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  • 1
    Language: English
    In: Cell Calcium, August 2013, Vol.54(2), pp.132-143
    Description: Amyotrophic lateral sclerosis is a neurodegenerative disease characterized by a progressive loss of motor neurons. Although the etiology remains unclear, disturbances in Ca homoeostasis and protein folding are essential features of neurodegeneration. The correct folding of proteins is managed by folding proteins, which are regulated by Ca levels. Therefore, Ca -sensitive folding proteins represent an important link between disturbed Ca handling and protein misfolding in amyotrophic lateral sclerosis. In the first part of this review, we focus on Ca handling in the endoplasmic reticulum and mitochondria in terms of their roles in protein misfolding. In the second part, we draw attention to the main Ca -sensitive folding proteins that play a role in motor neuron degeneration such as calreticulin and calnexin, which are involved in the folding of glycosylated proteins. In addition, calmodulin and the Ca /calmodulin-dependent protein kinase are discussed as one correlation to oxidative stress. The heat shock protein endoplasmin is associated with the anti-apoptotic insulin-like growth factor pathway that is altered in amyotrophic lateral sclerosis. Grp78, which influences Ca homeostasis in the intraluminal endoplasmic reticulum is upregulated in mice models and amyotrophic lateral sclerosis patients and constitutes a core component of the unfolded protein response. Lastly, the protein disulfide isomerase family is responsible for mediating oxidative protein folding in the endoplasmic reticulum.
    Keywords: Protein Misfolding ; Calcium ; Grp78 ; Calreticulin ; Sod1 ; Endoplasmin ; Medicine ; Biology ; Anatomy & Physiology
    ISSN: 0143-4160
    E-ISSN: 1532-1991
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  • 2
    Language: English
    In: PLoS ONE, 01 January 2014, Vol.9(8), p.e104894
    Description: Amyotrophic lateral sclerosis (ALS) is characterized by progressive loss of upper and lower motor neurons. Advanced MRI techniques such as diffusion tensor imaging have shown great potential in capturing a common white matter pathology. However the sensitivity is variable and diffusion tensor imaging is not yet applicable to the routine clinical environment. Voxel-based morphometry (VBM) has revealed grey matter changes in ALS, but the bias-reducing algorithms inherent to traditional VBM are not optimized for the assessment of the white matter changes. We have developed a novel approach to white matter analysis, namely voxel-based intensitometry (VBI). High resolution T1-weighted MRI was acquired at 1.5 Tesla in 30 ALS patients and 37 age-matched healthy controls. VBI analysis at the group level revealed widespread white matter intensity increases in the corticospinal tracts, corpus callosum, sub-central, frontal and occipital white matter tracts and cerebellum. VBI results correlated with disease severity (ALSFRS-R) and patterns of cerebral involvement differed between bulbar- and limb-onset. VBI would be easily translatable to the routine clinical environment, and once optimized for individual analysis offers significant biomarker potential in ALS.
    Keywords: Sciences (General)
    E-ISSN: 1932-6203
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  • 3
    Language: English
    In: PLoS ONE, 01 January 2015, Vol.10(6), p.e0131114
    Description: Amyotrophic lateral sclerosis (ALS) is a fatal, progressive neurodegenerative disorder, characterised by widespread white matter damage. There is growing evidence that disturbances in iron metabolism contribute to white matter alterations.We analysed the data of susceptibility-weighted imaging (SWI) of white matter in a cohort of 27 patients with ALS and 30 healthy age-matched controls.Signal alterations were found on SWI in the corpus callosum; along the corticospinal tract (subcortical motor cortex, posterior limb of the internal capsule and brainstem levels) and in the subgyral regions of frontal, parietal, temporal, occipital and limbic lobes. Alterations of white matter in the corpus callosum correlated with disease severity as assessed by the revised ALS functional rating scale.SWI is capable of indicating iron and myelin disturbances in white matter of ALS patients. The SWI patterns observed in this study suggest that widespread alterations due to iron disturbances occur in patients with ALS and correlate with disease severity.
    Keywords: Sciences (General)
    E-ISSN: 1932-6203
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  • 4
    Language: English
    In: The Open Critical Care Medicine Journal, 07/07/2011, Vol.4(1), pp.24-27
    Keywords: Medicine;
    ISSN: 18748287
    Source: Bentham Science (via CrossRef)
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  • 5
    Language: English
    In: Frontiers in Neurology, March 12, 2018
    Description: Parkinson's disease (PD) is a common, progressive and multisystem neurodegenerative disorder characterized by motor and non-motor symptoms. Advanced magnetic resonance imaging, positron emission tomography, and functional magnetic resonance imaging can render the view toward understanding the neural basis of these non-motor syndromes, as they help to understand the underlying pathophysiological abnormalities. This review provides an up-to-date description of structural and functional brain alterations in patients with PD with cognitive deficits, visual hallucinations, fatigue, impulsive behavior disorders, sleep disorders, and pain.
