多发性硬化与视神经脊髓炎谱系疾病的OCT评价

目的利用OCT检测MS与NMOSD患者黄斑区神经节细胞复合体(ganglion cell complex,GCC)和视网膜神经纤维层(retinal nerve fiber layer,RNFL)厚度,对其所致的视神经及轴突损伤进行分析。方法采用回顾性对照研究方法。收集河南科技大学第一附属医院治疗的MS患者30例为MS组,NMOSD患者32例为NMOSD组,同期健康受试者30例为对照组。采用OCT检测GCC(上、下象限和平均)和RNFL(鼻、颞、上、下4个象限及平均)的厚度,并进行比较分析。结果 NMOSD组和MS组GCC厚度(上、下象限和平均)及RNFL(4个象限和平均)厚度均显著低于对照组(P0.05)。NMOSD组上、下象限及平均GCC厚度低于MS组(P0.05)。NMOSD组上方RNFL厚度低于MS组(P0.05),但鼻、颞及下方象限RNFL厚度与MS组差异无统计学意义(P0.05)。结论 MS和NMOSD患者均存在明显的视神经及轴突损伤,但NMOSD患者损伤更为明显。     

Regularity changes of the retinal nerve fiber layer and macular ganglion cell complex in patients with the amnestic mild cognitive impairment

Purpose/aim: We investigated the regularity changes of the retinal nerve fiber layer (RNFL) and macular ganglion cell complex (mGCC) of the amnestic mild cognitive impairment (aMCI) patients in this prospective cohort study.

Materials and methods: Twenty-four aMCI patients and 30 health controls, who are more than 60 years old, were recruited into the study. The RNFL and the mGCC average thickness were measured with Fourier-domain optical coherence tomography (FD-OCT).

Results: Compared with that in the controls, the intraocular pressure (IOP) was significantly lower in the aMCI patients. A significant decrease in RNFL thickness in superior temporal, temporal upper (TU), and temporal upper and lower (TL) (TU+TL) quadrants was found in the aMCI patients than in the controls. The average thickness of the mGCC was also significantly thinner in the aMCI patients than in the controls.

Conclusions: Retinal degeneration in the aMCI patients detected by OCT together with lower IOP may indicate disease pathological progression.     

Benign Multiple Sclerosis is Associated with Reduced Thinning of the Retinal Nerve Fiber and Ganglion Cell Layers in Non-Optic-Neuritis Eyes

Background and Purpose

It is exceedingly difficult to differentiate benign multiple sclerosis (BMS) from relapsing-remitting multiple sclerosis (RRMS) based on clinical characteristics, neuroimaging, and cerebrospinal fluid tests. Optical coherence tomography (OCT) allows quantification of retinal structures, such as the retinal nerve fiber layer (RNFL) thickness, at the optic disc and the ganglion cell layer (GCL) at the macula, on a micrometer scale. It can also be used to trace minor alterations and the progression of neurodegeneration, help predict BMS, and influence the choice of therapy. To utilize OCT to detect the extent of changes of the optic disk and macular microstructure in patients with BMS and RRMS compared to healthy controls (HCs), with special focus on changes related to the presence/absence of optic neuritis (ON).

Methods

Spectral-domain OCT was applied to examine eyes from 36 patients with multiple sclerosis (MS), comprising 11 with BMS and 25 with RRMS, and 34 HCs.

Results

The RNFL and GCL were significantly thinner in eyes previously affected by ON, irrespective of the type of MS (i.e., BMS or RRMS), than in HCs. Significant thinning of the GCL was also observed in non-ON RRMS (and not non-ON BMS) compared to HCs. Correspondingly, a significant association between disease duration and thinning rates of the RNFL and GCL was observed only in non-ON RRMS (-0.54±0.24 and -0.43±0.21 µm/year, mean±SE; p<0.05 for both), and not in non-ON BMS (-0.11±0.27 and -0.24±0.24 µm/year).

