Socioeconomic and Health Determinants of the Prevalence of COVID-19 in a Population of Children with Respiratory Diseases and Symptoms.

BACKGROUND: Most epidemiological studies indicate that bronchial asthma is not a risk factor for COVID-19, but previous analyses have not additionally focused on the socioeconomic determinants of SARS-CoV-2 infection in children with asthma, bronchitis, and respiratory symptoms. AIMS: This research aimed to investigate the correlation between the socioeconomic status of families and the prevalence of respiratory conditions such as asthma, bronchitis, and respiratory symptoms in children, in addition to exploring their association with the prevalence of COVID-19. The study involved a cross-sectional epidemiological investigation conducted in 2022, encompassing 2454 students from elementary schools in Poland. The parents of the students completed a questionnaire modeled after the International Study on Asthma and Allergies in Childhood (ISAAC). Socioeconomic status (SES) indicators were determined based on parental education, self-reported economic status, and housing conditions. To assess the impact of social factors and health on the occurrence of COVID-19, odds ratios (ORs) were calculated. The findings revealed several COVID-19 risk factors, including higher maternal (OR 2.2; 95%CI: 1.3-3.0) and paternal education (OR 1.9; 95%CI: 1.3-2.4), urban residence (OR 1.7; 95%CI: 1.3-2.1), the presence of mold in residences (OR 1.7; 95%CI: 1.0-2.3), bronchitis (OR 1.5; 95%CI: 1.2-2.0), and chronic cough (OR 1.8; 95%CI: 1.3-2.4). Further analysis, stratifying children based on their baseline health status (i.e., presence or absence of asthma, bronchitis, and chronic cough), indicated that higher parental education increased the risk of COVID-19 solely for children without pre-existing conditions. The occurrence of SARS-CoV-2 infections was found to be notably associated with mold exposure in children who did not have bronchial asthma. Rigorous multivariate analyses substantiated the collective impact of factors such as residential environment, the existence of mold and moisture, and a history of bronchitis. This study's conclusions highlight a higher frequency of SARS-CoV-2 infections in cases where bronchitis had been diagnosed previously and chronic cough was prevalent. Interestingly, the initially hypothesized higher prevalence of COVID-19 among children with bronchial asthma did not receive confirmation in our findings. This study highlights the importance of urban residence, exposure to mold or dampness, and higher parental education in the incidence of COVID-19. Higher parental education was a significant factor in increasing the risk of COVID-19 among children without bronchitis, chronic cough, and asthma.

Pre- and Postnatal Determinants Shaping the Microbiome of the Newborn in the Opinion of Pregnant Women from Silesia (Poland).

Pre- and postnatal factors influence the formation of the newborn's microbiome as early as birth and the intrauterine period has a substantial impact on the composition of the baby's gastrointestinal microbiota and its subsequent development. This study intends to measure pregnant women's knowledge of the importance of microbiota for the health of the newborn. The sample was selected based on defined inclusion and exclusion criteria. The assessment of women's knowledge was assessed by the Kolmogorov-Smirnov and Kruskal-Wallis statistical tests. This study population comprised 291 adult pregnant women with a mean age of 28.4 ± 4.7 years. A total of 34% (n = 99), 35% (n = 101), and 31.3% (n = 91) were at the 1-3 trimester, respectively. The results showed that 36.4% of the women were aware that the intrauterine period changes the makeup of the gastrointestinal microbiota, whereas 5.8% exhibited awareness of the composition of the child's normal gut microbiota. Most of the women surveyed-(72.1%)-know that colonization of the tract occurs as early as the birth period. Women with student status (those who will pursue higher education in the future) and those who had given birth to the most children exhibited higher levels of knowledge.

Risk factors for reoperation after surgical treatment for degenerative spinal disease in Poland: a nationwide retrospective study of 38,953 hospitalisations.

