitude was greater than that of P50 amplitude. No difference was detected in PERG latencies among groups. GCC was significantly correlated with VF and RNFL in the POAG group. Conclusion:Significant thinning of the GCC and RNFL occurs in addition to VF pathologies in patients with early POAG, and these examinations should be concomitantly evaluated. During diagnostic assessment of patients with early POAG, GCC and RNFL analysis by FD-OCT are highly effective. GCC is as reliable as RNLF in the early diagnosis of glaucoma and there is a highly significant correlation between them. Dysfunction of ganglion cells in patients with OHT may be detected earlier using PERG amplitude analysis.

Importance of taurine in maintaining retinal function

AbstractTaurine depletion has been known to induce photoreceptor degeneration since the 70s. Recently, investigating the retinal toxicity of the antiepileptic drug, vigabatrin, we showed that taurine depletion causes retinal ganglion cell loss. Indeed, the primary site of vigabatrin‐induced injury appears to be located in retinal ganglion cells in patients. The taurine depletion was demonstrated in both vigabatrin‐treated animals and patients. In fact, an increase in intraocular pressure as in glaucoma could also induce a local retinal taurine depletion. Taurine supplementation was neuroprotective in 4 animal models with retinal ganglion cell loss. Taurine acts directly on retinal ganglion increasing their survival in pure cultures. Taurine also reduces their glutamate excitotoxicity in retinal explants. Therefore, taurine is required for the normal maintenance of photoreceptors but also for the maintenance of retinal ganglion cells. Taurine supplementation may therefore provide a novel neuroprotective strategy for retinal diseases like glaucoma.

Transneuronal Retrograde Degeneration of the Retinal Ganglion Cells in Patients with Cerebral Infarction

The objective of this study was to determine whether transneuronal retrograde degeneration (TRD) of the retinal ganglion cells (RGCs) could be detected by optical coherence tomography (OCT) in humans with lesions other than of the occipital lobe or visual cortex. In addition, whether laterality and severity of retinal nerve fiber layer (RNFL) damage correlated with 3 other variables was determined: laterality of hemispheric damage, arterial territory of infarct, and age of infarct. Cross-sectional, case-control design. Forty-six patients with cerebral ischemic infarction diagnosed based on brain magnetic resonance imaging and 46 normal controls were enrolled. All subjects underwent a complete ophthalmic examination including OCT. Cerebral infarction was categorized by arterial territory: anterior cerebral artery (ACA), middle cerebral artery (MCA), and posterior cerebral artery (PCA). Eyes on the same side of the infarction were referred to as ipsilateral eyes, and eyes on the opposite side of the infarction were referred as contralateral eyes. Retinal nerve fiber layer thickness. Average, superior, temporal, inferior, and nasal RNFL thicknesses were different significantly between patients with cerebral infarction and normal controls. The RNFL thicknesses were reduced significantly at the superior, inferior, and nasal quadrants in the contralateral eyes and at the superior, inferior, and temporal quadrants in the ipsilateral eyes. The RNFL thickness reduction was greater in patients with PCA infarction, followed by MCA and ACA infarction, respectively. Factors related to the average RNFL thickness were time after stroke onset and infarction territory based on both univariate (P = 0.027 and P = 0.046, respectively) and multivariate (P = 0.036 and P = 0.047, respectively) analysis. Retinal nerve fiber layer thickness was reduced in patients with cerebral infarction, providing evidence for TRD of the RGCs. Transneuronal retrograde degeneration was more pronounced in the nasal nerve fiber layer of the contralateral side and in the temporal nerve fiber layer of the ipsilateral side of cerebral damage. The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Anti-neuronal autoantibodies and neurological paraneoplastic syndromes.

Remote effects of cancer on the nervous system or paraneoplastic syndromes are diseases of unknown cause that occur exclusively or with increased frequency in patients with cancer. Several hypotheses have been used to explain the pathogenesis of these disorders. One hypothesis is that the tumour and the nervous system share antigens, so that an autoimmune response generated against the tumour causes the neurological disorder. The best evidence for this hypothesis is found in the Lambert-Eaton myasthenic syndrome which can be transferred to animals by injecting them with IgG from patients. The identification of autoantibodies against Purkinje cell cytoplasmic protein antigens in patients with paraneoplastic cerebellar degeneration, autoantibodies against neuronal nuclear antigens in patients with paraneoplastic syndromes associated with small cell lung cancer and autoantibodies against retinal ganglion cells in patients with small cell lung cancer and retinal degeneration supports this hypothesis. The possible role of these autoantibodies in the pathogenesis of paraneoplastic neurological disease is discussed.

Labyrinthine pathology of chronic renal failure patients treated with hemodialysis and kidney transplantation.

AbstractAn audiologic study of 290 hemodialysis and renal transplant patients revealed that in 43 of these patients significant hearing loss developed which could be directly attributed to the therapy of the kidney problem.The clinicopathologic findings in 16 temporal bones of eight chronic hemodialysis and renal transplant patients were presented. The five patients treated with 59 or less hemodialyses had no subjective hearing loss; on the other hand, the three patients receiving 264 or more hemodialyses and multiple transplants, complained of hearing and vestibular difficulties. The pathologic findings common to all 14 temporal bones of the seven patients who underwent transplantation were blue stained concretions in the stria vascularis and/or vestibular receptors. The cochlear changes noted ranged from mild loss of outer hair cells and spiral ganglion cells in patients with few hemodialyses and transplants to complete absence of the organ of Corti in patients receiving more than 264 hemodialyses and multiple transplants; thus, the severity of the clinical and histopathological temporal bone findings was directly proportional to the number of hemodialyses and transplants to which the patient had been subjected. This seems to suggest that numerous hemodialyses or recurrent kidney transplants can induce electrolytic, osmotic, biochemical, vascular and/or immunological changes in the inner ear which can lead to severe audioves‐tibular symptoms and pathology.