Conclusions

The RNFL and GCL were thinner in both ON- and non-ON MS, but the change was more pronounced in ON MS, irrespective of the MS subtype studied herein. GCL thinning and the thinning rate of both the GCL and RNFL were less pronounced in non-ON BMS than in non-ON RRMS. These findings may help to predict the course of BMS.     

Eyes are mirror of the brain: comparison of multiple sclerosis patients and healthy controls using OCT

Abstract

Objective: To evaluate the thickness of choroid and retinal nerve fiber layer (RNFL) in multiple sclerosis (MS) patients with and without optic neuritis using enhanced depth imaging optical coherence tomography (EDI-OCT).

Methods: In this cross-sectional study, both eyes of 52?MS patients [n?=?104 eyes; 62 eyes of MS patients without optic neuritis (MS-NON) and 42 eyes of MS patients with optic neuritis (MS-ON)] and only one eye of 36 healthy control subjects (n?=?36 eyes) were evaluated. Complete ophthalmologic examination and EDI-OCT scanning were completed for all participants. Choroidal thickness measurements were executed at three different points.

Results: Choroidal thickness measurements were similar between MS patients and healthy control subjects. However, the mean subfoveal choroidal thickness was increased significantly in MS-ON group (399.13?±?82.91?μm) compared to MS-NON group (342.71?±?82.46?μm; p?=?0.004). Mean RNFL thickness was significantly reduced in MS patients (90.42?±?13.31?μm) compared to healthy controls (101.18?±?10.75?μm; p?<?0.001). Moreover, temporal RNFL thickness was significantly thinner in MS-ON group (54?±?14.50?μm) than MS-NON group (62.15?±?15.88?μm; p?=?0.01). In MS patients, temporal RNFL thickness was correlated with both Expanded Disability Status Score (r?=?0.383; p?<?0.001) and longer disease duration (r=–0.202; p?=?0.04).

Conclusion: The results of the present study suggest that RNFL thickness can be used as an important parameter while following up with MS patients. However, more studies using EDI-OCT are required with larger MS patient groups and automated method.     

Progressive cerebral atrophy in multiple system atrophy

Nine patients with multiple system atrophy (MSA) were studied based on MRI findings of cerebral hemispheric involvement. The age at onset was 56.4+/-8.6 (mean+/-S.D.) years, duration of illness at the first MRI study 2.1+/-1.1 years, duration of illness at the last study 9.7+/-2.6 years, and the follow-up duration 7.6+/-2.3 years. Controls were 85 neurologically intact persons (60.2+/-11.1 years age). In the MRI study, measurements of the ratio of each area to the intracranial area were performed for the cerebral hemisphere, frontal, temporal and parietal-occipital lobes. A significant progression of atrophy to under the normal limit was observed in the cerebrum, frontal and temporal lobes. Besides the typical pathological lesions in MSA, five autopsied patients revealed frontal lobe atrophy with mild gliosis, mild demyelination and glial cytoplasmic inclusions (GCIs). One of these patients showed remarkable frontal lobe atrophy with degenerative changes in the cerebral cortex. We observed the involvement of the cerebral hemisphere, especially the frontal lobe.     

光相干连续断层成像术在视神经脊髓炎谱系病的临床应用

目的探讨光相干连续断层成像术(OCT)在视神经脊髓炎谱系病(NMOSD)的临床应用。方法利用OCT对49例NMOSD患者(NMOSD组)和1 5例健康对照志愿者(对照组)视乳头周围视网膜神经纤维层(RNFL)厚度进行比较;NMOSD组再根据是否伴视神经炎、水通道蛋白抗体状态(AQP4-IgG)等对NMOSD组分为伴视神经炎(NMOSD-ON)亚组(34例);不伴视神经炎(NMOSD-NON)亚组(15例)。比较各亚组的RNFL厚度差异;采用扩展病残状态评分(EDSS)评价神经功能缺损程度,分析EDSS与RNFL是否存在相关性。结果 NMOSD患者受累眼的RNFL各象限厚度与未受累眼和对照组比较显著变薄(P0.01);NMOSD未受累眼与对照组比较,RNFL厚度差异无显著性(P0.05)。AQP4-IgG阳性或阴性NMOSD-ON亚组的RNFL厚度比较差异无显著性(P0.05);RNFL厚度变化与NMOSD残障的严重程度无相关性(P0.05)。结论 RNFL变薄仅在NMOSD受累眼表现,厚度变化与AQP4-IgG无关,OCT可能有助于临床对NMOSD诊疗提供参考指标。     

