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Upper motor neuron

Ida K Fox, Christine B Novak, Emily M Krauss, Gwendolyn M Hoben, Craig Zaidman, Rimma Ruvinskaya, Neringa Juknis, Anke C Winter, Susan E Mackinnon
BACKGROUND: Nerve transfer surgery to restore upper extremity (UE) function in cervical spinal cord injury (SCI) is novel and may transform treatment. Determining candidacy even years post-SCI is ill-defined and deserves investigation. OBJECTIVE: To develop a diagnostic algorithm, focusing on electrodiagnostic studies (EDX), to determine eligibility for nerve transfer surgery. DESIGN: Retrospective descriptive case series SETTING: Tertiary university-based institution PATIENTS: Individuals with cervical SCI (n = 45) METHODS: The electronic medical records of people referred to the Plastic and Reconstructive Surgery UE Surgery in SCI clinic from 2010-2015 were reviewed...
March 15, 2018: PM & R: the Journal of Injury, Function, and Rehabilitation
Pascal Röderer, Lara Klatt, Felix John, Verena Theis, Konstanze F Winklhofer, Carsten Theiss, Veronika Matschke
Amyotrophic lateral sclerosis is a devastating motor neuron disease and to this day not curable. While 5-10% of patients inherit the disease (familiar ALS), up to 95% of patients are diagnosed with the sporadic form (sALS). ALS is characterized by the degeneration of upper motor neurons in the cerebral cortex and of lower motor neurons in the brainstem and spinal cord. The wobbler mouse resembles almost all phenotypical hallmarks of human sALS patients and is therefore an excellent motor neuron disease model...
March 16, 2018: Molecular Neurobiology
Giuseppe Battaglia, Valeria Bruno
Amyotrophic lateral sclerosis (ALS) is a complex genetic, late age-onset, progressive neurodegenerative disorder leading to the death of upper and lower motor neurons. Life expectancy after diagnosis is short due to the ongoing degeneration and to the lack of effective treatments. Axonal alterations, mitochondrial deficits, RNA changes, protein misfolding and turnover, glial dysfunction and hyperexcitability are key players in molecular mechanisms involved in the degeneration of motor neurons. In the context of hyperexcitability, metabotropic glutamate (mGlu) receptors, which are widely distributed throughout the central nervous system and act through many intracellular signaling pathways, are emerging as novel potential drug targets for the therapeutic treatment of ALS, as they are able to counteract excitotoxicity by reducing glutamate release and inducing the production of neurotrophic factors...
March 9, 2018: Current Opinion in Pharmacology
Emily R Seminary, Samantha L Sison, Allison D Ebert
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder caused by the selective loss of the upper and lower motor neurons. Only 10% of all cases are caused by a mutation in one of the two dozen different identified genes, while the remaining 90% are likely caused by a combination of as yet unidentified genetic and environmental factors. Mutations in C9orf72, SOD1 , or TDP-43 are the most common causes of familial ALS, together responsible for at least 60% of these cases. Remarkably, despite the large degree of heterogeneity, all cases of ALS have protein aggregates in the brain and spinal cord that are immunopositive for SOD1, TDP-43, OPTN, and/or p62...
2018: Frontiers in Neuroscience
Paul M Macey, Natasha Haris, Rajesh Kumar, M Albert Thomas, Mary A Woo, Ronald M Harper
INTRODUCTION: Obstructive sleep apnea (OSA) affects approximately 10% of adults, and alters brain gray and white matter. Psychological and physiological symptoms of the disorder are sex-specific, perhaps related to greater injury occurs in female than male patients in white matter. Our objective was to identify influences of OSA separated by sex on cortical gray matter. METHODS: We assessed cortical thickness in 48 mild-severe OSA patients (mean age±std[range] = 46...
2018: PloS One
Jorik Nonnekes, Nathalie Benda, Hanneke van Duijnhoven, Frits Lem, Noël Keijsers, Jan Willem K Louwerens, Allan Pieterse, Bertjo Renzenbrink, Vivian Weerdesteyn, Jaap Buurke, Alexander C H Geurts
Importance: Gait impairments are common in patients with chronic supratentorial upper motor neuron lesions and are a source of disability. Clinical management aimed at improving the gait pattern in these patients is generally perceived as a challenging task because many possible abnormalities may interact. Moreover, a multitude of treatment options exist, ranging from assistive devices and muscle stretching to pharmacologic and surgical interventions, but evidence is inconclusive for most approaches and clear treatment guidelines are lacking...
