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https://www.readbyqxmd.com/read/28736850/progressive-ataxia-and-palatal-tremor-two-autopsy-cases-of-a-novel-tauopathy
#1
Andrew F Gao, Achinoam Faust-Socher, Maryam Al-Murshed, Marc R Del Bigio, Anthony E Lang, David G Munoz
BACKGROUND: Sporadic progressive ataxia and palatal tremor is a rare syndrome characterized by mid- to late-adult-onset symptomatic palatal tremor and slowly progressive cerebellar ataxia. To date, there has been only one autopsy report, which described a novel 4-repeat tauopathy with hypertrophic olivary degeneration and tau-positive inclusions in olivary neurons and dystrophic neuritic processes termed glomeruloid bodies. We report on 2 additional autopsy cases. METHODS: Sections from selected paraffin-embedded brain regions were stained with hematoxylin and eosin/Luxol fast blue and processed for phosphorylated tau, 3-repeat tau, 4-repeat tau, neurofilament, glial fibrillary acid protein, phosphorylated α-synuclein, phosphorylated TAR DNA-binding protein 43, beta-amyloid, and p62 immunohistochemistry...
July 24, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28733960/a-review-of-fluid-biomarkers-for-alzheimer-s-disease-moving-from-csf-to-blood
#2
REVIEW
Kaj Blennow
A set of core cerebrospinal fluid (CSF) biomarkers for Alzheimer's disease (AD) includes total tau (T-tau), phosphorylated tau (P-tau) and β-amyloid 42 (Aβ42). These biomarkers reflect some of the key aspects of AD pathophysiology, including neuronal degeneration, tau phosphorylation with tangle formation, and Aβ aggregation with deposition of the peptide into plaques. The core AD CSF biomarkers have been validated clinically in numerous studies, and found to have a very high diagnostic performance to identify AD, both in the dementia and in the mild cognitive impairment stages of the disease...
July 2017: Neurology and Therapy
https://www.readbyqxmd.com/read/28728117/alzheimer-s-disease-markers-in-the-aged-sheep-ovis-aries
#3
Suzanne J Reid, Natasha E Mckean, Kristen Henty, Erik Portelius, Kaj Blennow, Skye R Rudiger, C Simon Bawden, Renee R Handley, Paul J Verma, Richard L M Faull, Henry J Waldvogel, Henrik Zetterberg, Russell G Snell
This study reports the identification and characterization of markers of Alzheimer's disease (AD) in aged sheep (Ovis aries) as a preliminary step toward making a genetically modified large animal model of AD. Importantly, the sequences of key proteins involved in AD pathogenesis are highly conserved between sheep and human. The processing of the amyloid-β (Aβ) protein is conserved between sheep and human, and sheep Aβ1-42/Aβ1-40 ratios in cerebrospinal fluid (CSF) are also very similar to human. In addition, total tau and neurofilament light levels in CSF are comparable with those found in human...
June 24, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28718500/neurofilament-light-as-an-immune-target-for-pathogenic-antibodies
#4
Fabiola Puentes, Baukje J van der Star, Stephanie D Boomkamp, Markus Kipp, Louis Boon, Isabel Bosca, Joel Raffel, Sharmilee Gnanapavan, Paul van der Valk, Jodie Stephenson, Susan C Barnett, David Baker, Sandra Amor
Antibodies to neuronal antigens are associated with many neurological diseases including paraneoplastic neurological disorders, epilepsy, amyotrophic lateral sclerosis and multiple sclerosis. Immunisation with neuronal antigens such as neurofilament light NF-L, a neuronal intermediate filament in axons, has been shown to induce neurological disease and spasticity in mice. Also, while antibodies to NF-L are widely used as surrogate biomarkers of axonal injury in amyotrophic lateral sclerosis and multiple sclerosis, it remains to be elucidated if antibodies to NF-L contribute to neurodegeneration and neurological disease...