    Keywords: Magnetic Resonance Imaging -- Usage ; Parkinson Disease -- Research ; Parkinson Disease -- Development And Progression ; Positron Emission Tomography -- Usage
    ISSN: 1664-2295
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  • 6
    Language: English
    In: BMC public health, 29 July 2019, Vol.19(1), pp.1012
    Description: Poor adherence is a major issue and is associated with increased morbidity, mortality, and immense costs for the healthcare system. Due to demographic changes, the burden of neurological diseases is increasing with a crucial exacerbation of the problem of nonadherence. However, comprehensive data on geriatric patients with neurological disorders do not exist to date. In this cross-sectional observational study we aim to identify disease-specific adherence-modulating factors in neurogeriatric patients. Patients 60 years or older with neurological disorders will receive an assessment of adherence (Stendal Adherence with Medication Score) and a comprehensive geriatric assessment during their stay in the Department of Neurology or Geriatrics at the Jena University Hospital (baseline data). In addition disease specific data will be derived from medical records. After one and twelve months a telephone interview will be conducted to evaluate if and why changes of medication occurred (follow up data). This study aims to explore disease-specific patterns of nonadherence in elderly patients with neurological disorders and characteristics of information transfer between a specialized center, practicing neurologists, general practitioners, and the patients and their caregivers. This comprehensive data may help to develop and apply complex and disease-specific interventions to enhance adherence. German Clinical Trials Register DRKS00016774. Registered 19.02.2019.
    Keywords: Elderly ; Health-Related Quality of Life ; Nonadherence ; Parkinson’s Disease ; Stroke ; Medication Adherence -- Statistics & Numerical Data ; Nervous System Diseases -- Drug Therapy
    E-ISSN: 1471-2458
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  • 7
    Language: English
    In: Social Indicators Research, 2015, Vol.120(3), pp.871-882
    Description: Quality of life (QOL) is an important issue in patients with amyotrophic lateral sclerosis (ALS). QOL measurements can help the support care team understand how to maintain or improve QOL in ALS patients. The purpose of this study was to describe the relationship between health-related QOL, disease severity and social support in ALS patients. In the current study, 49 German ALS patients were evaluated using the European quality of life score (EQ-5D), ALS Functional Rating Scale in its revised form (ALSFRS-R), Social Support Questionnaire (F-SozU K-14) and the Beck depression inventory. Data concerning patient history and socioeconomic background were collected using a semi-structured interview. Age, gender, number of children, habitation, socioeconomic status and disease duration were not significantly related to health-related QOL (EQ-5D). Positive correlations were found between the ALSFRS-R, social support and health-related QOL, whereas depression was negatively correlated with the ALSFRS-R score. A multiple linear regression model indicated a significant influence of the ALSFRS-R score on health-related QOL in ALS patients, with an additional trend for social support as a predictor. These results suggest that because of the logarithmic association between measures, compensatory therapy for loss of health-related QOL should be optimised during the course of the disease in ALS patients.
    Keywords: Quality of life ; Health-related quality of life ; Social support ; ALSFRS-R ; Motor neuron disease
    ISSN: 0303-8300
    E-ISSN: 1573-0921
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  • 8
    Language: English
    In: Journal of Neurology, Neurosurgery & Psychiatry, 03/24/2016, p.jnnp-2015-312185
    ISSN: 0022-3050
    E-ISSN: 1468-330X
    Source: CrossRef
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  • 9
    Language: English
    In: Journal of Neurology, Neurosurgery & Psychiatry, 21 March 2016
    Description: Non-invasive positive-pressure ventilation (NPPV) is an established, effective, long-term treatment for patients with amyotrophic lateral sclerosis (ALS), but the correct indicators for the establishment of NPPV have not been defined.
    Keywords: Medicine;
    ISSN: 0022-3050
    ISSN: 00223050
    E-ISSN: 1468-330X
    E-ISSN: 1468330X
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  • 10
    Language: English
    In: PLoS ONE, August 18, 2014, Vol.9(8)
    Description: Amyotrophic lateral sclerosis (ALS) is characterized by progressive loss of upper and lower motor neurons. Advanced MRI techniques such as diffusion tensor imaging have shown great potential in capturing a common white matter pathology. However the sensitivity is variable and diffusion tensor imaging is not yet applicable to the routine clinical environment. Voxel-based morphometry (VBM) has revealed grey matter changes in ALS, but the bias-reducing algorithms inherent to traditional VBM are not optimized for the assessment of the white matter changes. We have developed a novel approach to white matter analysis, namely voxel-based intensitometry (VBI). High resolution T1-weighted MRI was acquired at 1.5 Tesla in 30 ALS patients and 37 age-matched healthy controls. VBI analysis at the group level revealed widespread white matter intensity increases in the corticospinal tracts, corpus callosum, sub-central, frontal and occipital white matter tracts and cerebellum. VBI results correlated with disease severity (ALSFRS-R) and patterns of cerebral involvement differed between bulbar- and limb-onset. VBI would be easily translatable to the routine clinical environment, and once optimized for individual analysis offers significant biomarker potential in ALS.
    Keywords: Amyotrophic Lateral Sclerosis ; Magnetic Resonance Imaging
    ISSN: 1932-6203
    Source: Cengage Learning, Inc.
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