February 26, 2018: JAMA Neurology
E Fernandez-Gomez, A Sanchez-Cabeza
INTRODUCTION: Motor imagery or mental practice of movement is a relatively new intervention that is being used on an increasingly more frequently basis in the treatment of stroke patients. It consists in the person evoking a movement or gesture in order to learn or improve its execution. Neuroimaging studies have shown that imagining movements activates neuronal patterns that are similar to those produced when they are actually performed. PATIENTS AND METHODS: A systematic review was conducted between January and June 2017 in the Web of Science, PubMed, CINHAL, PEDro and Scopus databases to select clinical trials carried out with stroke patients in whom this technique was used as rehabilitation...
March 1, 2018: Revista de Neurologia
Craig Blackstone
The hereditary spastic paraplegias (HSPs) are a heterogeneous group of neurologic disorders with the common feature of prominent lower-extremity spasticity, resulting from a length-dependent axonopathy of corticospinal upper motor neurons. The HSPs exist not only in "pure" forms but also in "complex" forms that are associated with additional neurologic and extraneurologic features. The HSPs are among the most genetically diverse neurologic disorders, with well over 70 distinct genetic loci, for which about 60 mutated genes have already been identified...
2018: Handbook of Clinical Neurology
Stephen A Goutman, Kevin S Chen, Ximena Paez-Colasante, Eva L Feldman
Amyotrophic lateral sclerosis (ALS) is a progressive, noncurable neurodegenerative disorder of the upper and lower motor neurons causing weakness and death within a few years of symptom onset. About 10% of patients with ALS have a family history of the disease; however, ALS-associated genetic mutations are also found in sporadic cases. There are over 100 ALS-associated mutations, and importantly, several genetic mutations, including C9ORF72, SOD1, and TARDBP, have led to mechanistic insight into this complex disease...
2018: Handbook of Clinical Neurology
Abhirami K Iyer, Kathryn J Jones, Virginia M Sanders, Chandler L Walker
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive loss of lower and upper motor neurons (MN) leading to muscle weakness, paralysis and eventually death. Although a highly varied etiology results in ALS, it broadly manifests itself as sporadic and familial forms that have evident similarities in clinical symptoms and disease progression. There is a tremendous amount of knowledge on molecular mechanisms leading to loss of MNs and neuromuscular junctions (NMJ) as major determinants of disease onset, severity and progression in ALS...
February 23, 2018: International Journal of Molecular Sciences
Marcelo R S Briones, Amanda M Snyder, Renata C Ferreira, Elizabeth B Neely, James R Connor, James R Broach
Amyotrophic lateral sclerosis (ALS) is the third most prevalent neurodegenerative disease affecting upper and lower motor neurons. An important pathway that may lead to motor neuron degeneration is neuroinflammation. Cerebrospinal Fluids of ALS patients have increased levels of the inflammatory cytokine IL-18. Because IL-18 is produced by dendritic cells stimulated by the platelet-activating factor (PAF), a major neuroinflammatory mediator, it is expected that PAF is involved in ALS. Here we show pilot experimental data on amplification of PAF receptor (PAFR) mRNA by RT-PCR...
2018: Frontiers in Neurology
Natalie Ambrose, Karen A Waters, Michael L Rodriguez, Kendall Bailey, Rita Machaalani
The purpose of this study was to examine the neuronal expression of apoptotic markers in the rostral medulla of a newly characterized dataset of sudden unexpected death in infancy (SUDI), and to determine the impact of diagnostic groupings on these findings and whether they pertain to the intrinsic apoptotic pathway. Immunohistochemical staining was quantified to determine the percentage of neurons positive for active caspase-9 (specific to the intrinsic apoptotic pathway), active caspase-3 (common to the intrinsic and extrinsic apoptotic pathways) and Terminal deoxynucleotidyl transferase mediated dUTP nick-end labelling (TUNEL) (labels DNA fragmentation) in nine nuclei of the rostral medulla...
February 19, 2018: Forensic Science, Medicine, and Pathology
M Ceccanti, E Onesti, A Rubino, C Cambieri, G Tartaglia, A Miscioscia, V Frasca, M Inghilleri
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that causes an impairment in both the upper and lower motor neurons. The recent description of numerous non-motor signs points to an involvement of the neocortex networks that is more complex than was previously believed. Paired associative stimulation (PAS), a combination of transcranial magnetic stimulation (TMS) and peripheral nerve stimulation, can enhance motor output in the contralateral hand through an NMDA-mediated sensorimotor mechanism...
February 7, 2018: Brain Stimulation
Alexander Starr, Rita Sattler
Amyotrophic lateral sclerosis (ALS) is characterized by a progressive degeneration of upper and lower motor neurons, resulting in fatal paralysis due to denervation of the muscle. Due to genetic, pathological and symptomatic overlap, ALS is now considered a spectrum disease together with frontotemporal dementia (FTD), the second most common cause of dementia in individuals under the age of 65. Interestingly, in both diseases, there is a large prevalence of RNA binding proteins (RBPs) that are mutated and considered disease-causing, or whose dysfunction contribute to disease pathogenesis...
