Wyniki wyszukiwania - Biblioteka Nauki

1

ALS - cichy zabojca [nasze szanse w walce ze stwardnieniem bocznym zanikowym]

100%

Jantas-Skotniczna D.

Wszechświat

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2006

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tom 107

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nr 01-03

31-33

2

Jak obronić neurony przed SLA

86%

Aebisher P. , Kato A. C.

Świat Nauki

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2007

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nr 12

3

Speech therapy for dysarthric disorders in amyotrophic lateral sclerosis

86%

Boksa E. , Żabińska K.

Logopedia Silesiana

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2022

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tom 11

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nr 1

EN

Zaprezentowany artykuł dotyczy diagnozy i terapii dyzartrii w przebiegu stwardnienia zanikowego bocznego (łac. sclerosis lateralis amyotrophica – SLA, choroba Charcota), jednej z nieuleczalnych chorób neurodegeneracyjnych. Na przykładzie 46-letniej pacjentki przeanalizowano charakterystyczne objawy choroby w odniesieniu do literatury logopedycznej. Artykuł został zaprezentowany w formie studium przypadku ze względu na małą liczbę pacjentów, u których występuje stwardnienie zanikowe boczne. Zapadalność na sporadyczną postać stwardnienia zanikowego bocznego (SSLA) wynosi średnio 1,9 na 100 000 na rok zarówno w Europie, jak i w Ameryce Północnej. Częstość występowania SLA szacuje się na około 7 na 100 000 w populacji ogólnej. W badaniu wykorzystano „Ocenę stanu dyzartrii” Izabeli Gatkowskiej, uzupełnioną o wywiad z respondentką i analizę dokumentacji medycznej, a następnie zaproponowano plan terapii.

PL

The presented paper concerns the diagnostics and therapy of dysarthria in the course of amyotrophic lateral sclerosis (ALS, sclerosis lateralis amyotrophica, SLA, Charcot disease), one of the incurable neurodegenerative diseases. On the example of a 46-year-old patient, the characteristic symptoms of the disease were analyzed in relation to the speech therapy literature. The article was presented in the form of a case study due to the small number of patients with amyotrophic lateral sclerosis. The incidence of sporadic amyotrophic lateral sclerosis (SSLA) is on average 1.9 per 100,000 per year in both Europe and North America. The incidence of SLA is estimated to be about 7 out of 100,000 in the general population. The study used the “Ocena stanu dyzartrii” [Assessment of dysarthria status] by Izabela Gatkowska was supplemented with an interview with the respondent and available medical documentation, and then a therapy plan was proposed.

4

Progressive die-back and death of motoneurons in a transgenic mouse model of amyotrophic lateral sclerosis

72%

Gordon T. , Tam S. L. , Sharp P. S. , Hegedus. J.

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tom Vol. 25, no. 4

17-26

EN

Fischer et al. [1] commented that the longest and largest nerve fibers with the highest metabolic demand appear to be the most susceptible to "dying-back" in a wide variety of degenerative and toxic conditions of the central and peripheral nervous systems. In the high copy number G93AmSOD1 transgenic mouse model of familial amyotrophic lateral sclerosis (ALS) we tested the hypothesis that the largest motoneurons which have the most terminal connections are the most susceptible to the disease. In a time course study of motoneuron, muscle and motor unit properties in fast - and slow-twitch hindlimb muscles of G93A and wild type mice, we found a rapid decline in numbers of functional motor units and motoneurons that progresses from birth to a plateau after 90-100 days of life with surprisingly Iittle compensatory axonal sprouting. Fast motoneurons are the most susceptible, contrasting with the slow motoneurons. Preliminary evidence of loss of S100 reactive perisynaptic Schwann cells at the denervated endplate regions of the affected muscles indicate loss paralIeIs rapid progression of disease with consequent decline in muscle force and motor unit numbers, folIowed closely in time by motoneuron death.

5

Analysis of matrix metalloproteinases (MMPs) in cerebrospinal fluid of patients with amyotrophic lateral sclerosis (ALS)

72%

Sokołowska B. , Jóźwik A. , Niebroj-Dobosz I. , Janik P. , Kwieciński H.

