Visual aspects of neurologic protein misfolding disorders

Pula, John H.; Kim, Jisoon; Nichols, Jeffrey

doi:10.1097/icu.0b013e3283319899

Cited by 7 publications

(5 citation statements)

References 55 publications

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“…Aside from misfolding of Aβ peptides in AD, there are many other neurological disorders in which protein misfolding plays a crucial role is disease pathology, such as misfolding of α-synuclein in α-synucleinopathies such as PD, dementia with Lewy bodies and multiple system atrophy, and misfolding of tau in tauopathies such as AD, dementia pugilistica, progressive supranuclear palsy, among others [29–32]. These aggregating proteins all share similar biophysical and biochemical properties that influence how they misfold, aggregate, and propagate in disease [33].…”

Section: Discussionmentioning

confidence: 99%

Protective roles of intestinal microbiota derived short chain fatty acids in Alzheimer’s disease-type beta-amyloid neuropathological mechanisms

Ono

Tsuji

et al. 2017

Expert Review of Neurotherapeutics

272

183

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Background Dietary fibers are metabolized by gastrointestinal (GI) bacteria into short-chain fatty acids (SCFAs). We investigated the potential role of these SCFAs in β-amyloid (Aß) mediated pathological processes that play key roles in Alzheimer’s disease (AD) pathogenesis. Research design and methods Multiple complementary assays were used to investigate individual SCFAs for their dose-responsive effects in interfering with the assembly of Aß1-40 and Aß1-42 peptides into soluble neurotoxic Aß aggregates. Results We found that several select SCFAs are capable of potently inhibiting Aß aggregations, in vitro. Conclusion Our studies support the hypothesis that intestinal microbiota may help protect against AD, in part, by supporting the generation of select SCFAs, which interfere with the formation of toxic soluble Aß aggregates.

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Section: Discussionmentioning

confidence: 99%

Protective roles of intestinal microbiota derived short chain fatty acids in Alzheimer’s disease-type beta-amyloid neuropathological mechanisms

Ono

Tsuji

et al. 2017

Expert Review of Neurotherapeutics

272

183

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“…Visual event related potentials using a visual oddball paradigm were reported to be abnormal in all four diseases [13]. The often-observed blurry vision of PSP patients is usually attributed to the pronounced impairment of ocular movements and diplopia [14], but might also be associated with retinal changes. PSP and CBS have been reported to be associated with visuospatial deficits [15].…”

Section: Introductionmentioning

confidence: 98%

Optical Coherence Tomography in Parkinsonian Syndromes

et al. 2012

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Background/ObjectiveParkinson's disease (PD) and the atypical parkinsonian syndromes multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are movement disorders associated with degeneration of the central nervous system. Degeneration of the retina has not been systematically compared in these diseases.MethodsThis cross-sectional study used spectral-domain optical coherence tomography with manual segmentation to measure the peripapillar nerve fiber layer, the macular thickness, and the thickness of all retinal layers in foveal scans of 40 patients with PD, 19 with MSA, 10 with CBS, 15 with PSP, and 35 age- and sex-matched controls.ResultsThe mean paramacular thickness and volume were reduced in PSP while the mean RNFL did not differ significantly between groups. In PSP patients, the complex of retinal ganglion cell- and inner plexiform layer and the outer nuclear layer was reduced. In PD, the inner nuclear layer was thicker than in controls, MSA and PSP. Using the ratio between the outer nuclear layer and the outer plexiform layer with a cut-off at 3.1 and the additional constraint that the inner nuclear layer be under 46 µm, we were able to differentiate PSP from PD in our patient sample with a sensitivity of 96% and a specificity of 70%.ConclusionDifferent parkinsonian syndromes are associated with distinct changes in retinal morphology. These findings may serve to facilitate the differential diagnosis of parkinsonian syndromes and give insight into the degenerative processes of patients with atypical parkinsonian syndromes.

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“…The progressive and severe oculomotor dysfunction with the characteristic vertical gaze palsy is caused by central nervous dysfunction and was name-giving for the disease. Although impairment of visual acuity, color vision or contrast 1 , and diplopia 2 can also be found in patients with PD, visual disturbance in patients with PSP is much more profound and PSP patients frequently suffer from reading difficulties, indistinct vision, and diplopia, which are likely caused mainly by the progressive oculomotor dysfunction 3 . However, findings of visuospatial deficits 4 or detected abnormal visual event-related potentials in patients with PSP 5 suggest additional dysfunction of central processing mechanisms and potential changes of the retina as part of the pathology.…”

Section: Introductionmentioning

confidence: 99%

Retinal degeneration in progressive supranuclear palsy measured by optical coherence tomography and scanning laser polarimetry

Stemplewitz

Kromer

Vettorazzi

et al. 2017

Sci Rep

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This cross-sectional study compared the retinal morphology between patients with progressive supranuclear palsy (PSP) and healthy controls. (The retinal nerve fiber layer (RNFL) around the optic disc and the retina in the macular area of 22 PSP patients and 151 controls were investigated by spectral domain optical coherence tomography (SD-OCT). Additionally, the RNFL and the nerve fiber index (NFI) were measured by scanning laser polarimetry (SLP). Results of RNFL measurements with SD-OCT and SLP were compared to assess diagnostic discriminatory power. Applying OCT, PSP patients showed a smaller RNFL thickness in the inferior nasal and inferior temporal areas. The macular volume and the thickness of the majority of macular sectors were reduced compared to controls. SLP data showed a thinner RNFL thickness and an increase in the NFI in PSP patients. Sensitivity and specificity to discriminate PSP patients from controls were higher applying SLP than SD-OCT. Retinal changes did not correlate with disease duration or severity in any OCT or SLP measurement. PSP seems to be associated with reduced thickness and volume of the macula and reduction of the RNFL, independent of disease duration or severity. Retinal imaging with SD-OCT and SLP might become an additional tool in PSP diagnosis.

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Visual aspects of neurologic protein misfolding disorders

Cited by 7 publications

References 55 publications

Protective roles of intestinal microbiota derived short chain fatty acids in Alzheimer’s disease-type beta-amyloid neuropathological mechanisms

Protective roles of intestinal microbiota derived short chain fatty acids in Alzheimer’s disease-type beta-amyloid neuropathological mechanisms

Optical Coherence Tomography in Parkinsonian Syndromes

Retinal degeneration in progressive supranuclear palsy measured by optical coherence tomography and scanning laser polarimetry

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