February 14, 2018: Brain Research
Ceren Tunca, Fulya Akçimen, Cemre Coşkun, Aslı Gündoğdu-Eken, Cemile Kocoglu, Betül Çevik, Can Ebru Bekircan-Kurt, Ersin Tan, A Nazlı Başak
Amyotrophic lateral sclerosis (ALS) is a late-onset motor neuron disease with mostly dominant inheritance and a life expectancy of 2-5 years; however, a quite common occurrence of atypical forms of the disease, due to recessive inheritance, has become evident with the use of NGS technologies. In this paper, we describe a family with close consanguinity for at least four generations, suffering from a slowly progressive form of ALS. Spastic walking is observed since teenage years, while bulbar symptoms start much later, at the fifth or sixth decade of life...
February 16, 2018: European Journal of Human Genetics: EJHG
Enrica Bersano, Maria Francesca Sarnelli, Valentina Solara, Fabiola De Marchi, Gian Mauro Sacchetti, Alessandro Stecco, Lucia Corrado, Sandra D'alfonso, Roberto Cantello, Letizia Mazzini
We describe a 64-year-old woman, suffering from late-onset obsessive-compulsive disorder (OCD) from the age of 57, who developed dysarthria and dysphagia, spastic diplegic, and proximal muscles weakness. Needle electromyography showed no active denervation. Neuropsychological evaluation showed intact cognitive functioning. We diagnosed upper motor neuron disease (MND), with no known genetic correlates. Brain magnetic resonance (MRI) detected bilateral hippocampal atrophy with sclerosis of right hippocampus...
February 16, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Anastasya Birger, Miri Ottolenghi, Liat Perez, Benjamin Reubinoff, Oded Behar
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by cell death of upper and lower motor neurons (MNs). The cause of MN cell loss is not completely understood but involves both cell autonomous and non-cell autonomous mechanisms. Numerous molecules have been implicated to be involved in the death of MNs. One such candidate is semaphorin 3A (Sema3A). In ALS patients, Sema3A was shown to be significantly upregulated in the motor cortex and downregulated in the spinal cord. In the mouse, Sema3A was shown to be an axon repellent molecule for MNs...
February 15, 2018: Cell Death & Disease
HaoJie Hu, HuiQian Lin, WeiSong Duan, Can Cui, ZhongYao Li, YaKun Liu, Wan Wang, Di Wen, Ying Wang, ChunYan Li
Amyotrophic lateral sclerosis (ALS) is a chronic, fatal neurodegenerative disorder characterized by the progressive loss of upper and lower motor neurons. Currently, there is no effective drug for ALS. Recent studies in ALS model mice have shown that insulin-like growth factor-1 (IGF1) may be a promising therapeutic drug. We demonstrate that self-complementary adeno-associated virus serum type 9 encoding the human IGF1 (scAAV9-hIGF1) could significantly postpone the onset and slow down the progression of the disease owning to inhibiting the NF-κB signalling pathway...
February 12, 2018: Neuroscience
Rebecca F D'Cruz, Patrick B Murphy, Georgios Kaltsakas
Motor neurone disease (MND) is a neurodegenerative disease defined by axonal loss and gliosis of upper and lower motor neurones in the motor cortex, lower brainstem nuclei and ventral horn of the spinal cord. MND is currently incurable and has a poor prognosis, with death typically occurring 3 to 5 years after disease onset. The disease is characterised by rapidly progressive weakness leading to paralysis, fasciculations, bulbar symptoms (including dysarthria and dysphagia) and respiratory compromise. Respiratory complications arise as a result of weakness of upper airway (pharyngeal and laryngeal) muscles and respiratory muscles (diaphragm, intercostal and accessory muscles) leading to respiratory failure...
January 2018: Journal of Thoracic Disease
Shahram Abootalebi, Mahmoud Reza Azarpazhooh, Luciano Sposato, Vladimir Hachinski
Background: The upgoing thumb sign as a subtle clinical finding of upper motor neuron involvement has been frequently reported in patients with TIAs and minor strokes. This study was designed to show the method of examination and interpretation and the interobserver/intraobserver reliability. Methods: The thumb sign was elicited in TIA/minor strokes or stroke mimics. After obtaining the participant's permission, the examinations were recorded. Two independent neurologists reviewed all patients for the possibility of an upgoing thumb sign...
December 2017: Neurology. Clinical Practice
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