The relationship between atherosclerosis and dementia.

OBJECTIVE: The main objective is to confirm a hypothesis that atherosclerosis, through various mechanisms, considerably influences cognitive impairment and significantly increases the risk for developing dementia. Complete sample should be 920 individuals. The present study aimed to analyse epidemiological data from a questionnaire survey. METHODS: The work was carried out in the form of an epidemiological case control study. Subjects are enrolled in the study based on results of the following examinations carried out in neurology departments and outpatient centres during the project NU20-09-00119 from 2020 to 2023. Respondents were divided into four research groups according to the results of clinical examination for the presence of atherosclerosis and dementia. The survey was mainly concerned with risk factors for both atherosclerosis and dementia. It contained questions on lifestyle factors, cardiovascular risk factors, leisure activities, and hobbies. RESULTS: Analysis of the as yet incomplete sample of 877 subjects has yielded the following selected results: on average, 16% of subjects without dementia had primary education while the proportion was 45.2% in the group with both dementia and atherosclerosis. Subjects with dementia did mainly physical work. Low physical activity was more frequently noted in dementia groups (Group 2 - 54.4% and Group 3 - 47.2%) than in subjects without dementia (Group 1 - 19.6% and Group 4 - 25.8%). Coronary heart disease was more frequently reported by dementia patients (33.95%) than those without dementia (16.05%). CONCLUSION: Cognitively impaired individuals, in particular those with vascular cognitive impairment, have poorer quality of life and shorter survival. Risk factors contributing to such impairment are similar to those for ischaemic or haemorrhagic stroke. It may be concluded that most of the analysed risk factors play a role in the development of both atherosclerosis and dementia.

Intraoperative electrophysiological monitoring determines the final electrode position for pallidal stimulation in dystonia patients.

Background: Bilateral deep brain stimulation (DBS) of the globus pallidus internus (GPi) is an effective treatment for refractory dystonia. Neuroradiological target and stimulation electrode trajectory planning with intraoperative microelectrode recordings (MER) and stimulation are used. With improving neuroradiological techniques, the need for MER is in dispute mainly because of the suspected risk of hemorrhage and the impact on clinical post DBS outcome. Objective: The aim of the study is to compare the preplanned GPi electrode trajectories with final trajectories selected for electrode implantation after electrophysiological monitoring and to discuss the factors potentially responsible for differences between preplanned and final trajectories. Finally, the potential association between the final trajectory selected for electrode implantation and clinical outcome will be analyzed. Methods: Forty patients underwent bilateral GPi DBS (right-sided implants first) for refractory dystonia. The relationship between preplanned and final trajectories (MicroDrive system) was correlated with patient (gender, age, dystonia type and duration) and surgery characteristics (anesthesia type, postoperative pneumocephalus) and clinical outcome measured using CGI (Clinical Global Impression parameter). The correlation between the preplanned and final trajectories together with CGI was compared between patients 1-20 and 21-40 for the learning curve effect. Results: The trajectory selected for definitive electrode implantation matched the preplanned trajectory in 72.5% and 70% on the right and left side respectively; 55% had bilateral definitive electrodes implanted along the preplanned trajectories. Statistical analysis did not confirm any of the studied factors as predictor of the difference between the preplanned and final trajectories. Also no association between CGI and final trajectory selected for electrode implantation in the right/left hemisphere has been proven. The percentages of final electrodes implanted along the preplanned trajectory (the correlation between anatomical planning and intraoperative electrophysiology results) did not differ between patients 1-20 and 21-40. Similarly, there were no statistically significant differences in CGI (clinical outcome) between patients 1-20 and 21-40. Conclusion: The final trajectory selected after electrophysiological study differed from the preplanned trajectory in a significant percentage of patients. No predictor of this difference was identified. The anatomo-electrophysiological difference was not predictive of the clinical outcome (as measured using CGI parameter).

Echogenicity of Brain Structures in Huntington's Disease Patients Evaluated by Transcranial Sonography - Magnetic Resonance Fusion Imaging using Virtual Navigator and Digital Image Analysis.

