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https://www.readbyqxmd.com/read/28632899/novel-intracytoplasmic-inclusions-immunoreactive-for-phosphorylated-tdp43-and-cystatin-c-in-anterior-horn-cells-in-a-case-of-sporadic-amyotrophic-lateral-sclerosis
#1
Masayuki Shintaku, Daita Kaneda, Kiyomitsu Oyanagi
Novel intracytoplasmic inclusions immunoreactive for phosphorylated transactivation response DNA-binding protein 43 (p-TDP43), cystatin C, and transferrin were found in anterior horn cells in a case of sporadic amyotrophic lateral sclerosis (ALS). The patient was a 59-year-old woman, who died of ALS after a clinical course of 8 years. She had been receiving mechanical support for respiration for 6 years and in a "totally locked-in" state for 4 years prior to death. The spinal cord showed severe degeneration involving the anterior and lateral funiculi, whereas the posterior funiculus was preserved...
June 20, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28630311/a-prokaryotic-viral-sequence-is-expressed-and-conserved-in-mammalian-brain
#2
Yang-Hui Yeh, Vignesh Gunasekharan, Laura Manuelidis
A natural and permanent transfer of prokaryotic viral sequences to mammals has not been reported by others. Circular "SPHINX" DNAs <5 kb were previously isolated from nuclease-protected cytoplasmic particles in rodent neuronal cell lines and brain. Two of these DNAs were sequenced after Φ29 polymerase amplification, and they revealed significant but imperfect homology to segments of commensal Acinetobacter phage viruses. These findings were surprising because the brain is isolated from environmental microorganisms...
June 19, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28584672/keratoacanthoma-of-the-nasal-septum-secondary-to-ranibizumab-use
#3
Jason E Cohn, Hilary M Caruso Sales, Giang Huong Nguyen, Harvey Spector, Kenneth Briskin
Keratoacanthoma (KA) is a benign epithelial tumor that typically presents as a firm, cone-shaped, flesh-colored nodule with a central horn-filled crater. KA is considered to be a low-grade variant of squamous cell carcinoma (SCC). We report a rare case of a 72-year-old male who presented with a KA involving the nasal septum, possibly related to ranibizumab use. A flesh-colored lesion on the right anterior nasal septum lesion was visualized on examination. Histologic examination revealed a well-circumscribed, dome-shaped central crater filled with keratin, well-differentiated squamous epithelium with ground-glass cytoplasm with pushing margins, and intraepithelial microabscesses establishing the diagnosis of KA...
2017: Case Reports in Pathology
https://www.readbyqxmd.com/read/28554554/expanding-the-phenotypic-spectrum-associated-with-mutations-of-dync1h1
#4
Sarah J Beecroft, Catriona A McLean, Martin B Delatycki, Kurian Koshy, Eppie Yiu, Goknur Haliloglu, Diclehan Orhan, Phillipa J Lamont, Mark R Davis, Nigel G Laing, Gianina Ravenscroft
Autosomal dominant mutations of DYNC1H1 cause a range of neurogenetic diseases, including mental retardation with cortical malformations, hereditary spastic paraplegia and spinal muscular atrophy. Using SNP array, linkage analysis and next generation sequencing, we identified two families and one isolated proband sharing a known spinal muscular atrophy, lower extremity predominant (SMALED) causing mutation DYNC1H1 c.1792C>T, p.Arg598Cys, and another family harbouring a c.2327C>T, p.Pro776Leu mutation...
July 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28549966/lentivirus-mediated-inhibition-of-aqp4-accelerates-motor-function-recovery-associated-with-ngf-in-spinal-cord-contusion-rats
#5
Jianmin Chen, Xi Zeng, Shengnan Li, Zhanqiong Zhong, Xi Hu, Hongyu Xiang, Ying Rao, Lixin Zhang, Xiuya Zhou, Qingjie Xia, Tinghua Wang, Xiao Zhang
Aquaporin-4 (AQP4) is a water channel protein in spinal cord and plays a critical role in the pathophysiological process of spinal cord injury (SCI). However, little is known about the molecular mechanism of AQP4 involved in SCI. The present study was performed to investigate the possible molecules regulated by AQP4 after SCI by use of lentiviral-mediated RNA interference (RNAi). First, the motor function was evaluated by Basso, Beattie, Bresnahan (BBB) scale and the expression of AQP4 was measured by western blot, immunohistochemical staining and immunofluorescence in rats after contusion spinal cord injury (cSCI)...
