Sleep disturbances in progressive supranuclear palsy syndrome (PSPS) and corticobasal syndrome (CBS).

INTRODUCTION: Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are clinical manifestations of tauopathies. They are commonly associated with rapid motor and cognitive deterioration. Sleep disturbances are less frequently described as a feature of these diseases, though they are reported among 50-75% of PSP patients. STATE OF THE ART: Apart from various clinical manifestations, sleep abnormalities in PSP and CBS seem to be a factor enhancing pathogenesis as well its consequences. Multiple researchers have looked into the issue of whether the complexity of sleep disturbances in PSP and CBS could be linked to atrophic changes within structures crucial for daytime regulation, coexisting pathologies, or other less explored mechanisms. CLINICAL SIGNIFICANCE: Among sleep abnormalities in PSP and CBS have been reported excessive daytime sleepiness, night-time insomnia, reduction of total sleep time, more pronounced sleep fragmentation, restless leg syndrome (RLS), agrypnia excitata, periodic limb movements, sleep respiratory disturbances, rapid-eye movement behaviour disorder, and others. FUTURE DIRECTIONS: The aim of this review was to elaborate upon the significance of sleep abnormalities in tauopathic parkinsonian syndromes, and to determine their usefulness in differential diagnosis with synucleinopathic parkinsonian syndromes. Extended analyses of sleep disturbances may provide a different perspective on atypical parkinsonisms.

Glucose Metabolism and Cognitive Decline in Progressive Supranuclear Palsy and Corticobasal Syndrome: A Preliminary Study.

Multiple studies have analyzed the possible correlations between diabetes and Alzheimer's disease. Less is known about the context of cognitive deterioration among patients with atypical Parkinsonian syndromes and glucose metabolism impairment. The aim of this study was to evaluate the association between the impaired glucose metabolism and cognitive decline among patients with progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). The study included 22 patients with PSP and CBS with disease durations varying from 3 to 6 years. The levels of glycated hemoglobin (HbA1C), fasting blood glucose, fasting C-peptide and the presence of microalbuminuria were evaluated, and oral glucose tolerance tests (OGTT) were performed. Based on the OGTT results, the glycemic variability, mean glycemia, glycemia standard deviation (SD) and coefficient of variation (%CV) were calculated. All patients underwent a three-Tesla brain magnetic resonance (MRI) examination and neuropsychological cognitive assessment with the use of standardized scales: Montreal Cognitive Assessment (MoCA), Mini-Mental State Examination (MMSE) and Frontal Assessment Battery (FAB). A statistical analysis revealed that poor control of glycemia with high glycemic variability and increased atrophy of the medial temporal lobe among patients with PSP and CBS correlated with worse cognitive performance independent of age or sex, even among patients who did not fulfill the criteria for diabetes. The study results indicate the importance of glucose metabolism control and optimal treatment in the context of cognition maintenance among patients with PSP and CBS. Due to the relatively small number of analyzed patients, the issue requires further assessment. To the best of our knowledge, this is the first study discussing the role of glycemic variability in atypical Parkinsonian syndromes.

The significance of glial cell line-derived neurotrophic factor analysis in Progressive Supranuclear Palsy.

Progressive Supranuclear Palsy (PSP) is an atypical parkinsonism. Major subtypes of the disease: PSP-Richardson's Syndrome (PSP-RS) and PSP Parkinsonism Predominant (PSP-P) vary in clinical features, the pathomechanism remains unexplored. The aim of this work is to analyze the relevance of glial cell line-derived neurotrophic factor (GDNF) evaluation in the serum and cerebrospinal fluid (CSF) in PSP subtypes and to verify its significance as a possible factor in the in vivo examination. Authors assessed the concentration of GDNF in the serum and CSF of 12 patients with PSP-RS, 12 with PSP-P and 12 controls. Additionally authors evaluated patients using Unified Parkinson's Disease Rating Scale-III part (UPDRS-III), Frontal Assessment Battery (FAB) and Magnetic Resonance Imaging (MRI). The evaluation revealed significantly increased concentrations of GDNF in the CSF among PSP-RS patients and substantially increased concentrations of GDNF in the serum in PSP-P. Though the GDNF concentrations differentiated PSP subtypes, no correlations between with clinical factors were observed however certain correlations with atrophic changes in MRI were detected. GDNF is a factor which may impact the pathogenesis of PSP. Possible implementation of GDNF as a therapeutic factor could be a perspective in the search for therapy in this currently incurable disease.

The Role of the Evans Index and the Maximal Width of the Frontal Horns of the Lateral Ventricles in the Diagnostic Imaging of Progressive Supranuclear Palsy and Multiple-System Atrophy.

Progressive Supranuclear Palsy and Multiple-System Atrophy are entities within the spectrum of atypical parkinsonism. The role of imaging methods in the diagnosis and differentiation between PSP and MSA is limited and Magnetic Resonance Imaging (MRI) is currently used as a reference modality. In this study, the authors examined a group of patients with atypical parkinsonism using a 1.5 T MRI system and aimed to find simple and repeatable measurements that may be useful to distinguish between these diseases. The results of the study indicate that the maximal width of the frontal horns of the lateral ventricles and Evans' Index may, to some extent, be useful as basic and simple measurements in the diagnostic imaging of patients with atypical parkinsonism.