INTRODUCTION: Degenerative spinal disease (DSD) is one of the most common musculoskeletal conditions and a leading cause of sickness absence. It also contributes significantly to the global burden of disease. The aim of this study was to assess the frequency of reoperation after surgical treatment of DSDs in Poland, and to identify risk factors for reoperation. MATERIAL AND METHODS: A retrospective analysis of hospitalisations for DSD in 2018 that were reported to Poland's National Health Fund (NHF) was performed. Reoperations reported within 365 days of hospital discharge were identified. Demographic factors and multimorbidities were included in the analysis. A logistic regression model was then performed to assess risk factors for reoperations. RESULTS: In 2018, 38,953 surgical hospitalszations for DSD were reported. A total of 3,942 hospitalised patients (10.12%) required reoperation within 365 days. Patients requiring reoperation were predominantly female (female-to-male ratio 1.34:1) and elderly (mean age of reoperated patients 56.66 years, mean age of other patients 53.24). The percentage reoperated upon correlated with multiple diseases (from 8.81% in the group of patients without comorbidities to 15.31% in the group of patients with three or more comorbidities). The risk of reoperation was most increased by comorbid depression, neurological diseases, obesity, and older age. The risk of reoperation was reduced by instrumented spinal surgery, surgery in a neurosurgical unit, and hospitalisations other than same-day surgery. CONCLUSIONS: Reoperations within a year after DSD surgical treatment are common. Identifying risk factors for reoperation, including those related to the presence of comorbidities and the phenomenon of multimorbidity, can be an important tool in reducing reoperation rates.

Impact of the COVID-19 Pandemic on Nutrition, Sleep, Physical Activity, and Mood Disorders of Polish Children.

The harmful consequences of the COVID-19 pandemic on children are its impact on eating habits, physical activity, sleep, and mood disorders. In the future, this may result in a higher prevalence of obesity and diet-related diseases. Therefore, this study aimed to assess the impact of the COVID-19 pandemic on children's eating behavior and lifestyle. The study was conducted using a proprietary questionnaire on dietary and lifestyle habits before and during the pandemic, and the reasons for changes due to the pandemic. The study involved 294 parents of children in grades 1-8 in elementary schools in two regions of Poland. The survey showed that during the pandemic, the percentage of children eating five regular meals daily, including fruits and vegetables, and engaging in daily physical activity decreased. However, the percentage of children spending more than 4 h a day in front of a screen increased (p < 0.05). The main reasons for changes in eating habits and physical activity were less eating out, lack of motivation, obstruction, and lack of access to sports facilities (p < 0.05). The pandemic had a significant impact on reduced levels of physical activity and increased time spent in front of a screen. In summary, among the reasons for changes in children's dietary and lifestyle habits, factors related to the pandemic itself, i.e., social restrictions, restrictions, closure of schools and other facilities, and fear of coronavirus infection, had the greatest impact.

Properties of Polyunsaturated Fatty Acids in Primary and Secondary Prevention of Cardiovascular Diseases in the View of Patients (Silesia, Poland).

BACKGROUND: Cardiovascular diseases are a major cause of morbidity and mortality in Europe. Lifestyle plays an important role in the primary and secondary prevention of cardiovascular diseases, apart from pharmacotherapy and diagnostics. Numerous studies confirm that the type and quality of fat consumed in the diet have a huge impact on the risk of cardiovascular diseases. Reducing the risk of cardiovascular disease can be helped by minimizing the proportion of saturated fatty acids in the diet and replacing them with polyunsaturated fatty acids. These acids and, above all, their long-chain forms have a positive effect on health. AIM: This study aims to assess the awareness of the properties of polyunsaturated fatty acids in the primary and secondary prevention of cardiovascular diseases in the opinions of patients of the Cardiology Department of the Racibórz Medical Center. MATERIAL AND METHODS: The analysis included 302 patients (113 women and 189 men) hospitalized in the Cardiology Department. The research method was the authors' questionnaire consisting of the patients' record and thirty closed questions. To answer the research questions posed and test the hypotheses, statistical analyses were carried out using the IBM SPSS Statistics version 25 package. RESULTS: Among the respondents, the least frequently used healthy eating habit was the infrequent eating of fried foods. A total of 18.2% of respondents had such a habit. The most commonly used healthy eating habit was checking the fat content in products, which was performed by 67.2% of respondents. Among the respondents, 58.3% said that butter and margarine increase serum cholesterol. CONCLUSIONS: The analysis of the data shows that the place of residence, education, sex, and reason for hospitalization of the respondents did not affect the frequency of healthy eating habits. In addition, the subjects had a low amount of healthy eating habits.

