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Upper motor neuron

Suma Babu, Erik P Pioro, Jianbo Li, Yuebing Li
INTRODUCTION: We compared the yield of limb and thoracic paraspinal muscles for revealing lower motor neuron (LMN) dysfunction on electromyography (EMG) in amyotrophic lateral sclerosis (ALS). METHODS: A retrospective review of 354 patients with clinically definite or probable ALS was performed. Seventeen limb muscles and thoracic paraspinal muscles were evaluated for the presence of both active and chronic denervation. RESULTS: Distal limb muscles showed the highest electrodiagnostic sensitivities of LMN dysfunction in ALS regardless of onset region and diagnostic certainty at the time of diagnosis...
October 19, 2016: Muscle & Nerve
Gianina Ravenscroft, Nataliya Di Donato, Gabriele Hahn, Mark R Davis, Paul D Craven, Gemma Poke, Katherine R Neas, Teresa M Neuhann, William B Dobyns, Nigel G Laing
Autosomal dominantly inherited mutations of BICD2 are associated with congenital-onset spinal muscular atrophy characterised by lower limb predominance. A few cases have also showed upper motor neuron pathology, including presenting with features resembling hereditary spastic paraplegia. The age-of-onset for the published families is usually at birth but also included cases with childhood- and adult-onset disease. In this report we described two isolated probands that presented in utero with features associated with reduced fetal movements...
September 19, 2016: Neuromuscular Disorders: NMD
David S Lynch, Wei Jia Zhang, Rahul Lakshmanan, Justin A Kinsella, Günes Altiokka Uzun, Merih Karbay, Zeynep Tüfekçioglu, Hasmet Hanagasi, Georgina Burke, Nicola Foulds, Simon R Hammans, Anupam Bhattacharjee, Heather Wilson, Matthew Adams, Mark Walker, James A R Nicoll, Jeremy Chataway, Nick Fox, Indran Davagnanam, Rahul Phadke, Henry Houlden
Importance: Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a frequent cause of adult-onset leukodystrophy known to be caused by autosomal dominant mutations in the CSF1R (colony-stimulating factor 1) gene. The discovery that CSF1R mutations cause ALSP led to more accurate prognosis and genetic counseling for these patients in addition to increased interest in microglia as a target in neurodegeneration. However, it has been known since the discovery of the CSF1R gene that there are patients with typical clinical and radiologic evidence of ALSP who do not carry pathogenic CSF1R mutations...
October 17, 2016: JAMA Neurology
Adam G Rouse, Marc H Schieber
: Reaching and grasping typically are considered to be spatially separate processes that proceed concurrently in the arm and the hand, respectively. The proximal representation in the primary motor cortex (M1) controls the arm for reaching, while the distal representation controls the hand for grasping. Many studies of M1 activity therefore have focused either on reaching to various locations without grasping different objects, or else on grasping different objects all at the same location...
October 12, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Naohiko Okabe, Takashi Shiromoto, Naoyuki Himi, Feng Lu, Emi Maruyama-Nakamura, Kazuhiko Narita, Nobuhisa Iwachidou, Yoshiki Yagita, Osamu Miyamoto
Motor map reorganization is believed to be one mechanism underlying rehabilitation-induced functional recovery. Although the ipsilesional secondary motor area has been known to reorganize motor maps and contribute to rehabilitation-induced functional recovery, it is unknown how the secondary motor area is reorganized by rehabilitative training. In the present study, using skilled forelimb reaching tasks, we investigated neural network remodeling in the rat rostral forelimb area (RFA) of the secondary motor area during 4 weeks of rehabilitative training...
October 7, 2016: Neuroscience
Valerio Vitale, Ferdinando Caranci, Chiara Pisciotta, Fiore Manganelli, Francesco Briganti, Lucio Santoro, Arturo Brunetti
BACKGROUND: Hirayama's disease (HD), is a benign, self-limited, motor neuron disease, characterized by asymmetric weakness and atrophy of one or both distal upper extremities. In the present study we report the clinical, electrophysiological and MRI features of a group of Italian patients, with review of the literature. Moreover we propose an optimized MRI protocol for patients with suspected or diagnosed HD in order to make an early diagnosis and a standardized follow up. METHODS: Eight patients with clinical suspicion of Hirayama disease underwent evaluation between January 2007 and November 2013...
