The Strengths and Obstacles in the Differential Diagnosis of Progressive Supranuclear Palsy—Parkinsonism Predominant (PSP-P) and Multiple System Atrophy (MSA) Using Magnetic Resonance Imaging (MRI) and Perfusion Single Photon Emission Computed Tomography (SPECT)

Alster, Piotr; Nieciecki, Michał; Migda, Bartosz; Kutyłowski, Michał; Madetko, Natalia; Duszyńska-Wąs, Karolina; Charzyńska, Ingeborga; Koziorowski, Dariusz; Królicki, Leszek; Friedman, Andrzej

doi:10.3390/diagnostics12020385

Cited by 12 publications

(14 citation statements)

References 29 publications

(51 reference statements)

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“…Second, the requirement of only one PSP diagnosis given by any healthcare professional increases the sensitivity of this study and may decrease specificity. In the clinic, let alone a database scenario, there is a known difficulty in differentiating between atypical parkinsonism, such as PSP, multiple system atrophy and corticobasal degeneration due to overlapping clinical manifestations [ 48 , 49 ]. Nonetheless, the external validity of our study population is supported by previous publications.…”

Section: Discussionmentioning

confidence: 99%

Epidemiology of Progressive Supranuclear Palsy: Real World Data from the Second Largest Health Plan in Israel

et al. 2022

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Progressive supranuclear palsy (PSP) is a rare and fatal neurodegenerative movement disorder and no disease modifying therapy (DMT) is currently available. This study aims to assess the epidemiology of PSP in Israel and to describe its clinical features. This retrospective analysis identified patients with PSP between 2000 and 2018 over the age of 40 years at first diagnosis (index date). We identified 209 patients with ≥1 diagnosis of PSP. Of those, 88 patients satisfied the inclusion criteria with a mean age at diagnosis of 72 years (SD = 8) and 53% were female. The 2018 prevalence and incidence rates were 5.3 and 1 per 100,000 persons, respectively. Median survival time was 4.9 years (95% CI 3.6–6.1) and median time from initial symptom to diagnosis was 4.2 years. The most common misdiagnoses were Parkinson’s disease, cognitive disorder and depression. The present study demonstrates that the clinic-epidemiological features of PSP in Israel are similar to PSP worldwide. In light of PSP’s rarity, investigation of PSP cohorts in different countries may create a proper platform for upcoming DMT trials.

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Section: Discussionmentioning

confidence: 99%

Epidemiology of Progressive Supranuclear Palsy: Real World Data from the Second Largest Health Plan in Israel

et al. 2022

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“…In this respect, it must be acknowledged that specific PSP subtypes may represent misdiagnoses due to similarities in their clinical presentation with other neurodegenerative diseases, such as Multiple System Atrophy (MSA), Parkinson's disease, and Dementia with Lewy bodies (39,40). For example, early diagnostic discrimination between PSP-P and the parkinsonian subtype of MSA is particularly challenging (9,41), an issue which may have influenced our results in relation to the PSP-P subtype in this study.…”

Section: Discussionmentioning

confidence: 99%

The Burden of Progressive Supranuclear Palsy on Patients, Caregivers, and Healthcare Systems by PSP Phenotype: A Cross-Sectional Study

et al. 2022

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Progressive supranuclear palsy (PSP) is a rare, relentlessly progressive, ultimately fatal neurodegenerative brain disease. The objective of this study was to assess the burden of PSP on patients, caregivers, and healthcare systems by PSP phenotype. Data were drawn from the Adelphi PSP Disease Specific Programme™, a cross-sectional study of neurologists and people living with PSP in the United States of America, France, Germany, Italy, Spain, and the United Kingdom. All people living with PSP with a reported phenotype were included. PSP phenotype was reported for 242 patients (mean age: 70.2 years, 58% male): PSP-Richardson's syndrome, n = 96; PSP-predominant Parkinsonism, n = 88; PSP-predominant corticobasal syndrome, n = 28; PSP-predominant speech/language disorder, n = 12; PSP-progressive gait freezing, n = 9; PSP-predominant frontal presentation, n = 9. Most patients reported impaired cognitive, motor, behavioral and ocular functionality; 67–100% of patients (across phenotypes) had moderate-to-severe disease at the time of data collection. Post-diagnosis, the majority were provided with a visual and/or mobility aid (55–100%, across phenotypes), and/or required home modification to facilitate their needs (55–78%, across phenotypes). Patients required multiple types of healthcare professionals for disease management (mean 3.6–4.4, across phenotypes), and the majority reported receiving care from at least one caregiver (mean 1.3–1.8, across phenotypes). There is a high burden on patients, caregivers, and healthcare systems across all PSP phenotypes. Although phenotypes manifest different symptoms and are associated with different diagnostic pathways, once diagnosed with PSP, patients typically receive similar care.