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2009

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tom Vol. 13

147--150

EN

Matrix metalloproteinases (MMPs) are implicated in the pathogenesis of motor neuron degeneration in amyotrophic lateral sclerosis (ALS) and might be potential markers of diagnosis, prognosis and monitoring treatment effects. The aim of the present study was evaluation of the MMPs significance in cerebrospinal fluid (CSF MMPs) of patients with ALS in relation to severity of the disease. Metalloproteinases MT-MMP-1, MMP-2, MMP-9 and additionally age of subjects and disease duration were analyzed. The results demonstrate that the error of differentiation between healthy subjects and ALS patients (for MMP-2 feature) as well as between mild and severe ALS states (for CSF MMPs set) equalled to 0.033. In conclusion, the pattern recognition approach may be useful for differentation of ALS progressing on the basis of CSF MMPs features.

6

Choroba na szalce

72%

Hall S. S.

Świat Nauki

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2011

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nr 04

7

Ekspresja transferazy S-glutationowej pi w transgenicznych mysich modelach neurodegeneracji

72%

Kazmierczak B. , Usarek E. , Kuzma-Kozakiewicz M. , Baranczyk-Kuzma A.

Medycyna Weterynaryjna

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2008

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tom 64

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nr 06

812-814

EN

Glutathione S-transferase pi (GST pi) inactivates a large variety of toxic, electrophilic compounds. The substrates of GST pi include environmental toxins and intracellular reactive oxygen species, factors significant in the pathogenesis of neurodegenerative diseases. The aim of the present study was to investigate the expression of glutathione S-transferase pi in transgenic mouse models of neurodegeneration on both the mRNA and protein levels. Experiments were conducted on the frontal cortex of transgenic B6-C3H hybrids SOD1, Cra1 and SOD1/Cra1, aged 70 and 140 days. The SOD1 mice express a human SOD1ᴳ⁹³ᴬ mutation, the Cra1 strain carries mutation in the cytoplasmic dynein heavy chain 1 (Dnchc1), and the double heterozygote SOD1/Cra1 mice show a delayed disease progression as well as an increased lifespan compared with the SOD1 strain. A wild strain of mice were used as a control. The expression of GST pi mRNA in younger mice (age 70 days) was found to be similar in all studied groups of animals. In older (aged 140 days) controls and Cra1 mice the GST pi expression was at a similar level and it did not significantly differ from younger animals. In SOD1 and SOD1/Cra1 strains, the mRNA-GST pi expression was lower when compared to 140-day-old controls and the Cra1 strain. Moreover, it was significantly lower than in corresponding 70-day-old animals. A decrease in the GST pi expression on the mRNA level was accompanied by a decrease in the protein level. High and unchanged GST pi expression in the frontal cortex of Cra1 mice indicates that the antioxidant-detoxification system plays an important role in protection against neurodegeneration. A significant decrease of GST pi expression in the frontal cortex of SOD1 and SOD1/Cra1 mice at the symptomatic stage of the disease suggests that the expression of this enzyme is related more to the G93A mutation in the SOD1 gene than to the efficient axonal transport.

8

Machine learning for analysis of gene expression data in fast- and slow-progressing amyotrophic lateral sclerosis murine models

72%

Iadanza E. , Fabbri R. , Goretti F. , Nardo G. , Niccolai E. , Bendotti C. , Amedei A.

Biocybernetics and Biomedical Engineering

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2022

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tom Vol. 42, no. 1

273--284

EN

Amyotrophic lateral sclerosis is a fatal motor neuron disease characterised by degenerative changes in both upper and lower motor neurons. Current treatment options in the general cohort of ALS patients have only a minimal impact on survival. Only two approved medications are available today, just addressing the management of symptoms and supporting the respiration. In this work, gene expression data from genetically modified murine motor neurons have been analysed with machine learning techniques, with the scope of distinguishing between mice developing a fast progression of the disease, and mice showing a slower progression. Results showed high accuracy (above 80%) in all tasks, with peaks of accuracy for specific ones – such as distinguishing between fast and slow progression. In the above mentioned task the best performing algorithm reached an accuracy of 100%. This research group is currently working on three more investigations on data from mice, using similar approaches and methodology, focusing on thoracic and lumbar metabolomic data as well as microbiome data. We believe that, based on the findings in the murine models, machine learning could be used to discover ALS progression markers in humans by looking at features related to the immune response. This could pave the path for the discovery of druggable targets and disease biomarkers for homogeneous ALS patient subgroups.