PURPOSE: Transcranial sonography (TCS) magnetic resonance (MR) fusion imaging and digital image analysis are useful tools for the evaluation of various brain pathologies. This study aimed to compare the echogenicity of predefined brain structures in Huntington's disease (HD) patients and healthy controls by TCS-MR fusion imaging using Virtual Navigator and digitized image analysis. MATERIALS AND METHODS: The echogenicity of the caudate nucleus (CN), substantia nigra (SN), lentiform nucleus (LN), insula, and brainstem raphe (BR) evaluated by TCS-MR fusion imaging using digitized image analysis was compared between 21 HD patients and 23 healthy controls. The cutoff values of echogenicity indices for the CN, LN, insula, and BR with optimal sensitivity and specificity were calculated using receiver operating characteristic analysis. RESULTS: The mean echogenicity indices for the CN (67.0±22.6 vs. 37.9±7.6, p<0.0001), LN (110.7±23.6 vs. 59.7±11.1, p<0.0001), and insula (121.7±39.1 vs. 70.8±23.0, p<0.0001) were significantly higher in HD patients than in healthy controls. In contrast, BR echogenicity (24.8±5.3 vs. 30.1±5.3, p<0.001) was lower in HD patients than in healthy controls. The area under the curve was 90.9%, 95.5%, 84.1%, and 81.8% for the CN, LN, insula, and BR, respectively. The sensitivity and specificity were 86% and 96%, respectively, for the CN and 90% and 100%, respectively, for the LN. CONCLUSION: Increased CN, LN, and insula echogenicity and decreased BR echogenicity are typical findings in HD patients. The high sensitivity and specificity of the CN and LN hyperechogenicity in TCS-MR fusion imaging make them promising diagnostic markers for HD.

Serum neurofilament light chain levels in patients with cognitive deficits and movement disorders: comparison of cerebrospinal and serum neurofilament light chain levels with other biomarkers.

Background: Serum neurofilament light chain (S NfL) is a non-specific marker of neuronal damage, including Alzheimer's disease (AD). We aimed to verify the reference interval (RI) of serum NfL using a highly sensitive ELISA, and to estimate the optimal cut-off value for neuronal damage. Our second objective was to compare NfL in cerebrospinal fluid (CSF) and serum (S) with the routine neurodegeneration biomarkers used in AD, and to assess their concentrations relative to the degree of cognitive deficit. Methods: Samples from 124 healthy volunteers were used to estimate the S NfL RI. For the comparison study, we used CSF and S samples from 112 patients with cognitive disorders. Cognitive functions were assessed using the mini-mental state examination. ELISA assays were used to determine the CSF and S NfL levels, CSF ß-amyloid peptide42 (Aß42), CSF ß-amyloid peptide40 (Aß40), CSF total tau protein (tTau), CSF phosphorylated tau protein (pTau), and CSF alpha-synuclein (αS). Results: The estimated RI of S NfL were 2.25-9.19 ng.L-1. The cut-off value of S NfL for assessing the degree of neuronal impairment was 10.5 ng.L-1. We found a moderate statistically significant correlation between S NfL and CSF Aß42 in the group with movement disorders, without dementia (rs = 0.631; p = 0.016); between S NfL and CSF Aß40 in the group with movement disorder plus dementia (rs = -0.750; p = 0.052); between S NfL and CSF tTau in the control group (rs = 0.689; p = 0.009); and between S NfL and CSF pTau in the control group (rs = 0.749; p = 0.003). The non-parametric Kruskal-Wallis test revealed statistically significant differences between S NfL, CSF NfL, CSF Aß42, CSF tTau, and CSF pTau and diagnosis within groups. The highest kappa coefficients were found between the concentrations of S NfL and CSF NfL (κ = 0.480) and between CSF NfL and CSF tTau (κ = 0.351). Conclusion: Our results suggested that NfL and tTau in CSF of patients with cognitive decline could be replaced by the less-invasive determination of S NfL using a highly sensitive ELISA method. S NfL reflected the severity of cognitive deficits assessed by mini-mental state examination (MMSE). However, S NfL is not specific to AD and does not appear to be a suitable biomarker for early diagnosis of AD.

Histiocytic Sarcoma with an Unusual Clinical Manifestation Imitating Malignant Vascular Tumor: A Case Report.

BACKGROUND Histiocytic sarcoma is a rare malignant hematopoietic neoplasm with morphologic and immunohistochemical features of histiocytic differentiation, usually with unfavorable prognosis. Despite aggressive biological behavior, in subgroup of patients with localized disease, the prognosis can be very good. Few publications are available on localized cases of histiocytic sarcoma. These occur infrequently and continue to be a poorly-recognized morphological entity. CASE REPORT A 73-year old man treated for Parkinson syndrome presented with a tumor resistance on the dorsal surface of the left forearm. This lesion was clinically seen as an organized hematoma and was surgically resected. Histologically, the tumor was situated in the dermis and subcutis and it consisted of multiple neoplastic nodules. Vasoformative growth patterns with the vascular-like spaces containing erythrocytes and hemosiderin pigment presence simulated the morphology of angiosarcoma. Based on the immunohistochemical characteristics, we diagnosed the tumor as cutaneous histiocytic sarcoma. Genetic analysis revealed immunoglobulin heavy-chain gene rearrangement without any concomitant hematological malignancy. The patient demonstrated no systemic disease or impairment associated with diagnosed histiocytic sarcoma, and no recurrence has been found to date. CONCLUSIONS We report a case of primary cutaneous histiocytic sarcoma with an excellent outcome after surgical treatment only. Clinical data and histopathological and immunohistochemical evaluation were essential to rule out other malignant tumors in the differential diagnosis. Genetic analysis together with up-to-date knowledge and understanding of principles of tumorous transformations helped to diagnose this poorly-recognized entity with various clinical behaviors.

