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Neuro-degenerative Diseases

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https://www.readbyqxmd.com/read/28490024/a-novel-ataxic-mutant-mouse-line-having-sensory-neuropathy-shows-heavy-iron-deposition-in-kidney
#1
Hisashi Hashimoto, Tomonori Kawabe, Takahiro Fukuda, Moriaki Kusakabe
BACKGROUND/AIMS: A novel ataxic mouse line was established from the offspring of a male mouse administered cyclophosphamide in a juvenile period. METHODS: We have attempted to examine the phenotype and histopathological changes of affected mice. Furthermore, linkage analysis and sequencing of the mutant was performed to reveal the causative gene locus. RESULTS AND CONCLUSION: The affected mouse was characterized by heavy hind limb ataxia with gait disorder, which was first recognized at about 4 weeks of age and slowly progressed with advancing age...
May 11, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28478440/elevated-levels-of-selenium-species-in-cerebrospinal-fluid-of-amyotrophic-lateral-sclerosis-patients-with-disease-associated-gene-mutations
#2
Jessica Mandrioli, Bernhard Michalke, Nikolay Solovyev, Peter Grill, Federica Violi, Christian Lunetta, Amelia Conte, Valeria Ada Sansone, Mario Sabatelli, Marco Vinceti
BACKGROUND: Although an increasing role of genetic susceptibility has been recognized, the role of environmental risk factors in amyotrophic lateral sclerosis (ALS) etiology is largely uncertain; among neurotoxic chemicals, epidemiological and biological plausibility has been provided for pesticides, the heavy metal lead, the metalloid selenium, and other persistent organic pollutants. Selenium involvement in ALS has been suggested on the basis of epidemiological studies, in vitro investigations, and veterinary studies in which selenium induced a selective toxicity against motor neurons...
May 6, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28467996/increased-risk-of-bullous-pemphigoid-after-first-ever-stroke-a-population-based-study
#3
Ai-Ling Shen, Hsiu-Li Lin, Hsiu-Chen Lin, Yuan-Fu Tseng, Chien-Yeh Hsu, Che-Yi Chou
BACKGROUND: We hypothesize that autoantibodies are induced after the blood-brain barrier is damaged by stroke and the risk of bullous pemphigoid (BP) is increased after stroke. We assess the risk of BP after first-ever stroke in a nationwide population-based cohort of first-ever stroke patients. METHODS: We extracted data from the Longitudinal Health Insurance Database 2005 and identified patients with first-ever stroke as well as control patients matched for age, gender, and year of enrollment...
May 4, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28463835/neurodegeneration-and-the-cerebellum
#4
Margot Samson, Daniel O Claassen
BACKGROUND: The cerebellum modulates diverse neuronal networks, regulating motor, cognitive, behavioral, and limbic circuits. Structural and functional changes to the cerebellum that occur with neurodegenerative conditions have not been systematically reviewed. OBJECTIVE: We synthesize the current understanding of cerebellar somatotopy and function with previously described cerebellar changes in neurodegenerative diseases not associated with primary cerebellar pathology...
May 3, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28445885/patterns-of-microglial-cell-activation-in-alzheimer-disease-and-frontotemporal-lobar-degeneration
#5
Ricardo Taipa, Paulo Brochado, Andrew Robinson, Inês Reis, Patrício Costa, David M Mann, Manuel Melo Pires, Nuno Sousa
AIMS: Microglia-driven neuroinflammation can play an important role in the pathophysiology of neurodegenerative disorders. In this study, we sought to characterize the distribution of microglial cell activation in 2 neurodegenerative dementias with distinct protein signatures, Alzheimer disease (AD) and frontotemporal lobar degeneration (FTLD) of the TDP subtype, and to determine if there was an anatomical correlation with the phenotypes most commonly associated with these conditions...