May 23, 2017: Brain Research
https://www.readbyqxmd.com/read/28539831/aldehyde-dehydrogenases-1a2-expression-and-distribution-are-potentially-associated-with-neuron-death-in-spinal-cord-of-tg-sod1-g93a-1gur-mice
#6
Huiting Liang, Chengsi Wu, Youqing Deng, Lei Zhu, Jie Zhang, Weiming Gan, Chunyan Tang, Renshi Xu
The pathogenesis of amyotrophic lateral sclerosis (ALS) has not been unclear yet, it might be associated with the abnormal expression and distribution of certain proteins. Aldehyde dehydrogenases 1A2 (ALDH1A2) was thought to be one of potential candidates. Therefore, in this study we observed and analyzed the alteration of the expression and distribution of ALDH1A2 in the spinal cord of wild-type (WT) and Tg(SOD1*G93A)1Gur mice. We compared the expression and distribution of ALDH1A2 in the different segments, anatomic regions and neural cells of spinal cord at the different stages of WT and Tg(SOD1*G93A)1Gur mice applied the methods of fluorescent immunohistochemistry and western blot...
2017: International Journal of Biological Sciences
https://www.readbyqxmd.com/read/28528422/5-ala-fluorescence-guided-endoscopic-surgery-for-mixed-germ-cell-tumors
#7
Junichi Takeda, Masahiro Nonaka, Yi Li, Yumiko Komori, Takamasa Kamei, Ryoichi Iwata, Tetsuo Hashiba, Kunikazu Yoshimura, Akio Asai
5-Aminolevulinic acid (5-ALA) fluorescence-guided surgery is widely used for detection and planning of resection of malignant gliomas and other brain tumors. However, no reports have described 5-ALA fluorescence-guided surgery or direct visualization of germ cell tumors. Here, we report two cases of germ cell tumors in which a positive 5-ALA fluorescent signal was visualized with a neuroendoscope. Both cases had a tumor in the pineal region that was associated with hydrocephalus. The patients underwent surgery after administration of 5-ALA...
May 20, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28413866/inositol-hexakisphosphate-kinase-2-is-a-presymptomatic-biomarker-for-amyotrophic-lateral-sclerosis
#8
Yusuke Moriya, Eiichiro Nagata, Natsuko Fujii, Tadayuki Satoh, Haruko Ogawa, Shinji Hadano, Shunya Takizawa
OBJECTIVE: Inositol hexakisphosphate kinase 2 (InsP6K2), an enzyme that converts inositol hexakisphosphate (InsP6) to diphosphoinositol pentakisphosphate (InsP7), induces cell death. InsP6K2 is abundant in the central nervous system, especially anterior horn cells of spinal cord. To identify the role of InsP6K2 in amyotrophic lateral sclerosis (ALS), we investigated the expression levels of InsP6K2 in transgenic mice expressing mutant superoxide dismutase-1 (SOD1) (mSOD1 Tg mice). METHODS: The specimens of spinal cords were obtained from mSOD1 Tg mice and age-matched wild-type mice...
April 20, 2017: Tokai Journal of Experimental and Clinical Medicine
https://www.readbyqxmd.com/read/28382437/diffuse-leptomeningeal-glioneuronal-tumor-dlgnt-mimicking-whipple-s-disease-a-case-report-and-literature-review
#9
Vega Karlowee, Manish Kolakshyapati, Vishwa Jeet Amatya, Takeshi Takayasu, Ryo Nosaka, Kazuhiko Sugiyama, Kaoru Kurisu, Fumiyuki Yamasaki
INTRODUCTION: Diffuse leptomeningeal glioneuronal tumor is a new entity under the neuronal and mixed neuronal-glial tumors in the WHO 2016 updated classification and commonly found in children and adolescents. The initial diagnosis is challenging because of its non-specific radiologic feature and negative CSF cytology analysis. A 17 years male was presented with intractable headache subsequently followed by back pain and joint pain. MRI showed enhancement of arachnoid membrane at basal cistern, bilateral sylvian fissure and cerebral cistern with slight enlargement of ventricles...
