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Abstract:MSA patients feature significant reduction in nasal RNFLT and global mean deviation when compared to healthy controls, consistent with the multi-systemic nature of this neurodegenerative disorder. This finding provides first evidence for two independent deteriorations of the visual system in MSA.
“…Our results confirm that the macular ganglion cells (GCC) and their axons, that is, the RNFL are affected in patients with MSA . Our cross‐sectional study showed that patients with MSA had a significant reduction in the global as well as in the inferior RNFL quadrant of the peripapillary retina.…”
Section: Discussionsupporting
confidence: 82%
“…Indeed, in patients with MSA, a rare fatal adult‐onset synucleinopathy, preliminary imaging studies have identified a significant reduction in retinal nerve fiber layer (RNFL) thickness . This finding appeared unexpectedly because patients with MSA have no clinical visual complaints.…”
Background
Objective measures of disease progression that can be used as end-points in clinical trials of multiple system atrophy are necessary. We studied retinal thickness in patients with multiple system atrophy, and assessed changes over time to determine its usefulness as imaging biomarker of disease progression.
Methods
Cross sectional study including 24 patients with multiple system atrophy, 20 patients with Parkinson disease (PD) and 35 controls; followed by a longitudinal study of 13 multiple system atrophy (MSA) patients. Patients were evaluated with high definition-optical coherence tomography and the Unified Multiple System Atrophy Rating Scale. Evaluations were performed at baseline and at consecutive follow-up visits for up to 26 months.
Results
MSA subjects had normal visual acuity and color discrimination. Compared to controls, retinal nerve fiber layer (p=0.008 and p=0.001) and ganglion cell complex (p=0.013 and p=0.001) thicknesses were reduced in multiple system atrophy and in PD. No significant differences between MSA and PD were found. Over time, in patients with MSA, there was a significant reduction of the retinal nerve fiber layer and ganglion cell complex thicknesses, with estimated annual average losses of 3.7 μm and 1.8 μm respectively.
Conclusions
Visually asymptomatic MSA patients exhibit progressive reductions in the thickness of the retinal nerve fiber layer and, to a lesser extent, in the macular ganglion cell complex, which can be quantified by high-definition optical coherence tomography. Specific patterns of retinal nerve fiber damage could be a useful imaging biomarker of disease progression in future clinical trials.
“…Our results confirm that the macular ganglion cells (GCC) and their axons, that is, the RNFL are affected in patients with MSA . Our cross‐sectional study showed that patients with MSA had a significant reduction in the global as well as in the inferior RNFL quadrant of the peripapillary retina.…”
Section: Discussionsupporting
confidence: 82%
“…Indeed, in patients with MSA, a rare fatal adult‐onset synucleinopathy, preliminary imaging studies have identified a significant reduction in retinal nerve fiber layer (RNFL) thickness . This finding appeared unexpectedly because patients with MSA have no clinical visual complaints.…”
Background
Objective measures of disease progression that can be used as end-points in clinical trials of multiple system atrophy are necessary. We studied retinal thickness in patients with multiple system atrophy, and assessed changes over time to determine its usefulness as imaging biomarker of disease progression.
Methods
Cross sectional study including 24 patients with multiple system atrophy, 20 patients with Parkinson disease (PD) and 35 controls; followed by a longitudinal study of 13 multiple system atrophy (MSA) patients. Patients were evaluated with high definition-optical coherence tomography and the Unified Multiple System Atrophy Rating Scale. Evaluations were performed at baseline and at consecutive follow-up visits for up to 26 months.
Results
MSA subjects had normal visual acuity and color discrimination. Compared to controls, retinal nerve fiber layer (p=0.008 and p=0.001) and ganglion cell complex (p=0.013 and p=0.001) thicknesses were reduced in multiple system atrophy and in PD. No significant differences between MSA and PD were found. Over time, in patients with MSA, there was a significant reduction of the retinal nerve fiber layer and ganglion cell complex thicknesses, with estimated annual average losses of 3.7 μm and 1.8 μm respectively.