Microplastic in Food and Water: Current Knowledge and Awareness of Consumers.

In recent years, the widespread of microplastics in the food chain and environment became a topic of much research. This article focused on the knowledge and awareness of people with higher education levels-mostly young ones. The aim of this study is to analyze to what extent consumers know about and are aware of the source of microplastics, the level of exposure, and potential health hazards connected to the contamination of food and water with microplastics. The test group, consisting of 410 people, is mostly able to correctly characterize what microplastics mean and knows its sources. A majority of the group is aware of potential presence of microplastics in water; however, the knowledge about contamination of other elements of the environment seems to be gradually lowering. The majority of the people taking part in the research know that microplastic might be present in foods, and they are aware that after entering the human body, it might accumulate in internal organs. Moreover, when asked about potential health hazards, the group chose mostly tumors and gastrointestinal disorders, while disorders of the reproductive system were chosen less frequently. Consumers' knowledge regarding the sources and health hazards of microplastics seems to be more common among women, in groups living in cities and among people who studied physics-related subjects and medicine.

Caesarean delivery and risk of childhood asthma development: meta-analysis.

INTRODUCTION: Several studies indicate that delivery by caesarean section may be one of the risk factors for the development of childhood asthma. AIM: A meta-analysis was carried out to establish the relationship between delivery by caesarean section and asthma in children. MATERIAL AND METHODS: After a review of bibliographic databases, 41 articles were obtained and 12 of which were accepted for further analysis. The odds ratios (OR) included in the analysis were specified on the basis of data from the presented studies or were calculated using reported prevalence. The analysis took into account unadjusted OR. The heterogeneity of results was assessed using the χ2 test, determining p < 0.05 as the level of significance. The analysis was performed using Statistica 13.3 and kit 4.0.67. RESULTS: Caesarean delivery was associated with an increased risk of development of childhood asthma (OR = 1.41); however, significant heterogeneity of results was demonstrated. A significantly higher risk of asthma was found in children born by caesarean section in the case where the disease was confirmed in a questionnaire-based study (OR = 1.26, 95% CI: 1.05-1.5), and the results of that study were homogeneous. CONCLUSIONS: In the case of the diagnosis of asthma declared by parents in the questionnaire-based study, a significantly higher risk of disease occurrence was observed in children born by caesarean section. Due to the significant heterogeneity of the results of the studies, it cannot be clearly stated that caesarean delivery is a risk factor for the development of bronchial asthma.

Spinal muscular atrophy: epidemiology and health burden in children - a Polish national healthcare database perspective before introduction of SMA-specific treatment.

INTRODUCTION: Spinal muscular atrophy (SMA) is one of the most frequent autosomal recessive neuromuscular disorders. It leads to progressive muscle weakness, premature death or permanent ventilation. Significant disability, scoliosis, severe pulmonary infections and other problems require in- and outpatient medical care. Various approaches have been used to evaluate SMA epidemiology, healthcare burden and adherence to standard of care. The recent introduction of pharmacological treatment in a large SMA population will change the course of the disease and the healthcare requirements of patients. MATERIAL AND METHODS: We have used the National Health Fund database to identify children with SMA and the healthcare service they received in the pre-pharmacological treatment era. Pivotal phase II and III medical trials for nusinersen were conducted between 2013 and 2015. The National Treatment Programme of SMA patients with nusinersen in our country was started in January 2019. The year 2014 was used to evaluate incident cases. RESULTS: 51 new SMA cases (incidence 1:7,356) and 518 SMA patients younger than 18 were identified in 2014. 32 (6.2%) deaths were recorded, half in the first two years of life. 35 (6.8%) patients received palliative and 115 (22.2%) long-term care (including assisted ventilation). A total number of 3,057 days of hospital stay were reported. Only 65/518 (12.6%) patients did not receive publicly-funded healthcare service other than specialist or general practitioner's consultation. CONCLUSIONS: SMA caused significant mortality and morbidity in children. The National Health Fund database can be used to reliably record incident cases and track the care provided to paediatric SMA patients.