April 5, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28380696/long-term-neuromuscular-outcomes-of-west-nile-virus-infection-a-clinical-and-electromyographic-evaluation-of-patients-with-a-history-of-infection
#10
Parveen Athar, Rodrigo Hasbun, Melissa S Nolan, Lucrecia Salazar, Steven P Woods, Kazim Sheikh, Kristy O Murray
INTRODUCTION: Neuromuscular clinical manifestations during acute West Nile virus (WNV) infection are well documented; however, long-term neurologic outcomes still require investigation. METHODS: We conducted a long-term follow-up study in patients with history of WNV infection. Of the 117 patients who participated in neurologic and neurocognitive evaluations, 30 were referred for neuromuscular and electrodiagnostic evaluation based on abnormal findings. RESULTS: We found that 33% of these patients (10 of 30) showed abnormalities on nerve conduction and/or needle electromyography due to primary or secondary outcomes of WNV infection...
April 5, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28380320/reappraisal-of-the-f-m-amplitude-ratio-in-carpal-tunnel-syndrome
#11
F Ginanneschi, M Mondelli, A Aretini, Alessandro Rossi
The F-wave/M-wave amplitude (F/M-amp) ratio has been shown to be increased in peripheral neuropathies, provided the maximum M-wave is relatively preserved. Reduced M-wave amplitudes and central facilitation of antidromically-induced reactivation of the anterior horn cells' axon hillocks (F-wave) are believed to contribute to higher F/M-amp ratios. The present study was undertaken to re-evaluate mechanisms responsible for higher F/M-amp ratios in carpal tunnel syndrome (CTS). We enrolled 232 cases affected by CTS and 108 controls...
January 2017: Functional Neurology
https://www.readbyqxmd.com/read/28352498/traumatic-cervical-nerve-root-avulsion-with-pseudomeningocele-formation
#12
Ali S Haider, Ian T Watson, Suraj Sulhan, Dean Leonard, Eliel N Arrey, Umair Khan, Phu Nguyen, Kennith F Layton
Cervical nerve root avulsion is a well-documented result of motor vehicle collision (MVC), especially when occurring at high velocities. These avulsions are commonly traction injuries of nerve roots that may be accompanied by a tear in the meninges through the vertebral foramina with associated collections of cerebrospinal fluid (CSF), thereby resulting in a pseudomeningocele. We present a case of a 19-year-old male who experienced an MVC and was brought to the emergency department (ED) with right arm paralysis and other injuries...
February 14, 2017: Curēus
https://www.readbyqxmd.com/read/28251660/neuronal-and-microglial-mechanisms-for-neuropathic-pain-in-the-spinal-dorsal-horn-and-anterior-cingulate-cortex
#13
REVIEW
Makoto Tsuda, Kohei Koga, Tao Chen, Min Zhuo
Neuropathic pain is a debilitating chronic pain condition occurring after damage in the nervous system and is refractory to the currently available treatments. Major challenges include elucidating its mechanisms and developing new medications to treat it. Nerve injury-induced pain hypersensitivity involves aberrant excitability in spinal dorsal horn (SDH) neurons as a consequence of dysfunction of inhibitory interneurons and of hyperactivity of glial cells, especially microglia, the immune cells of the central nervous system...
May 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28124772/expression-and-distribution-of-arylsulfatase-b-are-closely-associated-with-neuron-death-in-sod1-g93a-transgenic-mice
#14
Jie Zhang, Huiting Liang, Lei Zhu, Weiming Gan, Chunyan Tang, Jiao Li, Renshi Xu
The known proteins only explained the partial pathogenesis of amyotrophic lateral sclerosis (ALS). Therefore, this study aimed to search the novel proteins possibly involved in ALS. In this study, we analyzed the expression and distribution of the candidate protein arylsulfatase B (ARSB) in the different segments, anatomic regions, and neural cells of spinal cord at the different stages of the wild-type and [Cu/Zn] superoxide dismutase 1 (SOD1) G93A transgenic mice using the fluorescent immunohistochemistry and the western blot...
January 26, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28035406/bambi-inhibits-inflammation-through-the-activation-of-autophagy-in-experimental-spinal-cord-injury
#15
Yin Yang, Chunyang Guo, Bo Liao, Junjun Cao, Chen Liang, Xijing He
Autophagy plays an important role in the progression of spinal cord injury (SCI). In this study, we aimed to examine the effects and potential mechanisms of action of BMP and activin membrane-bound inhibitor (BAMBI) in the progression of SCI. A rat model of SCI was established and the rats were injected with pLentiH1-BAMBI shRNA and pAd-BAMBI in the gray and white matter of the spinal cord at T8. After 14 days, motor function evaluation was measured according to the Basso Beattie Bresnahan (BBB) method and the number of motor neuron cell accounts in the anterior horns was measured by Nissl staining...
