Functional Magnetic Resonance Imaging (MRI) and MRI Tractography in Progressive Supranuclear Palsy-Like Syndrome.
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ABSTRACT:
SUBMITTER: Vaphiades MS
PROVIDER: S-EPMC5123098 | biostudies-literature | 2015 Apr
REPOSITORIES: biostudies-literature
Publications
Functional Magnetic Resonance Imaging (MRI) and MRI Tractography in Progressive Supranuclear Palsy-Like Syndrome.
Vaphiades Michael S MS Visscher Kristina K Rucker Janet C JC Vattoth Surjith S Roberson Glenn H GH
Neuro-ophthalmology (Aeolus Press) 20150401 2
An 18-year-old woman underwent an uneventful ascending aortic aneurysm repair then developed progressive supranuclear palsy-like syndrome. Extensive neuroimaging including contrasted fat-suppressed cranial and orbital magnetic resonance imaging (MRI), MRI tractography, and functional MRI (fMRI) revealed no clear radiographic involvement except for a single tiny hypoechoic midbrain dot on the T2*-weighted gradient-echo imaging, which is not considered sufficient to account for the patient's defic ...[more]
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Project description:Progressive supranuclear palsy is a neurodegenerative disease characterized by the deposition of four-repeat tau in neuronal and glial lesions in the brainstem, cerebellar, subcortical and cortical brain regions. There are varying clinical presentations of progressive supranuclear palsy with different neuroimaging signatures, presumed to be due to different topographical distributions and burden of tau. The classic Richardson syndrome presentation is considered a subcortical variant, whilst progressive supranuclear palsy with predominant speech and language impairment is considered a cortical variant, although the pathological underpinnings of these variants are unclear. In this case-control study, we aimed to determine whether patterns of regional tau pathology differed between these variants and whether tau burden correlated with neuroimaging. Thirty-three neuropathologically confirmed progressive supranuclear palsy patients with either the Richardson syndrome (n = 17) or speech/language (n = 16) variant and ante-mortem magnetic resonance imaging were included. Tau lesion burden was semi-quantitatively graded in cerebellar, brainstem, subcortical and cortical regions and combined to form neuronal and glial tau scores. Regional magnetic resonance imaging volumes were converted to Z-scores using 33 age- and sex-matched controls. Diffusion tensor imaging metrics, including fractional anisotropy and mean diffusivity, were calculated. Tau burden and neuroimaging metrics were compared between groups and correlated using linear regression models. Neuronal and glial tau burden were higher in motor and superior frontal cortices in the speech/language variant. In the subcortical and brainstem regions, only the glial tau burden differed, with a higher burden in globus pallidus, subthalamic nucleus, substantia nigra and red nucleus in Richardson's syndrome. No differences were observed in the cerebellar dentate and striatum. Greater volume loss was observed in the motor cortex in the speech/language variant and in the subthalamic nucleus, red nucleus and midbrain in Richardson's syndrome. Fractional anisotropy was lower in the midbrain and superior cerebellar peduncle in Richardson's syndrome. Mean diffusivity was greater in the superior frontal cortex in the speech/language variant and midbrain in Richardson's syndrome. Neuronal tau burden showed associations with volume loss, lower fractional anisotropy and higher mean diffusivity in the superior frontal cortex, although these findings did not survive correction for multiple comparisons. Results suggest that a shift in the distribution of tau, particularly neuronal tau, within the progressive supranuclear palsy network of regions is driving different clinical presentations in progressive supranuclear palsy. The possibility of different disease epicentres in these clinical variants has potential implications for the use of imaging biomarkers in progressive supranuclear palsy.
| S-EPMC11040515 | biostudies-literature
Project description:BackgroundTwo recent, randomized, placebo-controlled phase II/III trials (clinicaltrials.gov: NCT01110720, NCT01049399) of davunetide and tideglusib in progressive supranuclear palsy (PSP) generated prospective, 1-year longitudinal datasets of high-resolution T1-weighted three-dimensional MRI.ObjectiveThe objective of this study was to develop a quantitative MRI disease progression measurement for clinical trials.MethodsThe authors performed a fully automated quantitative MRI analysis employing atlas-based volumetry and provide sample size calculations based on data collected in 99 PSP patients assigned to placebo in these trials. Based on individual volumes of 44 brain compartments and structures at baseline and 52 weeks of follow-up, means and standard deviations of annualized percentage volume changes were used to estimate standardized effect sizes and the required sample sizes per group for future 2-armed, placebo-controlled therapeutic trials.ResultsThe highest standardized effect sizes were found for midbrain, frontal lobes, and the third ventricle. Using the annualized percentage volume change of these structures to detect a 50% change in the 1-year progression (80% power, significance level 5%) required lower numbers of patients per group (third ventricle, n = 32; midbrain, n = 37; frontal lobe, n = 43) than the best clinical scale (PSP rating scale total score, n = 58). A combination of volume changes in these 3 structures reduced the number of required patients to only 20 and correlated best with the progression in the clinical scales.ConclusionsWe propose the 1-year change in the volumes of third ventricle, midbrain, and frontal lobe as combined imaging read-out for clinical trials in PSP that require the least number of patients for detecting efficacy to reduce brain atrophy. © 2017 International Parkinson and Movement Disorder Society.
