Is brain perfusion a differentiating feature in the comparison of Progressive Supranuclear Palsy Syndrome (PSPS) and Corticobasal Syndrome (CBS)?

Alster, Piotr; Nieciecki, Michał; Koziorowski, Dariusz; Cacko, Andrzej; Charzyńska, Ingeborga; Królicki, Leszek; Friedman, Andrzej

doi:10.1016/j.jocn.2020.05.005

Cited by 8 publications

(12 citation statements)

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“…We are aware of the limitations of our study such as the relatively small number of patients. However, various studies evaluating other features, possibly facilitating the examination of CBS, have been based on smaller numbers of patients [9,10,20]. The disparity between the number of male and female patients in the CBS group is related to the rarity of this syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…The boundaries of CBS and PSPS are evaluated on various grounds. Due to overlapping clinical manifestations, interpreting PSPS and CBS as independent entities has become less obvious [8][9][10]. Contemporary criteria of PSP diagnosis mention PSP-CBS -a syndrome with mixed symptomatology, however multiple cases show dominant PSP or CBS syndrome, with insufficient evidence to diagnose PSP-CBS [11].…”

Section: Introductionmentioning

confidence: 99%

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Neutrophil-to-lymphocyte ratio (NLR) at boundaries of Progressive Supranuclear Palsy Syndrome (PSPS) and Corticobasal Syndrome (CBS)

Alster

Madetko

Friedman

2021

Neurol Neurochir Pol.

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Aim of the study. To examine possible features of neuroinflammation in progressive supranuclear palsy -Richardson syndrome and corticobasal syndrome (CBS).Clinical rationale for the study. Neutrophil-to-lymphocyte ratio (NLR) is a parameter reflecting inflammation used in numerous branches of medicine. The search for pathogenesis of the diseases partly related to inflammatory processes confirms the need to obtain possible factors which could be relatively easily verified. NLR is a benchmark routinely evaluated in most hospitalised patients.Materials and methods. 23 patients with a clinical diagnosis of PSP-RS, 18 patients with CBS, and 32 healthy controls, were included in the study. Blood samples were assessed in the context of neutrophil and lymphocyte rates. Subsequently, the results were transformed into neutrophil-to-lymphocyte ratio (NLR). The NLRs from each group were statistically assessed using a Kruskal-Wallis test and post-hoc analysis.Results. Statistical analysis confirmed significant differences in NLR between PSP-RS and control group. No other significant differences were observed.Clinical implications. The possible use of NLR in the additional examination of atypical parkinsonisms. Conclusions.To the best of our knowledge, this is the first study comparing this aspect of neuroinflammation in PSP and CBS. It presents NLR as a promising non-specific parameter in neurodegenerative diseases.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Neutrophil-to-lymphocyte ratio (NLR) at boundaries of Progressive Supranuclear Palsy Syndrome (PSPS) and Corticobasal Syndrome (CBS)

Alster

Madetko

Friedman

2021

Neurol Neurochir Pol.

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“…Additionally, due to the pilot character of the study, the authors were not granted permission by the Ethical Committee to use radiotracers in patients without neurodegenerative disease in this study considering the availability of a software database of healthy volunteers age-matched with the examined group of PSP/CBS patients. The authors did not create separate groups for PSP and CBS patients, as previous studies showed that perfusion assessment does not show significant differences in the differential diagnoses of these diseases ( 6 ). Due to the fact that all included patients are alive, no neuropathological verification could be conducted.…”

Section: Discussionmentioning

confidence: 99%

“…Although the boundaries between the pathologies of PSP and CBD related to tufted astrocytes and astrocytic plaques are not in question, the differentiation between their clinical manifestations is less obvious ( 4 ). Several studies highlight the questionable significance of PSP and CBS differentiation, especially in advanced stages ( 4 , 6 ). The diseases share similar risk factors as hypertension and diabetes, however, the exact mechanism of their correlation has not been explored ( 7 , 8 ).…”