9

NMR-based metabonomics of cerebrospinal fluid applied to amyotrophic lateral sclerosis

58%

Toczyłowska B. , Jamrozik Z. , Liebert A. , Kwieciński H.

Biocybernetics and Biomedical Engineering

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2013

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tom Vol. 33, no. 1

21--32

EN

The aim of this study was applications of cerebrospinal fluid (CSF) NMR-based metabolic fingerprinting to amyotrophic lateral sclerosis (ALS) as possible early diagnostic tool. Two CSF sample categories were collected: 9 ALS patients and 13 age-matched control patients (without neurological disease). Metabolic profile of the CSF was determined by high resolution proton NMR spectroscopy. For statistical analysis magnitudes of 33 signals of the NMR spectrum were selected. Partial least square discriminant analysis (PLS-DA) and orthogonal PLS-DA (OPLS-DA) modeling were used to find potential biomarkers of the disease. Those analyses showed that it was possible to distinguish the ALS patients from the control ones on the basis of the CSF metabolic profile. Significantly higher levels of metabolites observed in the patients with ALS may represent the state of anaerobic metabolism and excitotoxicity.

10

Odkrywanie tajników ALS

58%

Petrucelli L. , Gitter A. D.

Świat Nauki

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2017

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nr 07

11

The pair-wise linear classifier and the k-NN rule in application to ALS progression differentiation

58%

Sokołowska B. , Jóźwik A. , Niebroj-Dobosz I. , Janik P.

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2012

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tom Vol. 20

79--83

EN

The two kinds of classifier based on the k-NN rule, the standard and the parallel version, were used for recognition of severity of ALS disease. In case of the second classifier version, feature selection was done separately for each pair of classes. The error rate, estimated by the leave one out method, was used as a criterion as for determination the optimum values of k's as well as for feature selection. All features selected in this manner were used in the standard and in the parallel classifier based on k-NN rule. Furthermore, only for the verification purpose, the linear classifier was applied. For this kind of classifier the error rates were calculated by use the training set also as a testing one. The linear classifier was trained by the error correction algorithm with a modified stop condition. The data set concerned with the healthy subjects and patients with amyotrophic lateral sclerosis (ALS). The set of several biomarkers such as erythropoietin, matrix metalloproteinases and their tissue inhibitors measured in serum and cerebrospinal fluid (CSF) were treated as features. It was shown that CSF biomarkers were very sensitive for the ALS progress.

12

Assessment of human motoneuron afterhyperpolarization duration in health and disease

58%

Piotrkiewicz M. , Kudina L. , Jason Chen J. J. , Hausmanowa-Petrusewicz I.

Biocybernetics and Biomedical Engineering

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2012

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tom Vol. 32, no. 3

43-61

EN

The results of the investigatigation of afterhyperpolarization (AHP) duration in normal aging and selected neuromuscular disorders are presented. This investigation yielded unexpected results: the AHP shortening in myogenic disease (DMD) and no significant difference from control values in neurogenic disease (ALS). However, introduction of age factor revealed novel aspects of the human ALS, which can be interpreted on the basis of the results obtained in a SOD1 mice, thus confirming usefulness of this animal model of ALS. In spastic patients the AHP was prolonged and the difference from the control AHP duration decreased with age and disease duration. Our results suggest that the match between temporal characteristics of the AHP of MN and of the twitch of its muscle unit is preserved during normal aging and in spasticity, but not in the DMD.

13

Zespół pozderzeniowy

58%

Bartholet J.

Świat Nauki

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2012

|

nr 03

14

Niezwykłe świnki

58%

Wazbinski A.

Trzoda Chlewna

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2008

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tom 46

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nr 03

15

Dysfunkcje mitochondriów w chorobach neurodegeneracyjnych: potencjalny punkt uchwytu dla leków neuroprotekcyjnych

51%

Greda A. , Jantas D.

Postępy Biologii Komórki

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2012

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tom 39

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nr 3

16

Zalążki demencji

51%

Walker L. C. , Jucker M.

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2013

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nr 06

17

A Detailed Study of EEG based Brain Computer Interface

51%

Sharma G. , Sharma N. , Singh T. , Agrawal R.