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“…Positron emission tomography (PET) [9] Measure amyloid pathology, tau pathology, a-Synuclein pathology, metabolic activity by measuring changes in the glucose consumption PD-related spatial covariance pattern may involve increased pallidothalamic and pontine activity associated with decreased metabolism in supplementary motor area, premotor cortex, and parietal association areas Single photon emission computed tomography (SPECT) [12] Measure dopamine transporter (DAT) density, dopamine D2 receptor, metabolic activity by measuring changes in the cerebral blood flow Decreased striatal presynaptic DAT binding contralateral to parkinsonian symptomatology with greater reduction in posterior putamen than in anterior putamen or caudate nucleus Susceptibility weighted image (SWI) [13] Visualize iron-related contents sensitively Substantia nigra pars compacta, globus pallidus internus, the putamen, and the red nucleus have been described as regions with increased iron concentration Susceptibility weighted imaging (SWI), a type of iron-sensitive MRI, is frequently used to detect disease-specific patterns of uneven and localized iron concentration in brain regions [13]. Figure 1 shows the sample SWI axial slices of the MSA-P, MSA-C, PSP, and PD.…”

Section: Neuroimaging Modality Role Of Modality Potential Of Differen...mentioning

confidence: 99%

“…In addition, various studies using other modalities, including PET and SPECT, can achieve significant diagnostic relevance with respect to imaging of PD and APS [9,10]. However, there are also some disadvantages of these modalities, such as radiation exposure of CT [11], and obstacles to the clinical application of PET by limited access and high examination costs [12]. These common advanced neuroimaging techniques are summarized in Table 1.…”

Section: Introductionmentioning

confidence: 99%

Automated Differentiation of Atypical Parkinsonian Syndromes Using Brain Iron Patterns in Susceptibility Weighted Imaging

2022

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In recent studies, iron overload has been reported in atypical parkinsonian syndromes. The topographic patterns of iron distribution in deep brain nuclei vary by each subtype of parkinsonian syndrome, which is affected by underlying disease pathologies. In this study, we developed a novel framework that automatically analyzes the disease-specific patterns of iron accumulation using susceptibility weighted imaging (SWI). We constructed various machine learning models that can classify diseases using radiomic features extracted from SWI, representing distinctive iron distribution patterns for each disorder. Since radiomic features are sensitive to the region of interest, we used a combination of T1-weighted MRI and SWI to improve the segmentation of deep brain nuclei. Radiomics was applied to SWI from 34 patients with a parkinsonian variant of multiple system atrophy, 21 patients with cerebellar variant multiple system atrophy, 17 patients with progressive supranuclear palsy, and 56 patients with Parkinson’s disease. The machine learning classifiers that learn the radiomic features extracted from iron-reflected segmentation results produced an average area under receiver operating characteristic curve (AUC) of 0.8607 on the training data and 0.8489 on the testing data, which is superior to the conventional classifier with segmentation using only T1-weighted images. Our radiomic model based on the hybrid images is a promising tool for automatically differentiating atypical parkinsonian syndromes.

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The Strengths and Obstacles in the Differential Diagnosis of Progressive Supranuclear Palsy—Parkinsonism Predominant (PSP-P) and Multiple System Atrophy (MSA) Using Magnetic Resonance Imaging (MRI) and Perfusion Single Photon Emission Computed Tomography (SPECT)

Cited by 12 publications

References 29 publications

Epidemiology of Progressive Supranuclear Palsy: Real World Data from the Second Largest Health Plan in Israel

Epidemiology of Progressive Supranuclear Palsy: Real World Data from the Second Largest Health Plan in Israel

The Burden of Progressive Supranuclear Palsy on Patients, Caregivers, and Healthcare Systems by PSP Phenotype: A Cross-Sectional Study

Automated Differentiation of Atypical Parkinsonian Syndromes Using Brain Iron Patterns in Susceptibility Weighted Imaging

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