July 17, 2017: Immunology
https://www.readbyqxmd.com/read/28717351/serial-sampling-of-serum-protein-biomarkers-for-monitoring-human-traumatic-brain-injury-dynamics-a-systematic-review
#5
REVIEW
Eric Peter Thelin, Frederick Adam Zeiler, Ari Ercole, Stefania Mondello, András Büki, Bo-Michael Bellander, Adel Helmy, David K Menon, David W Nelson
BACKGROUND: The proteins S100B, neuron-specific enolase (NSE), glial fibrillary acidic protein (GFAP), ubiquitin carboxy-terminal hydrolase L1 (UCH-L1), and neurofilament light (NF-L) have been serially sampled in serum of patients suffering from traumatic brain injury (TBI) in order to assess injury severity and tissue fate. We review the current literature of serum level dynamics of these proteins following TBI and used the term "effective half-life" (t1/2) in order to describe the "fall" rate in serum...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28715117/differential-activation-of-neuronal-and-glial-stat3-in-the-spinal-cord-of-the-sod1-g93a-mouse-model-of-amyotrophic-lateral-sclerosis
#6
Tomohiro Ohgomori, Ryo Yamasaki, Hideyuki Takeuchi, Kenji Kadomatsu, Jun-Ichi Kira, Shozo Jinno
Signal transducer and activator of transcription (STAT) proteins are activated by phosphorylation in the spinal cord of patients suffering from amyotrophic lateral sclerosis (ALS). The major scope of our study is a comprehensive histological characterization of the mechanisms underlying neuronal and glial STAT3 activation in the pathogenesis of ALS using SOD1(G93A) mice. We calculated the fold changes (FCs, ratios vs. appropriate controls) of the numerical densities of the following phosphorylated STAT3-positive (pSTAT3)(+) cells: choline acetyltransferase (ChAT)(+) α-motoneurons, ionized calcium-binding adapter molecule 1 (Iba1)(+) microglia, and S100β(+) astrocytes in SOD1(G93A) mice...
July 17, 2017: European Journal of Neuroscience
https://www.readbyqxmd.com/read/28711740/early-manifestations-of-epileptic-encephalopathy-brain-atrophy-and-elevation-of-serum-neuron-specific-enolase-in-a-boy-with-beta-propeller-protein-associated-neurodegeneration
#7
Kyoko Takano, Kazuya Goto, Mitsuo Motobayashi, Keiko Wakui, Rie Kawamura, Tomomi Yamaguchi, Yoshimitsu Fukushima, Tomoki Kosho
Mutations in WDR45 are responsible for beta-propeller protein-associated neurodegeneration (BPAN), which is an X-linked form of neurodegeneration with brain iron accumulation. BPAN mainly affects females and is characterized by seizures and developmental delay or intellectual disability until adolescence or early adulthood, followed by severe dystonia, parkinsonism, and progressive dementia. However, rare male patients have recently been reported with hemizygous germline mutations in WDR45 and severe clinical manifestations, such as epileptic encephalopathies...
July 12, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28709447/cryptic-amyloidogenic-elements-in-mutant-nefh-causing-charcot-marie-tooth-2-trigger-aggresome-formation-and-neuronal-death
#8
Arnaud Jacquier, Cécile Delorme, Edwige Belotti, Raoul Juntas-Morales, Guilhem Solé, Odile Dubourg, Marianne Giroux, Claude-Alain Maurage, Valérie Castellani, Adriana Rebelo, Alexander Abrams, Stephan Züchner, Tanya Stojkovic, Laurent Schaeffer, Philippe Latour
Neurofilament heavy chain (NEFH) gene was recently identified to cause autosomal dominant axonal Charcot-Marie-Tooth disease (CMT2cc). However, the clinical spectrum of this condition and the physio-pathological pathway remain to be delineated. We report 12 patients from two French families with axonal dominantly inherited form of CMT caused by two new mutations in the NEFH gene. A remarkable feature was the early involvement of proximal muscles of the lower limbs associated with pyramidal signs in some patients...