April 27, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28441649/home-based-physical-behavior-in-late-stage-parkinson-disease-dementia-differences-between-cognitive-subtypes
#6
Bernhard Cerff, Walter Maetzler, Patricia Sulzer, Malte Kampmeyer, Jos Prinzen, Markus A Hobert, Dominik Blum, Rob van Lummel, Silvia Del Din, Susanne Gräber, Daniela Berg, Inga Liepelt-Scarfone
BACKGROUND: For the early diagnosis of Parkinson disease dementia (PDD), objective home-based tools are needed to quantify even mild stages of dysfunction of the activities of daily living (ADL). OBJECTIVES: In this pilot study, home-based physical behavior was assessed to examine whether it is possible to distinguish mild cognitive impairment (PD-MCI) from PDD. METHODS: Fifty-five patients with mild to severe Parkinson disease (PD) participated in this cross-sectional study...
April 26, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28268209/patterns-of-eye-movement-impairment-correlate-with-regional-brain-atrophy-in-neurodegenerative-parkinsonism
#7
Olga Vintonyak, Martin Gorges, Hans-Peter Müller, Elmar H Pinkhardt, Albert C Ludolph, Hans-Jürgen Huppertz, Jan Kassubek
BACKGROUND: One common feature of neurodegenerative parkinsonism including Parkinson's disease (PD), multisystem atrophy (MSA), and progressive supranuclear palsy (PSP) is altered eye movement control. Characteristic regional structural atrophy patterns in MRI can be observed in PD, MSA, and PSP. OBJECTIVE: To investigate the association between eye movement disturbances and regional brain atrophy in patients with PD, MSA, and PSP. METHODS: High-resolution 3-dimensional T1-weighted MRI images and video-oculographic recordings (EyeLink®) were obtained from 39 PD, 32 PSP, and 18 MSA patients and 24 matched healthy control subjects...
March 8, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28245482/neuropsychiatric-symptoms-in-alzheimer-disease-vascular-dementia-and-mixed-dementia
#8
Cassandra J Anor, Sean O'Connor, Amardeep Saund, David F Tang-Wai, Ron Keren, Maria Carmela Tartaglia
BACKGROUND/AIMS: Neuropsychiatric symptoms (NPS) are common in Alzheimer disease (AD) and vascular dementia (VaD), and are distressful to patients and caregivers. NPS are likely related to the underlying pathology. Previous studies suggest that frontal lobe lesions and vascular changes such as white matter hyperintensities (WMH) have a significant association with specific NPS. The current study aimed to compare NPS in patients with AD, VaD, and mixed AD/VaD, and to evaluate the differences in the prevalence of NPS in relation to frontal WMH volume...
March 1, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28297705/mechanisms-clinical-strategies-and-promising-treatments-of-neurodegenerative-diseases-13th-international-conference-ad-pdtm-vienna-austria-march-29-to-april-2-2017-abstracts
#9
(no author information available yet)
No abstract text is available yet for this article.
2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28122372/environmental-and-occupational-exposures-and-amyotrophic-lateral-sclerosis-in-new-england
#10
Angeline S Andrew, Tracie A Caller, Rup Tandan, Eric J Duell, Patricia L Henegan, Nicholas C Field, Walter G Bradley, Elijah W Stommel
BACKGROUND: Recent data provide support for the concept that potentially modifiable exposures are responsible for sporadic amyotrophic lateral sclerosis (ALS). OBJECTIVE: To evaluate environmental and occupational exposures as risk factors for sporadic ALS. METHODS: We performed a case-control study of ALS among residents of New England, USA. The analysis compared questionnaire responses from 295 patients with a confirmed ALS diagnosis to those of 225 controls without neurodegenerative illness...
2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28103595/identification-of-small-peptides-in-human-cerebrospinal-fluid-upon-amyloid-%C3%AE-degradation
#11
Naoki Mizuta, Kanta Yanagida, Takashi Kodama, Takeshi Tomonaga, Mako Takami, Hiroshi Oyama, Takashi Kudo, Manabu Ikeda, Masatoshi Takeda, Shinji Tagami, Masayasu Okochi
BACKGROUND: Amyloid-β (Aβ) degradation in brains of Alzheimer disease patients is a crucial focus for the clarification of disease pathogenesis. Nevertheless, the mechanisms underlying Aβ degradation in the human brain remain unclear. OBJECTIVE: This study aimed to quantify the levels of small C-terminal Aβ fragments generated upon Aβ degradation in human cerebrospinal fluid (CSF). METHODS: A fraction containing small peptides was isolated and purified from human CSF by high-pressure liquid chromatography...