| S-EPMC5808453 | biostudies-literature
Project description:Background: The regional distribution of the widespread cerebral morphological alterations in progressive supranuclear palsy (PSP) is considered to include segmental parts of the corpus callosum (CC). Objective: The study was designed to investigate the regional white matter (WM) of the CC by T1 weighted magnetic resonance imaging (T1w MRI) data combined with diffusion tensor imaging (DTI) data in PSP patients, differentiated in the variants Richardson syndrome and PSP-parkinsonism, and to compare them with Parkinson's Disease (PD) patients and healthy controls, in order to identify macro- and micro-structural alterations in vivo. Methods: MRI-based WM mapping was used to perform an operator-independent segmentation for the different CC segments in 66 PSP patients vs. 66 PD patients vs. 44 matched healthy controls. The segmentation was followed by both planimetric and texture analysis of the separated CC areas for the comparison of the three groups. Results were complemented by a DTI-based tract-of-interest analysis of the associated callosal tracts. Results: Significant alterations of the parameters entropy and homogeneity compared to controls were observed for PSP as well as for PD for the CC areas I, II, and III. The inhomogeneity in area II in the PSP cohort was the highest and differed significantly from PD. A combined score was defined as a potential marker for the different types of neurodegenerative parkinsonism; receiver operating characteristics (ROC) curves were calculated with areas under the curve values of 0.86 for PSP vs. controls, 0.72 for PD vs. controls, and 0.69 for PSP vs. PD, respectively. Conclusion: The multiparametric MRI texture and DTI analysis demonstrated extensive alterations of the frontal CC in neurodegenerative parkinsonism, whereas regional CC atrophy cannot be regarded as a constant neuroimaging feature of PSP. Specifically, the comparison PSP vs. PD revealed significant alterations in callosal area II. The combination of the texture and the DTI parameters might contribute as a neuroimaging marker for the assessment of the CC in PSP, including the differentiation vs. PD.
| S-EPMC8640496 | biostudies-literature
Project description:ObjectiveThe current study sought to describe the functional profiles of patients with early-stage progressive supranuclear palsy (PSP) in a large prospective, multisite study.MethodsUsing data from 202 individuals meeting criteria for clinically definite or probable PSP, 3 functional scales were examined. Functional scores were then compared to measures of motor, cognition, and psychiatric symptoms.ResultsFunctional disability was high in early-stage PSP, with 100% of patients having less than perfect scores on all functional scales. Whereas functional scores tended not to be related to cognition or psychiatric symptoms, they were strongly related to motoric ratings.ConclusionsBoth clinically and in research settings, the definition of functional intactness/impairment has important implications. Future studies should examine if functional impairment is this high in PSP or if new scales of functional abilities need to be developed for this condition.
| S-EPMC3589239 | biostudies-literature
Project description:BackgroundPrevious studies have shown that magnetic susceptibility is increased in several subcortical regions in progressive supranuclear palsy (PSP). However, it is still unclear how subcortical and cortical susceptibilities vary across different PSP variants, Parkinson's disease (PD), and corticobasal syndrome (CBS).ObjectiveThis study aims to clarify the susceptibility profiles in the subcortical and cortical regions in different PSP variants, PD, and CBS.MethodsSixty-four patients, 20 PSP-Richardson syndrome (PSP-RS), 9 PSP-parkinsonism (PSP-P), 7 PSP-progressive gait freezing, 4 PSP-postural instability, 11 PD, and 13 CBS, and 20 cognitively normal control subjects underwent a 3-Tesla magnetic resonance imaging scan to reconstruct quantitative susceptibility maps. Region-of-interest analysis was performed to obtain susceptibility in several subcortical and cortical regions. Bayesian linear mixed effect models were used to estimate susceptibility within group and differences between groups.ResultsIn the subcortical regions, patients with PSP-RS and PSP-P showed greater susceptibility than control subjects in the pallidum, substantia nigra, red nucleus, and cerebellar dentate (P < 0.05). Patients with PSP-RS also showed greater susceptibility than patients with PSP-progressive gait freezing, PD, and CBS in the red nucleus and cerebellar dentate, and patients with PSP-P showed greater susceptibility than PD in the red nucleus. Patients with PSP-postural instability and CBS showed greater susceptibility than control subjects in the pallidum and substantia nigra. No significant differences were observed in any cortical region.ConclusionsThe PSP variants and CBS had different patterns of magnetic susceptibility in the subcortical regions. The findings will contribute to our understanding about iron profiles and pathophysiology of PSP and may provide a potential biomarker to differentiate PSP variants, PD, and CBS. © 2023 International Parkinson and Movement Disorder Society.