July 14, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28702437/treadmill-exercise-with-bone-marrow-stromal-cells-transplantation-potentiates-recovery-of-locomotor-function-after-spinal-cord-injury-in-rats
#9
You-Mi Kim, Tae-Beom Seo, Chang-Ju Kim, Eun-Sang Ji
Transplantation of bone marrow stromal cells (BMSCs) is regarded as a promising candidate for the spinal cord injury (SCI). In the present study, we investigated whether treadmill exercise potentiate the effect of BM-SCs transplantation on the functional recovery in the SCI rats. The spinal cord contusion injury applied at the T9-T10 level using the impactor. Cultured BMSCs were transplanted into the lesion at 1 week after SCI induction. Treadmill exercise was conducted for 6 weeks. Basso-Beattie-Bresnahan (BBB) scale for locomotor function was determined...
June 2017: Journal of Exercise Rehabilitation
https://www.readbyqxmd.com/read/28701950/cerebrospinal-fluid-a%C3%AE-43-is-reduced-in-early-onset-compared-to-late-onset-alzheimer-s-disease-but-has-similar-diagnostic-accuracy-to-a%C3%AE-42
#10
Camilla Lauridsen, Sigrid B Sando, Ina Møller, Guro Berge, Precious K Pomary, Gøril R Grøntvedt, Øyvind Salvesen, Geir Bråthen, Linda R White
Background: Amyloid beta 1-43 (Aβ43) may be a useful additional biomarker for diagnosing Alzheimer's disease (AD). We have investigated cerebrospinal fluid (CSF) levels of Aβ43 in patients with early-onset AD in contrast to levels in late-onset AD. For comparison, in addition to the 'core' biomarkers, several other analytes were also determined [YKL-40, neurofilament light (NF-L), glial fibrillary acidic protein (GFAP), and progranulin]. Material and Methods: Cerebrospinal fluid samples were obtained from patients with early-onset AD (age ≤ 62, n = 66), late-onset AD (age ≥ 68, n = 25), and groups of cognitively intact individuals (age ≤ 62, n = 41, age ≥ 68, n = 39)...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28696566/super-resolution-imaging-of-subcortical-white-matter-using-stochastic-optical-reconstruction-microscopy-storm-and-super-resolution-optical-fluctuation-imaging-sofi
#11
Atticus H Hainsworth, Sarah Lee, Peter Foot, Anita Patel, Wayne W Poon, Alex E Knight
AIMS: The spatial resolution of light microscopy is limited by the wavelength of visible light (the "diffraction limit", approximately 250 nm). Resolution of sub-cellular structures, smaller than this limit, is possible with super resolution methods such as stochastic optical reconstruction microscopy (STORM) and super-resolution optical fluctuation imaging (SOFI). We aimed to resolve subcellular structures (axons, myelin sheaths and astrocytic processes) within intact white matter using STORM and SOFI...
July 11, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28692877/association-between-cerebrospinal-fluid-and-plasma-neurodegeneration-biomarkers-with-brain-atrophy-in-alzheimer-s-disease
#12
Joana B Pereira, Eric Westman, Oskar Hansson
The aggregation and deposition of amyloid-β (Aβ) peptides into plaques is an early event in Alzheimer's disease (AD), which is followed by different aspects of neurodegeneration that can be measured in the cerebrospinal fluid (CSF) or plasma using neurofilament light (NFL), neurogranin (Ng), total Tau (T-Tau), and phosphorylated tau (P-Tau) levels. The relationship between these biomarkers and regional brain atrophy across the different stages of AD remains largely unexplored. In this study, we assessed whether NFL, Ng, T-Tau, and P-Tau levels in CSF and NFL in plasma are associated with cortical thinning and subcortical volume loss in cognitively normal, mild cognitive impairment, and AD subjects with and without Aβ pathology...