Health inequalities among students of lower secondary schools in Bytom, Poland.

INTRODUCTION AND OBJECTIVE: Poverty and low level of education pose the biggest threats to public health. Moreover, they generate inequalities in public healthThe aim of the study was to check if there are any inequalities in health among teenagers living in Bytom, Poland. MATERIAL AND METHODS: In 2011 and 2012, an epidemiological cross-sectional study was conducted on 1,099 students from lower secondary schools from Bytom. The students completed a questionnaire which was based on an earlier Health Behaviour in School-aged Children study (HBSC). Socio-Economic Status of teenagers (SES) was determined according to the Family Affluence Scale (FAS), the intensity of possible problems in the place of residence and on parents' education. Impact of SES on health self-assessment, asthma, pneumonia, bronchitis with addition to spinal deformities were also investigated. RESULTS: A good or very good level of health was declared by students from families representing a high level of FAS and residing in a more peaceful, less troubled neighbourhood. The highest level of asthma prevalence (10.9%) occurred among students from families with a low level of FAS. The students from families with high FAS were less affected by spinal deformities (34.6%). Students living in a troubled neighbourhood more often suffered from bronchial asthma, pneumonia, bronchitis and spinal deformities. CONCLUSIONS: The level of family affluence depends on the parents' education and all the analysed health problems occurred more frequently in children whose parents had completed at least general secondary education. A high economic standard of living and a peaceful neighbourhood determined good or very good health self-assessment among the surveyed students.

Assessment of explicit and implicit linguistic impairments in patients with aphasia after resection of tumour of the left cerebral hemisphere. Preliminary results.

BACKGROUND AND PURPOSE: Classical definitions of aphasia des-cribe deficits of different language levels (syntactic, semantic, phonologic) hindering the ability to communicate. Recent studies indicate, however, that impairment of particular aspects of linguistic competencies in aphasia differs in severity. Contemporary approach to the aphasic symptoms presents them as disturbed access of linguistic representations to the awareness system. Accordingly, such an approach requires different types of tasks: direct, involving explicit language processes, and indirect, based on implicit language representations. The aim of our study was to examine explicit and implicit language processes in patients with aphasia after resection of the tumour of left cerebral hemisphere along with characterization of relationships between explicit and implicit language processes. MATERIAL AND METHODS: Our cohort included 28 right-handed patients who were divided into four equal groups: two clinical (brain tumours) and two control (lumbar disc disease). Four tasks that assess and compare language processes: lexical decisions (at explicit and implicit levels), sorting of picture captions and word monitoring were implemented. RESULTS: In direct tasks, patients with aphasia provided less correct lexical decisions at word level, but did not show deficits in sentence comprehension. In both groups, no priming effect was observed in tasks requiring implicit lexical decisions. The longest time was found in non-primed words, the shortest in pseudowords. The differences between groups regarding word monitoring were also observed. Patients with aphasia obtained longer reaction times in all types of sentences (of different grade of language correctness), with respect to low- and high- frequency words. CONCLUSIONS: Patients with aphasia after brain tumour resection show more pronounced impairments of explicit than implicit linguistic behavior; the same effect was found in studies on forgetting in amnestic syndrome.

Epidemiology of glial and non-glial brain tumours in Europe.