Conclusions
Visually asymptomatic MSA patients exhibit progressive reductions in the thickness of the retinal nerve fiber layer and, to a lesser extent, in the macular ganglion cell complex, which can be quantified by high-definition optical coherence tomography. Specific patterns of retinal nerve fiber damage could be a useful imaging biomarker of disease progression in future clinical trials.
“…Another study in 2013 evaluated 12 patients with MSA and 10 age-matched healthy controls (54). The study did not find differences in foveal thickness or global RNFL.…”
Section: Resultsmentioning
confidence: 99%
“…In this regard, the acquisition protocol of macular measurements (e.g., image resolution, number and dimension of slices, analyzed areas from those slices, layers analyzed, and segmentation methods) varied considerably among studies, and in some studies certain macular measurements were not reported.The statistical analysis of the OCT results was markedly different: most studies averaged the results of both eyes (51, 54–56); two considered the results of each eye as independent values (50, 52), whereas one study used only the results of the right eye (53). …”
Section: Resultsmentioning
confidence: 99%
“…The statistical analysis of the OCT results was markedly different: most studies averaged the results of both eyes (51, 54–56); two considered the results of each eye as independent values (50, 52), whereas one study used only the results of the right eye (53). …”
BackgroundMultiple system atrophy (MSA) is a rare, adult-onset, rapidly progressive fatal synucleinopathy that primarily affects oligodendroglial cells in the brain. Patients with MSA only rarely have visual complaints, but recent studies of the retina using optical coherence tomography (OCT) showed atrophy of the peripapillary retinal nerve fiber layer (RNFL) and to a lesser extent the macular ganglion cell layer (GCL) complex.MethodsWe performed a literature review and meta-analysis according to the preferred reporting items for systematic reviews and meta-analyses guidelines for studies published before January 2017, identified through PubMed and Google Scholar databases, which reported OCT-related outcomes in patients with MSA and controls. A random-effects model was constructed.ResultsThe meta-analysis search strategy yielded 15 articles of which 7 met the inclusion criteria. The pooled difference in the average thickness of the RNFL was −5.48 μm (95% CI, −6.23 to −4.73; p < 0.0001), indicating significant thinning in patients with MSA. The pooled results showed significant thinning in all the specific RNFL quadrants, except in the temporal RNFL quadrant, where the thickness in MSA and controls was similar [pooled difference of 1.11 µm (95% CI, −4.03 to 6.26; p = 0.67)]. This pattern of retinal damage suggests that MSA patients have preferential loss of retinal ganglion cells projecting to the magnocellular pathway (M-cells), which are mainly located in the peripheral retina and are not essential for visual acuity. Visual acuity, on the other hand, relies mostly on macular ganglion cells projecting to the parvocellular pathway (P-cells) through the temporal portion of the RNFL, which are relatively spared in MSA patients.ConclusionThe retinal damage in patients with MSA differs from that observed in patients with Parkinson disease (PD). Patients with MSA have more relative preservation of temporal sector of the RNFL and less severe atrophy of the macular GCL complex. We hypothesize that in patients with MSA there is predominant damage of large myelinated optic nerve axons like those originating from the M-cells. These large axons may require higher support from oligodendrocytes. Conversely, in patients with PD, P-cells might be more affected.
Eye disorders spanning a range of ocular tissue are common in patients with movement disorders. Highlighting these ocular manifestations will benefit patients and may even aid in diagnosis. In this educational review we outline the anatomy and function of the ocular tissues with a focus on the tissues most affected in movement disorders. We review the movement disorders associated with ocular pathology and where possible explore the underlying cellular basis thought to be driving the pathology and provide a brief overview of ophthalmic investigations available to the neurologist. This review does not cover intracranial primary visual pathways, higher visual function, or the ocular motor system.
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