June 17, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28692534/blood-neuron-derived-exosomes-as-biomarkers-of-cognitive-impairment-in-hiv
#13
Bing Sun, Pranjali Dalvi, Linda Abadjian, Norina Tang, Lynn Pulliam
OBJECTIVE: To investigate proteins associated with neuronal damage in plasma neuron-derived exosomes (NDE) of HIV-infected subjects as a liquid biomarker for cognitive impairment. METHODS: Plasma NDE were isolated using precipitation and immunoadsorption with antibody to a cell surface specific neuronal marker. Total exosomes and NDE were enumerated, characterized and proteins extracted and targets quantified by ELISA. RESULTS: Plasma NDE from 23 HIV seropositive individuals of which 11 had mild cognitive impairment, and 12 HIV seronegative controls of which 3 had cognitive impairment were isolated...
July 7, 2017: AIDS
https://www.readbyqxmd.com/read/28689355/vitamin-d3-reverses-the-hippocampal-cytoskeleton-imbalance-but-not-memory-deficits-caused-by-ovariectomy-in-adult-wistar-rats
#14
Cassiana Siebert, Paula Pierozan, Janaina Kolling, Tiago Marcon Dos Santos, Matheus Coimbra Sebotaio, Eduardo Peil Marques, Helena Biasibetti, Aline Longoni, Fernanda Ferreira, Regina Pessoa-Pureur, Carlos Alexandre Netto, Angela T S Wyse
The objective of study was to investigate changes caused by ovariectomy (OVX) on aversive and non-aversive memories, as well as on cytoskeleton phosphorylating system and on vitamin D receptor (VDR) immunocontent in hippocampus. The neuroprotective role of vitamin D was also investigated. Ninety-day-old female Wistar rats were divided into four groups: SHAM, OVX, VITAMIN D and OVX + VITAMIN D; 30 days after the OVX, vitamin D supplementation (500 IU/kg), by gavage, for 30 days was started. Results showed that OVX impaired short-term and long-term recognition, and long-term aversive memories...
July 8, 2017: Neuromolecular Medicine
https://www.readbyqxmd.com/read/28679595/poor-sleep-is-associated-with-csf-biomarkers-of-amyloid-pathology-in-cognitively-normal-adults
#15
Kate E Sprecher, Rebecca L Koscik, Cynthia M Carlsson, Henrik Zetterberg, Kaj Blennow, Ozioma C Okonkwo, Mark A Sager, Sanjay Asthana, Sterling C Johnson, Ruth M Benca, Barbara B Bendlin
OBJECTIVE: To determine the relationship between sleep quality and CSF markers of Alzheimer disease (AD) pathology in late midlife. METHODS: We investigated the relationship between sleep quality and CSF AD biomarkers in a cohort enriched for parental history of sporadic AD, the Wisconsin Registry for Alzheimer's Prevention. A total of 101 participants (mean age 62.9 ± 6.2 years, 65.3% female) completed sleep assessments and CSF collection and were cognitively normal...
July 5, 2017: Neurology
https://www.readbyqxmd.com/read/28675259/review-of-the-novelties-from-the-32nd-ectrims-congress-2016-presented-at-the-9th-post-ectrims-meeting-ii
#16
REVIEW
O Fernandez, A Oterino, C Oreja-Guevara, J M Prieto, M M Mendibe-Bilbao, J A Garcia-Merino, Ll Ramio-Torrenta, R Ginestal, J E Meca-Lallana, L Romero-Pinel, D Munoz, P Oliva-Nacarino, M C Calles-Hernandez, G Izquierdo, M L Martinez-Gines, A Saiz, M Comabella, B Casanova-Estruch, Ll Brieva, R Arroyo, A Rodriguez-Antiguedad
For the ninth year in a row the Post-ECTRIMS Meeting has been held in Madrid (Spain) with the aim of presenting and discussing the hottest issues debated at the ECTRIMS Congress by renowned specialists in multiple sclerosis in our country. One outcome of this scientific activity, endorsed by the Spanish Neurology Society, is this review article, which is published in two parts. This second part reflects the current controversy over the management of multiple sclerosis, especially as regards the progressive forms and their differential diagnosis...