To the central nervous system (CNS) belong a heterogeneous group of glial and non glial rare cancers. The aim of the present study was to estimate the burden (incidence, prevalence, survival and proportion of cured) for the principal CNS cancers in Europe (EU27) and in European regions using population-based data from cancer registries participating in the RARECARE project. We analysed 44,947 rare CNS cancers diagnosed from 1995 to 2002 (with follow up at 31st December 2003): 86.0% astrocytic (24% low grade, 63% high grade and 13% glioma NOS), 6.4% oligodendroglial (74% low grade), 3.6% ependymal (85% low grade), 4.1% Embryonal tumours and 0.1% choroid plexus carcinoma. Incidence rates vary widely across European regions especially for astrocytic tumours ranging from 3/100,000 in Eastern Europe to 5/100,000 in United Kingdom and Ireland. Overall, about 27,700 new rare CNS cancers were estimated every year in EU27, for an annual incidence rate of 4.8 per 100,000 for astrocytic, 0.4 for oligodendroglial, 0.2 for ependymal and embryonal tumours and less than 0.1 for choroid plexus carcinoma. More than 154,000 persons with rare CNS were estimated alive (prevalent cases) in the EU at the beginning of 2008. Five-year relative survival was 14.5% for astrocytic tumours (42.6% for low grade, 4.9% for high grade and 17.5% for glioma NOS), 54.5% for oligodendroglial (64.9% high grade and 29.6% low grade), 74.2% for ependymal (80.4% low grade and 36.6% high grade), 62.8% for choroid plexus carcinomas and 56.8% for embryonal tumours. Survival rates for astrocytic tumours were relatively higher in Northern and Central Europe than in Eastern Europe and in UK and Ireland. The different availability of diagnostic imaging techniques and/or radiation therapy equipment across Europe may contribute to explain the reported survival differences. The estimated proportion of cured patients was 7.9% for the 'glial' group to which belong astrocytic tumours. Overall results are strongly influenced by astrocytic tumours that are the most common type. This is the first study to delineate the rare CNS cancer burden in Europe by age, sex and European region.

Correlation of clinical findings and results of percutaneous balloon compression for patients with trigeminal neuralgia.

OBJECTIVE: To investigate pain relief and recurrence after percutaneous balloon compression (PBC) and its association with type of pain, prior surgery, or other clinical factors. METHODS: Fifty-nine patients with medically refractory trigeminal pain were enrolled into this study. Patients were divided into those with typical trigeminal neuralgia (TN), and those with other types of trigeminal pain or "atypical pain." The post-surgical rate of recurrence was estimated by the Kaplan-Meier method. Cox-proportional hazards models were used to investigate associations between patient characteristics and recurrence of pain. RESULTS: Forty-two patients had TN, 17 patients had atypical pain. At last follow-up, 40 patients had excellent, 9 good, 7 fair and 3 poor pain relief. Recurrence was observed in 35 patients, and was associated with pain type (relative risk (RR)=2.38, 95% confidence interval (CI): 1.22-4.63, P=0.011) and pain duration before PBC (RR=1.33, 95% CI: 1.02-1.72, P=0.033). Other clinical factors were not significant. Two patients had transient paresis of the sixth cranial nerve, however, there were no permanent post-surgical complications. CONCLUSIONS: Our study demonstrates the safety and efficacy of PBC with 83% of patients being pain free at last follow-up. Patients with atypical pain and longer pre-surgical symptom duration appear to have a higher risk of recurrence. Repeat surgery is just as effective as initial surgery, justification for being conservative in parameter selection at the initial procedure to minimize complications.

Blood flow velocity in the arteries of the anterior cerebral artery complex in patients with an azygos anterior cerebral artery aneurysm: a transcranial color-coded sonography study.