February 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28003344/differentiating-lower-motor-neuron-syndromes
#16
REVIEW
Nidhi Garg, Susanna B Park, Steve Vucic, Con Yiannikas, Judy Spies, James Howells, William Huynh, José M Matamala, Arun V Krishnan, John D Pollard, David R Cornblath, Mary M Reilly, Matthew C Kiernan
Lower motor neuron (LMN) syndromes typically present with muscle wasting and weakness and may arise from pathology affecting the distal motor nerve up to the level of the anterior horn cell. A variety of hereditary causes are recognised, including spinal muscular atrophy, distal hereditary motor neuropathy and LMN variants of familial motor neuron disease. Recent genetic advances have resulted in the identification of a variety of disease-causing mutations. Immune-mediated disorders, including multifocal motor neuropathy and variants of chronic inflammatory demyelinating polyneuropathy, account for a proportion of LMN presentations and are important to recognise, as effective treatments are available...
June 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/27999136/human-muse-cells-reconstruct-neuronal-circuitry-in-subacute-lacunar-stroke-model
#17
Hiroki Uchida, Kuniyasu Niizuma, Yoshihiro Kushida, Shohei Wakao, Teiji Tominaga, Cesario V Borlongan, Mari Dezawa
BACKGROUND AND PURPOSE: Multilineage-differentiating stress-enduring (muse) cells are endogenous nontumorigenic stem cells with pluripotency harvestable as pluripotent marker SSEA-3(+) cells from the bone marrow from cultured bone marrow-mesenchymal stem cells. After transplantation into neurological disease models, muse cells exert repair effects, but the exact mechanism remains inconclusive. METHODS: We conducted mechanism-based experiments by transplanting serum/xeno-free cultured-human bone marrow-muse cells into the perilesion brain at 2 weeks after lacunar infarction in immunodeficient mice...
February 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/27998013/neuroprotective-and-ameliorating-impacts-of-omega-3-against-aspartame-induced-neuronal-and-astrocytic-degeneration
#18
Eyad M T Ali, Hany M A Sonpol
Aspartame (ASP) is one of the commonest artificial sweetener used all over the world and considered as an extremely risky compound and raises a lot of controversy. Therefore, this study was designed to investigate cellular damage of the anterior horn cells in the spinal cord of albino male rats and the possibility of hindering these changes by using omega-3 (OM3).Thirty seven adult male albino rats were divided into three groups: Control, ASP-treated and ASP + OM3-treated groups. Spinal cord sections were prepared and stained with Hx&E, caspase-3 and GFAP immunostaining...
December 20, 2016: Anatomical Record: Advances in Integrative Anatomy and Evolutionary Biology
https://www.readbyqxmd.com/read/27897305/-cervical-ischaemic-neuronopathy-and-cardioembolism-another-cause-of-man-in-the-barrel-syndrome
#19
H Gonzalez-Usigli, A Gandarilla, J J Garcia, J Serrato, N Estrada
INTRODUCTION: Spinal infarction accounts for 1% of all strokes. Cardioembolism is a rare cause. Common areas of ischemic spinal damage are watershed in the dorsal or lumbar regions; however cervical spinal cord infarction has been reported previously. CASE REPORT: We present a new case of a man-in-the-barrel syndrome produced by cardiac embolization associated with atrial fibrillation during an acute myocardial infarction, which caused cervical ischemic neuronopathy (infarction of the anterior horn)...
December 16, 2016: Revista de Neurologia
https://www.readbyqxmd.com/read/27800001/clinical-experiences-of-uncommon-motor-neuron-disease-hirayama-disease
#20
Kyoung Hee Lee, Dae Seob Choi, Young Suk Lee, Dong Ho Kang
Hirayama disease, juvenile muscular atrophy of the distal upper limb, is a rare disease predominantly affecting the anterior horn cells of the cervical spinal cord in young men. This cervical myelopathy is associated with neck flexion. It should be suspected in young male patients with a chronic history of weakness and atrophy involving the upper extremities followed by clinical stability in few years. Herein, we report 2 cases of Hirayama disease on emphasis of diagnostic approach and describe the pathognomonic findings at flexion magnetic resonance imaging...
September 2016: Korean Journal of Spine
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