Annals of Computer Science and Information Systems

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2018

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tom Vol. 14

137--143

EN

Brain Computer Interface (BCI) generate a direct method to communicate with the outside world. Many patients are not able to communicate. For example:- the patient who are suffered with the several disease like post stroke - the process of thinking, remembering \& recognizing can be challenging. Because of spinal cord injuries or brain stem stroke the patient loss the monitoring power. EEG based brain computer interface (BCI) feature is beneficial to scale the brain movement \& convert them into a instruction for monitoring. In this paper our objective is to study about various applications of EEG based signal of the different disease like spinal cord injury, post stroke and ALS (amyotrophic lateral sclerosis) etc.

18

Lecznicze zastosowania marihuany

44%

Vetulani J.

Wszechświat

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2014

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tom 115

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nr 01-03

19

Ryby i owoce morza jako źródło narażenia człowieka na metylortęć

36%

Mania M. , Wojciechowska-Mazurek M. , Starska K. , Rebeniak M. , Postupolski J.

Roczniki Państwowego Zakładu Higieny

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2012

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tom 63

|

nr 3

PL

Ryby i owoce morza są zalecanymi składnikami diety, dostarczają one pełnowartościowego białka, witamin, soli mineralnych oraz kwasów tłuszczowych omega-3. Te środki spożywcze mogą być również źródłem pobrania metylortęci przez ludzi. Artykuł ten zawiera informacje na temat źródeł narażenia na organiczne połączenia rtęci, toksyczności, metabolizmu oraz przemian rtęci w środowisku. Przedstawiono zalecenia żywieniowe przygotowane przez Komisję Europejską i państwa członkowskie w odniesieniu do spożycia ryb drapieżnych, uwzględniające najbardziej wrażliwe grupy populacji, takie jak: kobiety planujące ciążę, ciężarne, karmiące matki oraz dzieci. Na podstawie piśmiennictwa omówiono również zanieczyszczenie ryb i owoców morza rtęcią i metylortecią. Opisano rolę selenu jako czynnika, który obniża toksyczność metylortęci jak również przedstawiono informacje o potencjalnych czynnikach etiologicznych związanych z chorobą autystyczną. W artykule zwrócono również uwagę na wzrastającą ilość powiadomień w ramach europejskiego Systemu Wczesnego Ostrzegania o Niebezpiecznej Żywności i Paszach (Rapid Alert System for Food and Feed –RASFF), dotyczących zanieczyszczenia rtęcią ryb i produktów rybnych. Omówiono także regulacje prawne dotyczące maksymalnych dopuszczalnych poziomów rtęci w żywności.

EN

Fish and seafood are recommended diet constituents providing high quality protein, vitamins, minerals and omega-3 fatty acids, mainly eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA). However, these foodstuffs can also be the major source of methylmercury intake in humans. In general, more than 90% of the mercury in fish is found as methylmercury, but contents of methylmercury can vary considerably between species. Predatory species that are at the top of the food chain and live a long time, may accumulate higher levels of methylmercury. This paper contains information about sources of human exposure to organic compounds of mercury, toxicity, metabolism and transformation of mercury in the environment. Assessment of methylmercury by international risk assessment bodies such as the Joint FAO/WHO Expert Committee on Food Additives (JECFA) and U.S. National Research Council (NRC) were presented. Climate changes and their influence on the mercury cycle in the environment especially mercury methylation and concentrations of methylmercury in marine species were also presented. Consumer advice prepared by European Commission and Member States as regards consumption of predatory fishes such as swordfish, tuna, shark, marlin and pike, taking into account the most vulnerable groups of population e.g. women planning pregnancy, pregnant or breastfeeding women and children were presented. Mercury and methylmercury contamination of fishes and seafood on the basis of the literature references as well as intake of mercury with fish and fish products in Poland and other European country were discussed. The role of selenium as a factor which counteracts methylmercury toxicity and protects against some neurological effects of methylmercury exposure in humans, as well as information on potential etiological factors connected with autism disorder were also described. Attention has also been drawn to increasing number of notifications to Rapid Alert System for Food and Feed (RASFF) concerning the contamination of fish and fish products with total mercury. European and national regulations concerning maximum permissible levels of mercury in food were also presented. Possibility of selection of different fish and seafood species, taking into account low methylmercury contamination and high contents of omega-3 fatty acids e.g. sardine, mackerel, anchovy, salmon, periwinkle, have been discussed.