OBJECTIVE: It is presumed that increased blood flow through the single azygos anterior cerebral artery (Az) may contribute to the formation of an Az aneurysm. The aim of this study was to assess the blood flow velocities in the arteries of the anterior cerebral artery (ACA) complex in patients with the Az aneurysm. PATIENTS AND METHODS: A series of three patients (2 men, aged: 65, 52 and 41) with an aneurysm (unruptured in two cases) of the distal Az was examined. Blood flow velocities in the Az and the A1 segment of the ACA were measured by means of a transcranial color-coded duplex sonography (TCCS) and the Az to A1 segment (Az/A1) velocity ratio was calculated. The control group consisted of 22 healthy subjects (mean age: 44 years). RESULTS: There was a trend toward decreased (p=0.06) mean blood flow velocity in the Az compared to the A2 segment of the ACA of the control group. Blood velocity in the A1 segment did not differ between the study and control groups. Pulsatility and resistance indices in the Az were similar to those in the A2 segment of the control group. There were no differences between the Az/A1 ratio in the study group and the A2/A1 velocity ratio in the control group. CONCLUSION: Our results suggest that Az aneurysms are not associated with increased blood flow velocity in the Az. Possibly, a hemodynamic stress related to the Az bifurcation geometry, together with a bent course of this artery around the genu of the corpus callosum, predispose to aneurysm formation.

MR imaging of brainstem atrophy in progressive supranuclear palsy.

BACKGROUND: To enhance the sensitivity and specificity of the clinical diagnosis of progressive supranuclear palsy (PSP), neuroradiological parameters established in pathologically proven cases are needed. METHODS: We examined brainstem atrophy in five pathologically confirmed PSP patients (three men, mean age at death 77 years, range 64-84 years). Time interval between symptom onset and MRI ranged from 1 to 5 years, and between MRI and death from 33 to 52 months. Only one patient had clinical diagnosis of PSP at the time of MRI. Control group consisted of 19 age- and gender-matched healthy subjects. Seventeen morphometric parameters of the midbrain and pons were measured on T1-weighted midsagittal and T2-weighted axial MRI scans with Image Analyzer. Measurements of superior cerebellar peduncle (SCP) width were performed on PSP autopsy specimens. RESULTS: Mean SCP width on MRI in PSP (2.7 +/- 0.8 mm, 95%CI: 2.1-3.3) was smaller than in controls (3.7 +/- 0.5 mm, 95%CI: 3.5-3.9). Mean SCP width at autopsy was 8% smaller than mean SCP width on MRI. Midsagittal midbrain area in PSP (99.1 +/- 6.9 mm(2), 95%CI: 90.5-107.6) was smaller than in controls (141.0 +/- 18.1 mm(2), 95%CI: 132.2-149.7). Midbrain/pons area ratio in PSP was 1:5 and in controls was 1:4 (p < 0.01). Repeat MRI 17 months later in one PSP case revealed 30% decrease of SCP width. CONCLUSIONS: MR imaging with quantitative analysis may be useful in the diagnosis of early PSP and in monitoring disease course.

Aldehydic lipid peroxidation products in human brain astrocytomas.

Among mediators of oxidative stress, highly reactive secondary aldehydic lipid peroxidation products can initiate the processes of spontaneous mutagenesis and carcinogenesis and can also act as a growth-regulating factors and signaling molecules. We explored whether these aldehydes and histone H3 mRNA levels could serve as biomarkers of malignancy and predictive factor in human brain astrocytomas. Histone H3 mRNA, a biomarker of cellular proliferation, was analyzed by QRT-PCR (TaqMan). Aldehydic lipid peroxidation products were determined as their dinitrophenylhydrazone derivatives in specimens obtained from 26 adult patients with brain astrocytomas. RP-HPLC with diode array detector and MSMS spectrometer were used for the analysis. H3 mRNA, 2-hydroxyhexanal, and 4-hydroxynonenal levels were higher in high-grade astrocytomas compared to low-grade astrocytomas and showed negative correlation with survival. Higher levels of 2-hydroxyhexanal and 4-hydroxynonenal, and lower levels of n-hexanal were associated with poorer patient prognosis. Our data suggest that tissue concentrations of aldehydic lipid peroxidation products can assist grading and predicting the clinical outcome in patients with astrocytic brain tumors. Possibly, this parameter will enhance optimal selection of patients for individualized treatment protocols, tailored to unique biochemical and molecular profile of the tumor.