| S-EPMC10840892 | biostudies-literature
Project description:The clinical differential diagnosis between Parkinson's disease (PD) and progressive supranuclear palsy (PSP) is often challenging. The description of milder PSP phenotypes strongly resembling PD, such as PSP-Parkinsonism, further increased the diagnostic challenge and the need for reliable neuroimaging biomarkers to enhance the diagnostic certainty. This review aims to summarize the contribution of a relatively simple and widely available imaging technique such as MR planimetry in the differential diagnosis between PD and PSP, focusing on the recent advancements in this field. The development of accurate MR planimetric biomarkers, together with the implementation of automated algorithms, led to robust and objective measures for the differential diagnosis of PSP and PD at the individual level. Evidence from longitudinal studies also suggests a role of MR planimetry in predicting the development of the PSP clinical signs, allowing to identify PSP patients before they meet diagnostic criteria when their clinical phenotype can be indistinguishable from PD. Finally, promising evidence exists on the possible association between MR planimetric measures and the underlying pathology, with important implications for trials with new disease-modifying target therapies.
| S-EPMC9313181 | biostudies-literature
Project description:ImportanceThe accurate diagnosis of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) is hampered by imperfect clinical-pathological correlations.ObjectiveTo assess and compare the diagnostic value of the magnetic resonance parkinsonism index (MRPI) and other magnetic resonance imaging-based measures of cerebral atrophy to differentiate between PSP, CBD, and other neurodegenerative diseases.Design, setting, and participantsThis prospective diagnostic study included participants with 4-repeat tauopathies (4RT), PSP, CBD, other neurodegenerative diseases and available MRI who appeared in the University of California, San Francisco, Memory and Aging Center database. Data were collected from October 27, 1994, to September 29, 2019. Data were analyzed from March 1 to September 14, 2021.Main outcomes and measuresThe main outcome of this study was the neuropathological diagnosis of PSP or CBD. The clinical diagnosis at the time of the MRI acquisition was noted. The imaging measures included the MRPI, cortical thickness, subcortical volumes, including the midbrain, pons, and superior cerebellar peduncle volumes. Multinomial logistic regression models (MLRM) combining different cortical and subcortical regions were defined to discriminate between PSP, CBD, and other pathologies. The areas under the receiver operating characteristic curves (AUROC) and cutoffs were calculated to differentiate between PSP, CBD, and other diseases.ResultsOf the 326 included participants, 176 (54%) were male, and the mean (SD) age at MRI was 64.1 (8.0) years. The MRPI showed good diagnostic accuracy for the differentiation between PSP and all other pathologies (accuracy, 87%; AUROC, 0.90; 95% CI, 0.86-0.95) and between 4RT and other pathologies (accuracy, 80%; AUROC, 0.82; 95% CI, 0.76-0.87), but did not allow the discrimination of participants with CBD. Its diagnostic accuracy was lower in the subgroup of patients without the canonical PSP-Richardson syndrome (PSP-RS) or probable corticobasal syndrome (CBS) at MRI. MLRM combining cortical and subcortical measurements showed the highest accuracy for the differentiation between PSP and other pathologies (accuracy, 95%; AUROC, 0.98; 95% CI, 0.97-0.99), CBD and other pathologies (accuracy, 83%; AUROC, 0.86; 95% CI, 0.81-0.91), 4RT and other pathologies (accuracy, 89%; AUROC, 0.94; 95% CI, 0.92-0.97), and PSP and CBD (accuracy, 91%; AUROC, 0.95; 95% CI, 0.91-0.99), even in participants without PSP-RS or CBS at MRI.Conclusions and relevanceIn this study, the combination of widely available cortical and subcortical measures of atrophy on MRI discriminated between PSP, CBD, and other pathologies and could be used to support the diagnosis of 4RT in clinical practice.