Enlarged Cisterna Chyli Diagnosed with Ultrasonography - Case Report.

Aim of the study: We present a case report of enlarged cisterna chyli in a 25-year-old woman. The diagnosis was made during a routine abdominal ultrasound examination and afterwards verified with contrast-enhanced MRI. Case description: Ultrasound revealed a large, lobulated, anechoic cystic structure with thin, smooth walls, lacking any solid components. The lesion was located in the retroperitoneal space, beneath the head of the pancreas, between the partially compressed inferior vena cava and the aorta, extending almost to the aortic bifurcation. We performed a contrast-enhanced MRI examination which confirmed the sonographic suspicion of enlarged cisterna chyli, showing a non-enhancing cystic lesion in continuity with the thoracic duct. Conclusions: Anatomy, sonographic and magnetic resonance appearance of cisterna chyli as well as differential diagnosis are discussed.

Is MRPI 2.0 More Useful than MRPI and M/P Ratio in Differential Diagnosis of PSP-P with Other Atypical Parkinsonisms?

Differential diagnosis of progressive supranuclear palsy remains difficult, especially when it comes to the parkinsonism predominant type (PSP-P), which has a more favorable clinical course. In this entity, especially during the advanced stages, significant clinical overlaps with other tauopathic parkinsonian syndromes and multiple system atrophy (MSA) can be observed. Among the available additional diagnostic methods in every-day use, magnetic resonance imaging (MRI) focused specifically on the evaluation of the mesencephalon seems to be crucial as it is described as a parameter associated with PSP. There is growing interest in relation to more advanced mesencephalic parameters, such as the magnetic resonance parkinsonism index (MRPI) and MRPI 2.0. Based on the evaluation of 74 patients, we demonstrate that only the mesencephalon/pons ratio and MRPI show a significant difference between PSP-P and MSA-parkinsonian type (MSA-P). Interestingly, this differential feature was not maintained by MRPI 2.0. The mesencephalon to pons ratio (M/P), MRPI and MRPI 2.0 were not found to be feasible for the differentiation of PSP-P from other atypical tauopathic syndromes.

The Significance of Asymmetry in the Assessment of Brain Perfusion in Atypical Tauopathic Parkinsonian Syndromes.

Progressive supranuclear palsy syndrome (PSPS) and corticobasal syndrome (CBS) are clinical manifestations of tauopathic Parkinsonian syndromes. Due to their overlapping symptomatology, the differential diagnosis of these entities may be difficult when bounded to clinical assessment. The manifestations are commonly associated with pathological entities-corticobasal degeneration and progressive supranuclear palsy, which are four-repeat tauopathies. In this study, the authors attempted to find whether the asymmetry typically associated with CBS may be feasible in the interpretation of perfusion single-photon computed tomography. The analysis based on the examination of patients with progressive supranuclear palsy-Richardson syndrome (PSP-RS), progressive supranuclear palsy-Parkinsonism predominant (PSP-P), and corticobasal syndrome (CBS) revealed significant asymmetry of perfusion of the amygdala in corticobasal syndrome. The more pronounced abnormalities of perfusion were observed in the left amygdala among patients with more severe Parkinsonian syndromes in CBS on the right. This study shows that the comparison of the perfusion of tauopathic Parkinsonian syndromes should be extended by asymmetry analysis. Interestingly, the differentiating potential of brain perfusion is present in the comparison of CBS and PSP-RS, but not in CBS and PSP-P. This phenomenon could be explained by more distinct asymmetry in the perfusion observed in PSP-P, which diminishes the differentiating potential of this parameter when it comes to the comparison of PSP-P and CBS. To the best of our knowledge, this is the first study evaluating which structures can be interpreted as significantly asymmetrical in the context of perfusion in CBS.

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).

Multiple System Atrophy-Parkinsonism Predominant (MSA-P) and Progressive Supranuclear Palsy-Parkinsonism Predominant (PSP-P) are the clinical manifestations of atypical parkinsonism. Currently, there are no efficient in vivo methods available relating to neuroimaging or biochemical analysis in the examination of these entities. Among the advanced methods available, using positron emission tomography is constrained by high cost and low accessibility. In this study the authors examined patients with two types of atypical parkinsonism-MSA-P and PSP-P, which are difficult to differentiate, especially in the early years of their development. The aim of this study was to assess whether the examination of patients in the period following the early years (3-6-year duration of symptoms) could be enhanced by perfusion single photon emission computed tomography (SPECT), magnetic resonance imaging (MRI) or evaluation of cognitive abilities. Extended examination using MRI and perfusion SPECT showed that the evaluation of the mesencephalon/pons ratio, mesencephalic volume decrease, the Magnetic Resonance Parkinsonism Index (MRPI) and frontal perfusion should be considered more feasible than screening cognitive evaluation in MSA-P and PSP-P with a 3-6-year duration of symptoms.