Section: Introductionmentioning

confidence: 99%

The Rate of Decrease in Brain Perfusion in Progressive Supranuclear Palsy and Corticobasal Syndrome May Be Impacted by Glycemic Variability—A Pilot Study

et al. 2021

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Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are tauopathic parkinsonian syndromes, presently lacking disease-modifying treatments. Patients affected by these diseases suffer due to multidimensional deteriorations resulting in motor and cognitive impairment. Previously published research has confirmed risk factors that may impact the course of PSP and CBS, among them hypertension and diabetes. Less data is available regarding prediabetes and glycemic variability. In this study, 26 patients with clinical diagnoses of PSP and CBS were examined using glycated hemoglobin and perfusion single-photon emission tomography (SPECT). Patients were divided into two groups—PSP/CBS patients with glycated hemoglobin (HbA1c) below and above 5.7%. The results of the perfusion evaluation were compared with the values from healthy volunteers from the software's database. A decrease in perfusion in certain regions of interest was observed among patients affected by increased glycemic variability. A more pronounced decrement in perfusion was observed only in some regions of interest—the hippocampus, pons, left thalamus, right insula. The results indicated that, among PSP/CBS patients, individuals with more pronounced glycemic variability had more severe hypoperfusion in certain brain regions in comparison with PSP/CBS patients without carbohydrate metabolism disorders. Due to the fact that PSP and CBS are associated with cognitive impairment, an additional decrease in perfusion in the hippocampal area may impact the rate of cognitive deterioration.

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“…Clinically, the diagnosis of CBD is now referred to CBS, which can be a manifestation of pathologies beyond CBD, as it overlaps with several forms of dementia of differing neuropathologies (Armstrong et al, 2013). As a result of its diverse clinical manifestations and a lack of biomarkers enabling early diagnosis, it is estimated that only 25-56% of patients are diagnosed correctly with CBS before death (Lee et al, 2011;Alster et al, 2018Alster et al, , 2020Svenningsson, 2019;Caixeta et al, 2020).…”

Section: Corticobasal Degenerationmentioning

confidence: 99%

Novel Therapies for Parkinsonian Syndromes–Recent Progress and Future Perspectives

Przewodowska

Marzec

Madetko

2021

Front. Mol. Neurosci.

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Background: Atypical parkinsonian syndromes are rare, fatal neurodegenerative diseases associated with abnormal protein accumulation in the brain. Examples of these syndromes include progressive supranuclear palsy, multiple system atrophy, and corticobasal degeneration. A common clinical feature in parkinsonism is a limited improvement with levodopa. So far, there are no disease-modifying treatments to address these conditions, and therapy is only limited to the alleviation of symptoms. Diagnosis is devastating for patients, as prognosis is extremely poor, and the disease tends to progress rapidly. Currently, potential causes and neuropathological mechanisms involved in these diseases are being widely investigated.Objectives: The goal of this review is to summarize recent advances and gather emerging disease-modifying therapies that could slow the progression of atypical parkinsonian syndromes.Methods: PubMed and Google Scholar databases were searched regarding novel perspectives for atypical parkinsonism treatment. The following medical subject headings were used: “atypical parkinsonian syndromes—therapy,” “treatment of atypical parkinsonian syndromes,” “atypical parkinsonian syndromes—clinical trial,” “therapy of tauopathy,” “alpha-synucleinopathy treatment,” “PSP therapy/treatment,” “CBD therapy/treatment,” “MSA therapy/treatment,” and “atypical parkinsonian syndromes—disease modifying.” All search results were manually reviewed prior to inclusion in this review.Results: Neuroinflammation, mitochondrial dysfunction, microglia activation, proteasomal impairment, and oxidative stress play a role in the neurodegenerative process. Ongoing studies and clinical trials target these components in order to suppress toxic protein accumulation. Various approaches such as stem cell therapy, anti-aggregation/anti-phosphorylation agent administration, or usage of active and passive immunization appear to have promising results.Conclusion: Presently, disease-modifying strategies for atypical parkinsonian syndromes are being actively explored, with encouraging preliminary results. This leads to an assumption that developing accurate, safe, and progression-halting treatment is not far off. Nevertheless, the further investigation remains necessary.

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Is brain perfusion a differentiating feature in the comparison of Progressive Supranuclear Palsy Syndrome (PSPS) and Corticobasal Syndrome (CBS)?

Cited by 8 publications

References 9 publications

Neutrophil-to-lymphocyte ratio (NLR) at boundaries of Progressive Supranuclear Palsy Syndrome (PSPS) and Corticobasal Syndrome (CBS)

Neutrophil-to-lymphocyte ratio (NLR) at boundaries of Progressive Supranuclear Palsy Syndrome (PSPS) and Corticobasal Syndrome (CBS)

The Rate of Decrease in Brain Perfusion in Progressive Supranuclear Palsy and Corticobasal Syndrome May Be Impacted by Glycemic Variability—A Pilot Study

Novel Therapies for Parkinsonian Syndromes–Recent Progress and Future Perspectives

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