Survival in multiple system atrophy

We here report survival in patients with multiple system atrophy (MSA) in a large, prospectively studied group of patients with MSA. Eighty‐five of 100 patients were known to have died. Three patients were rediagnosed as having PD. Twenty‐four patients came to autopsy, which showed MSA in 22 and idiopathic Parkinson's disease in 2. The median survival time was 8.6 and 7.3 years for men and women, respectively (hazard ratio for women was 1.49, 95% CI 0.97–2.31, P = 0.07). Except for rediagnosis as PD, no predictive factors for better survival could be identified. These data confirm the relatively poor prognosis of MSA of less than 9 years on average. © 2007 Movement Disorder Society     

视神经脊髓炎与复发缓解型多发性硬化患者视网膜神经纤维层厚度的对比研究

目的检测视神经脊髓炎（NMO）与复发缓解型多发性硬化（RRMS）患者的视神经纤维层（RNFL）厚度,从而分析NMO与RRMS的轴索损伤情况。方法采用光学相干断层成像（OCT）技术,检测NMO和RRMS患者的RNFL的厚度,并进行统计学分析。结果视神经受累的NMO与RRMS患者,其平均RNFL的厚度分别为：（71．41±22．88）,（88．38±12．16）μm;其中以颞侧象限L（51．94±9．29）,（69．50±18．02）μml和下象限[（90．59±40．77）,（113．94±16．64）μml的RNFL减少更明显,差异具有统计学意义（P〈0．01）。结论NMO患者的平均RNFL厚度较RRMS薄,提示NMO患者的轴索损伤较RRMS为重,并且以颞侧象限和下侧象限的轴索损伤为明显。     

The retinal ganglion cell layer predicts normal‐appearing white matter tract integrity in multiple sclerosis: A combined diffusion tensor imaging and optical coherence tomography approach

We investigated the relationship between retinal layers and normal‐appearing white matter (WM) integrity in the brain of patients with relapsing‐remitting multiple sclerosis (MS), using a combined diffusion tensor imaging and high resolution optical coherence tomography approach. Fifty patients and 62 controls were recruited. The patients were divided into two groups according to presence (n = 18) or absence (n = 32) of optic neuritis. Diffusion tensor data were analyzed with a voxel‐wise whole brain analysis of diffusion metrics in WM with tract‐based spatial statistics. Thickness measurements were obtained for each individual retinal layer. Partial correlation and multivariate regression analyses were performed, assessing the association between individual retinal layers and diffusion metrics across all groups. Region‐based analysis was performed, by focusing on tracts associated with the visual system. Receiver operating characteristic (ROC) curves were computed to compare the biomarker potential for the diagnosis of MS, using the thickness of each retinal layer and diffusion metrics. In patients without optic neuritis, both ganglion cell layer (GCL) and inner plexiform layer thickness correlated with the diffusion metrics within and outside the visual system. GCL thickness was a significant predictor of diffusion metrics in the whole WM skeleton, unlike other layers. No association was observed for either controls or patients with a history of optic neuritis. ROC analysis showed that the biomarker potential for the diagnosis of MS based on the GCL was high when compared to other layers. We conclude that GCL integrity is a predictor of whole‐brain WM disruption in MS patients without optic neuritis.     

Proposed neuroimaging criteria for the diagnosis of multiple system atrophy

In this article, we review the state of the art knowledge concerning structural and functional imaging in multiple system atrophy (MSA). The relative value of imaging modalities in the differential diagnosis of MSA from other parkinsonian syndromes is debated. It is concluded that, although neuroimaging biomarkers provide valuable supportive data alongside clinical assessments, it is not possible to use them as surrogate markers. © 2009 Movement Disorder Society     

Age-dependent retinal neuroaxonal degeneration in children and adolescents with Leber hereditary optic neuropathy under idebenone therapy

Background

The aim of this study was to investigate the neuroretinal structure of young patients with Leber hereditary optic neuropathy (LHON).