2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/27883992/precuneal-thickness-and-depression-in-parkinson-disease
#12
Stefano Zanigni, Luisa Sambati, Stefania Evangelisti, Claudia Testa, Giovanna Calandra-Buonaura, David Neil Manners, Rossana Terlizzi, Roberto Poda, Federico Oppi, Raffaele Lodi, Pietro Cortelli, Caterina Tonon
BACKGROUND: Depression-related gray matter changes in Parkinson disease (PD) patients have been reported, although studies investigating cortical thickness in early-stage disease are lacking. OBJECTIVE: We aimed to evaluate cortical changes related to depression in early-stage PD patients with an extensive neuropsychological evaluation. METHODS: 17 PD patients and 22 healthy controls underwent a 1.5-T brain MR protocol, and voxel-wise differences in cortical thickness among patients with (n = 6) and without (n = 11) depression and controls were evaluated using FreeSurfer software...
2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/27784025/arterial-stiffness-and-cardiovascular-autonomic-dysfunction-in-patients-with-parkinson-s-disease
#13
Joong-Seok Kim, Si-Hoon Lee, Yoon-Sang Oh, Jeong-Wook Park, Jae-Young An, Hyun-Seok Choi, Kwang-Soo Lee
BACKGROUND: Pulse wave velocity is a marker of arterial stiffness and a surrogate marker of vascular damage. Autonomic abnormalities associated with blood pressure are relatively commonly observed in patients with Parkinson's disease (PD). OBJECTIVE: The purpose of this study was to compare arterial stiffness between patients with PD and controls and investigate the associations between cardiovascular autonomic dysfunction and pulse wave velocity in PD. METHODS: One hundred twenty-five PD patients without diabetes mellitus were enrolled into this study, along with 22 age-matched controls...
2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/27760431/visual-hallucination-and-pattern-of-brain-degeneration-in-parkinson-s-disease
#14
Woong-Woo Lee, Eun Jin Yoon, Jee-Young Lee, Sun-Won Park, Yu Kyeong Kim
BACKGROUND AND OBJECTIVES: The incidence of visual hallucination (VH) increases with Parkinson's disease (PD) progression, and its development is thought to be related to a specific neurodegenerative process in PD. This study aimed to reveal brain degeneration related to VH in PD by analyzing neuroimaging data obtained from patients in their different stages of PD. METHODS: Data from 48 PD patients - 21 nondemented without VH (PNV group), 10 nondemented with VH (PV group), and 17 demented with VH (PVD group) - and 30 age-matched healthy controls (HC group) were analyzed...
2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/27760429/cerebrospinal-fluid-progranulin-but-not-serum-progranulin-is-reduced-in-grn-negative-frontotemporal-dementia
#15
Carlo Wilke, Frank Gillardon, Christian Deuschle, Markus A Hobert, Iris E Jansen, Florian G Metzger, Peter Heutink, Thomas Gasser, Walter Maetzler, Cornelis Blauwendraat, Matthis Synofzik
BACKGROUND AND OBJECTIVE: Reduced progranulin levels are a hallmark of frontotemporal dementia (FTD) caused by loss-of-function (LoF) mutations in the progranulin gene (GRN). However, alterations of central nervous progranulin expression also occur in neurodegenerative disorders unrelated to GRN mutations, such as Alzheimer's disease. We hypothesised that central nervous progranulin levels are also reduced in GRN-negative FTD. METHODS: Progranulin levels were determined in both cerebrospinal fluid (CSF) and serum in 75 subjects (37 FTD patients and 38 controls)...
2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/27760426/cl-nqtrp-alleviates-tauopathy-symptoms-in-a-model-organism-through-the-inhibition-of-tau-aggregation-engendered-toxicity
#16
Moran Frenkel-Pinter, Sharon Tal, Roni Scherzer-Attali, Malak Abu-Hussien, Idan Alyagor, Tal Eisenbaum, Ehud Gazit, Daniel Segal
Alzheimer's disease (AD) is the most abundant tauopathy and is characterized by Aβ-derived plaques and tau-derived tangles, resulting from the unfolding of the corresponding monomeric subunits into ordered β-sheet oligomers and fibrils. Intervening in the toxic aggregation process is a promising therapeutic approach, but, to date, a disease-modifying therapy is neither available for AD nor for other tauopathies. Along these lines, we have previously demonstrated that a small naphthoquinone-tryptophan hybrid, termed NQTrp, is an effective modulator of tauopathy in vitro and in vivo...