The Attitudes of Progressive Neurological Disease Patients and Their Family Members to End of Life Care: A Cross-Sectional Study.

The objective was to identify the attitudes of progressive neurological disease (PND) patients and their family members regarding end-of-life care, and their worries about dying. The sample included 327 participants. The Attitudes of Patients with PND to End-of-Life Care questionnaire was used to collect the data. Statistically significant differences in the assessment of attitudes towards end-of-life care between patients and family members were identified (p < 0.001). Family members more frequently favored patients being kept alive at any cost; patients more commonly wished to have their end of life under control. Respondents most frequently deferred to doctors when it came to decisions on treatment to keep patients alive. However, both patients and family members wanted patients to be able to decide on their treatment by leaving a written record of their previously stated wishes. The demands of patients and their families regarding end-of-life care should be documented in individual care plans.

Digitized Image Analysis of Insula Echogenicity Detected by TCS-MR Fusion Imaging in Wilson's and Early-Onset Parkinson's Diseases.

Transcranial sonography (TCS) can reveal pathology in brain structures including insula. This study compared insula echogenicity among 22 patients with Wilson's disease (WD), 21 patients with early-onset Parkinson's disease (EO-PD) and 24 healthy patients. Echogenicity of predefined brain structures (insula, lentiform nucleus, caudate nucleus, substantia nigra and raphe nuclei) was evaluated using digitized analysis of TCS fusion imaging with magnetic resonance. Cortical, subcortical and cerebellar atrophy and ventricle diameters were determined from magnetic resonance images. The mean echogenicity index of insula did not differ between males and females (p = 0.92), but the echogenicity of insula was higher in patients with WD than in patients with EO-PD and healthy patients (p < 0.05). The substantia nigra echogenicity was higher in patients with EO-PD, and lentiform nucleus echogenicity was higher in patients with WD (p < 0.05). The echogenicity of insula correlated with lentiform nucleus echogenicity (r = 0.75) but not with age (r = -0.14), disease duration (r = -0.36), symptom severity (r = 0.28), cortical (r = 0.11) nor subcortical (r = 0.05) atrophy.

Transcranial sonography and (123)I-FP-CIT single photon emission computed tomography in movement disorders.

Diagnosis of Parkinson's disease (PD) can be difficult in the early stages of the disease. The aim of the study described here was to assess the correlation between transcranial sonography (TCS) and (123)I-FP-CIT ([(123)I]ioflupane, N-ω-fluoropropyl-2ß-carbomethoxy-3ß-(4-[(123)I]iodophenyl)nortropane) SPECT (single photon emission computed tomography) findings and the diagnosis of PD. A total of 49 patients were enrolled in the study: 29 patients with PD, 7 patients with other parkinsonian syndromes, 11 patients with essential tremor and 2 with psychogenic movement disorder. Substantia nigra echogenicity was measured using TCS. SPECT was performed using DaTSCAN ([(123)I]ioflupane). TCS and SPECT findings were correlated in 84% of patients, with κ = 0.62 (95% confidence interval: 0.38-0.86). TCS-measured substantia nigra echogenicity and SPECT-measured striatal binding ratio were negatively correlated (r = -0.326, p = 0.003). TCS/SPECT sensitivity, specificity and positive and negative predictive values for the diagnosis of PD were 89.7%/96.6%, 60.0%/70.0%, 76.5%/82.4% and 80.0%/93.3%, respectively. Both positive TCS and SPECT findings correlated significantly with the diagnosis of PD (κ = 0.52, 95% confidence interval: 0.27-0.76, and κ = 0.69, 95% confidence interval: 0.49-0.90, respectively).

A new program for highly reproducible automatic evaluation of the substantia nigra from transcranial sonographic images.