Methods

For this retrospective cross-sectional analysis, the peripapillary retinal nerve fiber layer (pRNFL) thickness and the macular retinal layer volumes were measured by optical coherence tomography. Patients aged 12 years or younger at disease onset were assigned to the childhood-onset (ChO) group and those aged 13–16 years to the early teenage-onset (eTO) group. All patients received treatment with idebenone. The same measurements were repeated in age-matched control groups with healthy subjects.

Results

The ChO group included 11 patients (21 eyes) and the eTO group 14 patients (27 eyes). Mean age at onset was 8.6 ± 2.7 years in the ChO group and 14.8 ± 1.0 years in the eTO group. Mean best-corrected visual acuity was 0.65 ± 0.52 logMAR in the ChO group and 1.60 ± 0. 51 logMAR in the eTO group (p < 0.001). Reduced pRNFL was evident in the eTO group compared to the ChO group (46.0 ± 12.7 μm vs. 56.0 ± 14.5 μm, p = 0.015). Additionally, a significantly lower combined ganglion cell and inner plexiform layer volume was found in the eTO compared to the ChO group (0.266 ± 0.0027 mm³ vs. 0.294 ± 0.033 mm³, p = 0.003). No difference in these parameters was evident between the age-matched control groups.

Conclusion

Less neuroaxonal tissue degeneration was observed in ChO LHON than in eTO LHON, a finding that may explain the better functional outcome of ChO LHON.     

多系统萎缩与帕金森病的早期鉴别诊断

目的　为多系统萎缩 (m ultiple system atrophy,MSA)与帕金森病 (Parkinson disease,PD)的鉴别诊断提供依据。方法　对 12名首发锥体外系症状的 MSA患者和 40名 PD患者的临床症状及主要辅助检查行比较分析。MSA诊断依据 Quinn(1994)提出的临床诊断标准。PD诊断参考英国帕金森病协会 1997年提出的诊断标准。结果　与 PD患者比较 ,MSA患者发病年龄较小 (5 4.3 2± 9.5 0 vs 61.5 6± 9.5 5 ,P<0 .0 5 ) ,主要以行动困难或僵硬为首发症状 (66.7% vs 2 5 % ,P<0 .0 5 ) ;少数以震颤为首发症状 (3 3 .3 % vs 75 % ,P<0 .0 5 ) ,也可以单侧症状发病(5 8.3 % vs 80 % ,P>0 .0 5 ) ,多数对多巴胺反应不良 (66.7% vs 2 0 % ,P<0 .0 5 ) ,植物神经症状、语言障碍比 PD患者显著多见 (分别为 83 .3 % vs 2 5 % ;5 0 % vs12 .5 % ;P<0 .0 5 )。辅助检查 :MRI:大部分 MSA患者有阳性表现 ,橄榄体、脑桥和小脑部位有萎缩 ,PD患者未发现特征性病变。 PET:3例 MSA患者均呈阳性表现。神经心理 :MSA患者未出现智能障碍的情况 (0 % ) ,少数 PD(10 % )患者出现智能障碍 ,但两者比较无统计学意义 (P>0 .0 5 )。结论　依据详细的病史和全面的神经系统查体 ,并结合临床辅助检查 ,可提高 MSA和 PD早期鉴别诊断的准确性     

Analysis of the retinal nerve fiber and ganglion cell – Inner plexiform layer by optical coherence tomography in Parkinson’s patients