2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/27614955/differential-progression-of-midbrain-atrophy-in-parkinsonism-longitudinal-mri-study
#17
Minho Hwang, Hyunwoo Yang, Younsoo Kim, Jinyoung Youn, Jongkyu Park, Young Eun Huh, Hee-Tae Kim, Jin Whan Cho
AIMS: To elucidate different patterns of progression of midbrain atrophy in patients with Richardson's syndrome (RS), progressive supranuclear palsy-parkinsonism (PSP-P), and Parkinson's disease (PD) using magnetic resonance imaging (MRI)-based visual rating indexes. METHODS: We recruited 12 patients with PSP-RS, 12 with PSP-P, and 23 with PD for whom MRIs had been followed up for at least 2 years (mean ± SD, 4.9 ± 1.6 years) after the initial MRI. MRI-based visual rating indexes were used to estimate midbrain atrophy, including the ratio of the pontine to midbrain tegmental areas (P/M ratio) on a midsagittal image, the length between the interpeduncular fossa and the center of the cerebral aqueduct at the midmammillary-body level (MTEGM) on axial images, and the morning glory sign...
2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/27710962/voltage-gated-potassium-channel-antibodies-in-slow-progression-motor-neuron-disease
#18
Massimiliano Godani, Marco Zoccarato, Alessandro Beronio, Luigi Zuliani, Luana Benedetti, Bruno Giometto, Massimo Del Sette, Elisa Raggio, Roberta Baldi, Angela Vincent
BACKGROUND: The spectrum of autoimmune neurological diseases associated with voltage-gated potassium channel (VGKC)-complex antibodies (Abs) ranges from peripheral nerve disorders to limbic encephalitis. Recently, low titers of VGKC-complex Abs have also been reported in neurodegenerative disorders, but their clinical relevance is unknown. OBJECTIVE: The aim of the study was to explore the prevalence of VGKC-complex Abs in slow-progression motor neuron disease (MND)...
October 7, 2016: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/27617773/brain-derived-neurotrophic-factor-facilitates-functional-recovery-from-als-cerebral-spinal-fluid-induced-neurodegenerative-changes-in-the-nsc-34-motor-neuron-cell-line
#19
Shanmukha Shruthi, R Sumitha, Anu Mary Varghese, S Ashok, B K Chandrasekhar Sagar, T N Sathyaprabha, A Nalini, Boris W Kramer, Trichur R Raju, K Vijayalakshmi, Phalguni Anand Alladi
BACKGROUND: The survival of motor neurons is dependent upon neurotrophic factors both during childhood and adolescence and during adult life. In disease conditions, such as in patients with amyotrophic lateral sclerosis (ALS), the mRNA levels of trophic factors like brain-derived neurotrophic factor (BDNF), insulin-like growth factor-1 (IGF-1), fibroblast growth factor-2 (FGF-2), and vascular endothelial growth factor are downregulated. This was replicated in our in vivo experimental system following the injection of cerebral spinal fluid (CSF) of sporadic ALS (ALS-CSF) patients...
September 13, 2016: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/27614874/clinical-impact-of-123i-ioflupane-spect-datscan-in-a-movement-disorder-center
#20
Allison K Graebner, Daniel Tarsy, Ludy C Shih, Veronique Vanderhorst, Omesh Kulkarni, Scott Kaplan, David K Simon
BACKGROUND/AIMS: The clinical diagnosis of degenerative forms of parkinsonism is imperfect, with past studies reporting a high rate of misdiagnosis by neurologists and movement disorder specialists, particularly early in the disease course. 123I-ioflupane SPECT (DaTscan) is a diagnostic neuroimaging tool used to distinguish essential tremor from tremor due to degenerative parkinsonisms. The present study expands upon prior studies of the clinical impact of DaTscan imaging in movement disorder centers by assessing quantitative estimates of diagnostic certainty, the impact on subsequent clinical decisions, and the degree to which the asymmetry in the results corresponds to laterality by clinical history and examination...
September 10, 2016: Neuro-degenerative Diseases
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