Unilateral deep brain stimulation of the subthalamic nucleus for Parkinson disease.

OBJECT: The object of this study was to assess the results of unilateral deep brain stimulation (DBS) of the subthalamic nucleus (STN) for management of advanced Parkinson disease (PD). METHODS: A clinical series of 24 patients (mean age 71 years, range 56-80 years) with medically intractable PD, who were undergoing unilateral magnetic resonance imaging-targeted, electrophysiologically guided STN DBS, completed a battery of qualitative and quantitative outcome measures preoperatively (baseline) and postoperatively, using a modified Core Assessment Program for Intracerebral Transplantations protocol. The mean follow-up period was 9 months. Statistically significant improvement was observed in the Unified Parkinson's Disease Rating Scale (UPDRS) Part II score (18%), the total UPDRS PART III score (31%), the contralateral UPDRS Part III score (63%), and scores for axial motor features (19%), contralateral tremor (88%), rigidity (60%), bradykinesia (54%), and dyskinesia (69%), as well as the Parkinson's Disease Quality of Life questionnaire score (15%) in the on-stimulation state compared with baseline. Ipsilateral symptoms improved by approximately 15% or less. Performance on the Purdue pegboard test improved in the contralateral hand in the on-stimulation state compared with the off-stimulation state (38%, p < 0.05). The daily levodopa-equivalent dose was reduced by 21% (p = 0.018). Neuropsychological tests revealed an improvement in mental flexibility and a trend toward reduced letter fluency. There were no permanent surgical complications. Of the 16 participants with symmetrical disease, five required implantation of the DBS unit on the second side. CONCLUSIONS: Unilateral STN DBS is an effective and safe treatment for selected patients with advanced PD. Unilateral STN DBS provides improvement of contralateral motor symptoms of PD as well as quality of life, reduces requirements for medication, and possibly enhances mental flexibility. This method of surgical treatment may be associated with a reduced risk and may provide an alternative to bilateral STN DBS for PD, especially in older patients or patients with asymmetry of parkinsonism.

Frontotemporal dementia and Parkinsonism linked to chromosome 17 with the N279K tau mutation.

We present a case of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) harboring the N279K mutation in the MAPT gene from the family known as pallido-ponto-nigral degeneration (PPND). This 49-year-old man was followed for 17 years. He presented at age 41 years with left leg stiffness and en-bloc turning. During the course of his illness he developed a constellation of symptoms including parkinsonism, pyramidal signs, vertical gaze palsy, dysphagia, dystonia, personality and cognitive dysfunction, weight loss and mutism. Gross neuropathological examination showed mild atrophy of the cerebral cortex, hippocampal formation, amygdala, thalamus, subthalamic nucleus and depigmentation of the substantia nigra. Microscopy revealed neuronal loss and gliosis in the same regions. Tau immunohistochemistry showed pretangles, numerous threads, grain-like structures and oligodendroglial tau-positive inclusions ("coiled bodies"). In the spinal cord the tau pathology was more abundant in gray than white matter. Pretangles and threads were present in the anterior and, to a lesser extent, in the posterior horns. FTDP-17 should be suspected in patients with a history of familial parkinsonism combined with behavioral and cognitive changes, onset before age 65 years and an aggressive clinical course.

Clinical-pathologic study of biomarkers in FTDP-17 (PPND family with N279K tau mutation).

The objective of this clinical-pathologic study was to identify biomarkers for a pallidopontonigral degeneration (PPND) kindred of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) harboring the N279K tau mutation. Five affected subjects, one at-risk who later became symptomatic, and one at-risk asymptomatic mutation carrier, had abnormal (18)fluorodeoxyglucose PET demonstrating asymmetric temporal lobe hypometabolism. All except the asymptomatic mutation carrier had abnormal brain MRI. Parkinsonism, myoclonus, anosmia, insomnia, speech, and autonomic dysfunction were identified. Autopsy of six affected subjects showed frontotemporal degeneration with extensive tauopathy. Further studies of FTDP-17 patients are needed to replicate these findings.