July 16, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28672919/brain-injury-with-systemic-inflammation-in-newborns-with-congenital-heart-disease-undergoing-heart-surgery
#17
Rossitza P Pironkova, Joseph Giamelli, Howard Seiden, Vincent A Parnell, Dorota Gruber, Cristina P Sison, Czeslawa Kowal, Kaie Ojamaa
The potential role of systemic inflammation on brain injury in newborns with congenital heart disease (CHD) was assessed by measuring levels of central nervous system (CNS)-derived proteins in serum prior to and following cardiac surgery. A total of 23 newborns (gestational age, 39±1 weeks) with a diagnosis of CHD that required cardiac surgery with cardiopulmonary bypass (CPB) were enrolled in the current study. Serum samples were collected immediately prior to surgery and 2, 24 and 48 h following CPB, and serum levels of phosphorylated neurofilament-heavy subunit (pNF-H), neuron-specific enolase (NSE) and S100B were analyzed...
July 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28661099/self-administration-of-methamphetamine-alters-gut-biomarkers-of-toxicity
#18
Amanda Flack, Amanda L Persons, Sharanya M Kousik, T Celeste Napier, Anna Moszczynska
Methamphetamine (METH) is a highly abused psychostimulant that is associated with an increased risk for developing Parkinson's disease (PD). This enhanced vulnerability likely relates to the toxic effects of METH that overlap with PD pathology, e.g., aberrant functioning of α-synuclein and parkin. In PD, peripheral factors are thought to contribute to central nervous system (CNS) degeneration. For example, α-synuclein levels in the enteric nervous system (ENS) are elevated, and this precedes the onset of motor symptoms...
June 29, 2017: European Journal of Neuroscience
https://www.readbyqxmd.com/read/28656644/activation-of-gsk-3-disrupts-cholinergic-homoeostasis-in-nucleus-basalis-of-meynert-and-frontal-cortex-of-rats
#19
Yue Wang, Qing Tian, En-Jie Liu, Li Zhao, Jie Song, Xin-An Liu, Qing-Guo Ren, Xia Jiang, Juan Zeng, Yu-Tao Yang, Jian-Zhi Wang
The cholinergic impairment is an early marker in Alzheimer's disease (AD), while the mechanisms are not fully understood. We investigated here the effects of glycogen synthase kinse-3 (GSK-3) activation on the cholinergic homoeostasis in nucleus basalis of Meynert (NBM) and frontal cortex, the cholinergic enriched regions. We activated GSK-3 by lateral ventricular infusion of wortmannin (WT) and GF-109203X (GFX), the inhibitors of phosphoinositol-3 kinase (PI3-K) and protein kinase C (PKC), respectively, and significantly decreased the acetylcholine (ACh) level via inhibiting choline acetyl transferase (ChAT) rather than regulating acetylcholinesterase (AChE)...
June 28, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28656221/effect-of-transplantation-of-olfactory-ensheathing-cell-conditioned-medium-induced-bone-marrow-stromal-cells-on-rats-with-spinal-cord-injury
#20
Linjie Feng, Hongquan Gan, Wenguo Zhao, Yingjie Liu
Spinal cord injury is a serious threat to human health and various techniques have been deployed to ameliorate or cure its effects. Stem cells transplantation is one of the promising methods. The primary aim of the present study was to investigate the effect of the transplantation of olfactory ensheathing cell (OEC) conditioned medium‑induced bone marrow stromal cells (BMSCs) on spinal cord injury. Rat spinal cord compression injury animal models were generated, and the rats divided into the following three groups: Group A, (control) Dulbecco's modified Eagle's medium‑treated group; group B, normal BMSC‑treated group; group C, OEC conditioned medium‑induced BMSC‑treated group...
August 2017: Molecular Medicine Reports
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