AIMS: Recent studies report increased echogenicity of the substantia nigra (SN) in patients with Parkinson's disease (PD) using transcranial sonography (TCS). However, the main limitation to TCS is its dependence on the sonographer's experience. Experimental software for quantitative evaluation of the echogenic SN area was thus developed by us. The aim of this study was to test the reliability of the data using developed B-Mode Assist software in patients with parkinsonism and in healthy volunteers. METHODS: The SN was imaged from the right temporal bone window in mesencephalic plane using TCS. DICOM images of SN were saved, converted into JPEG format, encoded and processed. Two observers performed 3 automatic evaluations of the SN area (measurements of SN area in each gray scale intensity inside the region of interest) by counting the standard deviation of all 6 measurements using developed software. The average value of all 3 measurements of each observer was used for computing Cohen's kappa coefficient to determine inter-observer correlations. Cohen's kappa coefficients as an intra-observer correlation for observer 1 and observer 2 were counted from the first 2 measurements of both observers. RESULTS: In total, 92 images were evaluated using this software. The mean of the standard deviations was 3.87; Cohen's kappa for intra-observer agreement of two observers were 0.947, and 0.943, resp.; Cohen's kappa for inter-observers agreement was 0.880. The agreement between visual and automatic detection of SN pathology was in 97.8% images. The sensitivity, specificity, positive and negative predictive values of automatic measurement were 100, 96.2, 95.1, 100%, resp. CONCLUSIONS: The results show very reliable measurement of SN features using designed application with "almost perfect" inter-observer and intra-observer agreements.

Correlation between substantia nigra features detected by sonography and Parkinson disease symptoms.

OBJECTIVE: The aim of this study was to assess the correlation between substantia nigra (SN) echogenic features and area and the clinical symptoms in patients with Parkinson disease (PD). METHODS: Clinical examinations and transcranial sonographic evaluations of the SN were performed in 115 consecutive patients with PD. The presence of tremors, rigidity, bradykinesia, gait disorders, speech disorders, and hypomimia was evaluated according to the motor portion of the Unified Parkinson Disease Rating Scale. The Mann-Whitney U test, the Kruskal-Wallis test, analysis of variance, and multivariate analysis were applied when assessing statistical significance. RESULTS: An enlarged and hyperechoic SN existed in 84.0% of patients with bilateral rigidity but in only 70.6% of patients with unilateral rigidity (P < .05). Similarly, 85.0% of patients with bilateral bradykinesia in comparison with 65.7% of patients with unilateral bradykinesia had an enlarged and hyperechoic SN (P < .05). A significant correlation was shown between the SN echogenicity and area (r = 0.705; P < .01). Conclusions. An enlarged and hyperechoic SN seems to be a marker of structural involvement of the SN in patients with PD. This structural involvement is expressed more in patients with bilateral rigidity and bradykinesia.

Transcranial sonography in movement disorders.

BACKGROUND: Transcranial sonography (TCS) in the B-mode has the ability to image, infratentorial and supratentorial brain structures. For this reason, it has potential use in the diagnosis and differential diagnosis of various intracranial pathologies. METHODS AND RESULTS: The authors reviewed the contribution of TCS to the differentiation of a number of neurodegenerative diseases: in parkinsonian syndromes, TCS can evaluate echogenicity changes in specific structures such as the hyperechogenic area of the substantia nigra (SN) in Parkinson's disease and the hyperechogenic caudate nucleus in Huntington's disease as well as the hyperechogenic lentiform nucleus (LN) in dystonia and Wilson's disease. In parkinson-plus syndromes, TCS may detect changes in width of the third ventricle and of the frontal horns of the lateral ventricle. The hyperechogenic SN can also be used in healthy populations as a marker of subclinical injury to the nigrostriatal system. CONCLUSION: TCS is a quick, safe and non-invasive method. It could be helpful in differentiation between several movement disorders together with clinical examination and other neuroimaging methods.

Reproducibility of sonographic measurement of the substantia nigra.

The aim of this study was to evaluate inter-reader, intra-investigator and inter-investigator reproducibility and correlations in the assessment of substantia nigra (SN) echogenicity and area measurement by a physician-sonographer (PS), a sonographic laboratory assistant (SLA) and a physician without sonographic experience (PN). A total of 22 patients with extrapyramidal symptoms were examined using transcranial sonography (TCS). SN images were encoded and evaluated by the three readers. A second TCS examination was performed after 7+/-2 d. A second investigator performed TCS examination 1 mo later. Spearman rank correlation and Pearson's correlation coefficient were used when assessing the agreement between readers. All three readers identified the same 15 patients with SN echogenicity III or more. Inter-reader SN echogenicity and area measurement correlations were r=0.55 to 0.82 and r=0.31 to 0.74 between PS and SLA and r=0.55 to 0.77 and 0.49 to 0.62 between PS and PN, respectively (p<0.05 in all cases). Intra-reader echogenicity and area measurement correlations (r=0.85 to 0.96 and r=0.51 to 0.69) were statistically significant only for PS (p<0.001). All intra- and inter-investigator correlations of SN area measurement (r=0.69 to 0.88 and r=0.5 to 0.61) and SN echogenicity (r=0.64 to 0.92 and r=0.51 to 0.69) were statistically significant (p<0.05). Semiquantitative evaluation of SN echogenicity and area using TCS is highly dependent on the experience of the sonographer. Only an experienced sonographer was able to produce very reproducible results with statistically significant correlations; SLA and PN intra-reader correlations were poor.