AimTo measure and evaluate the thickness of the retinal nerve fiber layer (RNFL) and ganglion cell-inner plexiform layer (GC-IPL) in patients with Parkinson’s disease using optical coherence tomography (OCT).Methods58 eyes of 30 patients with Parkinson’s disease and 60 eyes of 30 healthy individuals were enrolled to this study according to defined criteria. RNFL thickness, central macular thickness (CMT) and ganglion cell-inner plexiform layer (GC-IPL) thickness were measured in these groups. The Parkinson’s patient group was also subjected to Unified Parkinson’s Disease Rating Scale (UPDRS) and Mini Mental Status Exam (MMSE).ResultsNo difference was found between the two groups with respect to age, sex and the best corrected visual acuity (BCVA). Mean, superior, and inferior quadrant RNFL values in the Parkinson’s patients were found statistically significantly lower than those in the control group (P < 0.001, P < 0.049, P < 0.001, respectively). While CMT was statistically similar between the groups, GC-IPL thickness was statistically significantly lower in Parkinson’s patients (p = 0.028). There was no significant correlation between the duration of Parkinson’s disease and RNFL thickness. While there was not any correlation between UPDRS total and motor scores and superior and temporal quadrant RNFL thicknesses, a significant negative correlation was established between RNFL nasal, inferior quadrant and RNFL mean thicknesses (P = 0.022; P = 0.035; P = 0.002, respectively). A significant positive correlation was found between MMSE and nasal and mean RNFL thicknesses (P = 0.046; P = 0.019, respectively).ConclusionRNFL and GC-IPL thicknesses were found lower in Parkinson’s patients. These parameters may be useful to evaluate neurodegeneration and to monitorize neuroprotective therapies.     

Functional imaging with positron emission tomography in multiple system atrophy

Summary. Although the current guidelines for the clinical diagnosis of multiple system atrophy (MSA) do not require structural or functional brain imaging, investigations utilizing positron emission tomography (PET) have been helpful diagnostically in differentiating between MSA and primary autonomic failure; idiopathic Parkinson’s disease; and sporadic olivopontocerebellar atrophy. These investigations have demonstrated different patterns of cerebral glucose utilization and of nigrostriatal projection abnormalities among these disorders and between the cerebellar and parkinsonian forms of MSA. Most of the studies have focused upon patients with well-established disease and none have examined the utility of PET imaging in early stage patients with follow-up of clinical course and autopsy verification to ensure accuracy of diagnosis and to determine the sensitivity and specificity of PET techniques for diagnosis. Recent PET studies have revealed denervation of myocardial post-ganglionic sympathetic neurons in some MSA patients, indicating that this disorder can affect the peripheral autonomic as well as the central nervous system. Investigations utilizing ligands to quantify central nervous system dopaminergic and cholinergic terminals have begun to provide insight into the neurochemical disorders that may underlie two of the sleep disturbances common in MSA, rapid eye movement sleep behavior disorder and obstructive sleep apnea.     

Subcortical atrophy and perfusion patterns in Parkinson disease and multiple system atrophy

BackgroundThe clinical differentiation between Parkinson disease (PD) and multiple system atrophy (MSA) is difficult.ObjectivesArterial spin labeling (ASL) is an advanced MRI technique that obviates the use of an exogenous contrast agent for the estimation of cerebral perfusion. We explored the value of ASL in combination with structural MRI for the differentiation between PD and MSA.MethodsNinety-four subjects (30 PD, 30 MSA and 34 healthy controls) performed a morphometric and ASL-MRI to measure volume and perfusion values within basal ganglia and cerebellum. A region-of-interest analysis was performed to test for structural atrophy and regional blood flow differences between groups.ResultsMSA patients showed higher subcortical atrophy than both PD patients and HC, while no differences were observed between the latter. MSA and PD showed lower volume-corrected perfusion values than HC in several cerebellar areas (Crus I, Crus II, right VIIb, right VIIIa, right VIIIb), right caudate and both thalami. MSA and PD patients displayed similar perfusion values in all aforementioned areas, but the right cerebellar area VIIIb (lower in MSA) and right caudate and both thalami (lower in PD). Similar results were obtained when comparing PD and MSA patients with the parkinsonian variant.ConclusionsA perfusion reduction was equally observed in both MSA and PD patients in cerebellar areas that are putatively linked to cognitive (i.e., executive) rather than motor functions. The observed hypo-perfusion could not be explained by atrophy, suggesting the involvement of the cerebellum in the pathophysiology of both MSA and PD.