| S-EPMC9055455 | biostudies-literature
Project description:BackgroundWaiting impulsivity in progressive supranuclear palsy-Richardson's syndrome (PSP-RS) is difficult to assess, and its regulation is known to involve nucleus accumbens (NAc) subregions. We investigated waiting impulsivity using the "jumping the gun" (JTG) sign, which is defined as premature initiation of clapping before the start signal in the three-clap test and compared clinical features of PSP-RS patients with and without the sign and analyzed neural connectivity and microstructural changes in NAc subregions.Materials and methodsA positive JTG sign was defined as the participant starting to clap before the start sign in the three-clap test. We classified participants into the JTG positive (JTG +) and JTG negative (JTG-) groups and compared their clinical features, microstructural changes, and connectivity between NAc subregions using diffusion tension imaging. The NAc was parcellated into core and shell subregions using data-driven connectivity-based methods.ResultsSeventy-seven patients with PSP-RS were recruited, and the JTG + group had worse frontal lobe battery (FAB) scores, more frequent falls, and more occurrence of the applause sign than the JTG- group. A logistic regression analysis revealed that FAB scores were associated with a positive JTG sign. The mean fiber density between the right NAc core and right medial orbitofrontal gyrus was higher in the JTG + group than the JTG- group.DiscussionWe show that the JTG sign is a surrogate marker of waiting impulsivity in PSP-RS patients. Our findings enrich the current literature by deepening our understanding of waiting impulsivity in PSP patients and introducing a novel method for its evaluation.
| S-EPMC10560850 | biostudies-literature
Project description:PurposeThe clinical presentation of idiopathic normal pressure hydrocephalus (iNPH) may overlap with progressive supranuclear palsy (PSP). The Magnetic Resonance Parkinsonism Index (MRPI), MRPI 2.0, and the interpeduncular angle (IPA) have been investigated to differentiate PSP from healthy controls (HC) and other parkinsonisms. We aimed to assess equivalences and differences in MRPI, MRPI 2.0, and IPA in iNPH, PSP, and HC groups.MethodsWe retrospectively recruited 99 subjects (30 iNPH, 32 PSP, 37 HC) from two institutions. MRI exams, acquired on either 1.5 T or 3 T scanners, included 3D T1-weighted images to measure MRPI, MRPI 2.0, and IPA. Inter- and intra-rater reliability was investigated with the intra-class correlation coefficient (ICC), and the two one-sided t tests (TOST) procedure was used to assess these markers in iNPH, PSP, and HC.ResultsFor all the three measures, intra-rater and inter-rater ICC were excellent (range = 0.91-0.93). In the comparison of iNPH and PSP with HC, differences for MRPI and MRPI 2.0 (p < 0.01 in all cases) and no equivalence (p = 1.00 in all cases) were found at TOST. iNPH and PSP MRPI showed no difference (p = 0.06) and no equivalence (p = 0.08). MRPI 2.0 was not equivalent (p = 0.06) and not different (p = 0.09) in the same two populations. PSP and HC IPA proved equivalent (p < 0.01) while iNPH IPA was different (p < 0.01) and not equivalent (p = 0.96 and 0.82) from both PSP and HC.ConclusionMRPI and MRPI 2.0 significantly overlap in iNPH and PSP, with risk of misdiagnosis, and for this reason may not be helpful in the differential diagnosis.
| S-EPMC7666671 | biostudies-literature
Project description:Currently [18F]FDDNP is the only PET imaging probe with the ability to visualize hyperphosphorylated tau fibrillar aggregates in living subjects. In this work, we evaluate in vivo [18F]FDDNP labeling of brain neuropathology, primarily tau fibrillar aggregates, in patients with progressive supranuclear palsy (PSP), a human tauopathy usually lacking amyloid-? deposits.Fifteen patients with PSP received [18F]FDDNP PET scanning. [18F]FDDNP distribution volume ratios, in reference to cerebellar gray matter, were determined for cortical and subcortical areas and compared with those of patients with Parkinson's disease with short disease duration, and age-matched control subjects without neurodegenerative disorders.[18F]FDDNP binding was present in subcortical areas (e.g., striatum, thalamus, subthalamic region, midbrain, and cerebellar white matter) regardless of disease severity, with progressive subcortical and cortical involvement as disease severity increased. Brain patterns of [18F]FDDNP binding were entirely consistent with the known pathology distribution for PSP. High midbrain and subthalamic region [18F]FDDNP binding was distinctive for PSP subjects and separated them from controls and patients with Parkinson's disease.These results provide evidence that [18F]FDDNP is a sensitive in vivo PET imaging probe to map and quantify the dynamic regional localization of tau fibrillar aggregates in PSP. Furthermore, [18F]FDDNP PET may provide a tool to detect changes in tau pathology distribution either associated with disease progression or as a treatment biomarker for future tau-specific therapies. Patterns of [18F]FDDNP binding may also be useful in diagnosis early in disease presentation when clinical distinction among neurodegenerative disorders is often difficult.
| S-EPMC3674205 | biostudies-literature