August 2016: Quantitative Imaging in Medicine and Surgery
Naoum P Issa, Samuel Frank, Raymond Roos, Betty Soliven, Vernon L Towle, Kourosh Rezania
INTRODUCTION: Simple laboratory tests of upper motor neuron involvement in amyotrophic lateral sclerosis (ALS) are not available. Intermuscular coherence has been shown to distinguish patients with primary lateral sclerosis, a pure upper motor neuron disorder, from normal subjects, suggesting it could be useful for assessing ALS. OBJECTIVE: To determine whether intermuscular coherence can distinguish ALS patients from normal subjects. METHODS: We measured biceps brachii and brachioradialis activity using surface electromyography while subjects held the elbow at flexion and the forearm in semi-pronation...
October 3, 2016: Muscle & Nerve
Gareth D Jones, Darren C James, Michael Thacker, David A Green
Individuals with sensorimotor pathology e.g., stroke have difficulty executing the common task of rising from sitting and initiating gait (sit-to-walk: STW). Thus, in clinical rehabilitation separation of sit-to-stand and gait initiation - termed sit-to-stand-and-walk (STSW) - is usual. However, a standardized STSW protocol with a clearly defined analytical approach suitable for pathological assessment has yet to be defined. Hence, a goal-orientated protocol is defined that is suitable for healthy and compromised individuals by requiring the rising phase to be initiated from 120% knee height with a wide base of support independent of lead limb...
2016: Journal of Visualized Experiments: JoVE
Dongpei Li, Seigo Usuki, Brandy Quarles, Michael H Rivner, Toshio Ariga, Robert K Yu
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive degeneration of upper and lower motor neurons. Although the etiology of ALS is obscure, genetic studies of familiar ALS suggest a multifactorial etiology for this condition. Similarly, there probably are multiple causes for sporadic ALS. Autoimmune-mediated motor neuron dysfunction is one proposed etiology for sporadic ALS. In the present study, anti-glycolipid antibodies including GM1, GD1b, GD3, and sulfoglucuronosyl paragloboside (SGPG) were investigated in the sera of a large number of patient samples, including 113 ALS patients and 50 healthy controls, by means of enzyme-linked immunosorbent assay with affinity parametric complex criterion evaluation and thin-layer chromatography immunooverlay (immuno-TLC)...
October 2016: ASN Neuro
Stavroula Tsitkanou, Paul A Della Gatta, Aaron P Russell
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is a fatal motor neuron disorder. It results in progressive degeneration and death of upper and lower motor neurons, protein aggregation, severe muscle atrophy and respiratory insufficiency. Median survival with ALS is between 2 and 5 years from the onset of symptoms. ALS manifests as either familial ALS (FALS) (~10% of cases) or sporadic ALS (SALS), (~90% of cases). Mutations in the copper/zinc (CuZn) superoxide dismutase (SOD1) gene account for ~20% of FALS cases and the mutant SOD1 mouse model has been used extensively to help understand the ALS pathology...
2016: Frontiers in Physiology
Lucie Chochina, Florian Naudet, Isabelle Bonan, Clément Chehensse, Mireille Damphousse, Andrea Manunta, François Giuliano
OBJECTIVE: To review the efficacy of intracavernous injections (ICI) in spinal cord injured (SCI) men and to identify prognostic factors affecting the efficacy of ICI in this population. MATERIAL/PATIENTS AND METHODS: Systematic review of the literature using 5 databases. Articles published up to november 2014 using the keywords: alprostadil; papaverine; moxisylyte; alpha-blocking agent; phentolamine; intracavernous injection; spinal cord injuries; paraplegia; quadriplegia; erectile dysfunction; were included...
September 2016: Annals of Physical and Rehabilitation Medicine
Jiyeon Kim Md PhD, Yu-Hsien Liao, Cristian Ionita, Allen E Bale, Basil Darras, Gyula Acsadi
BACKGROUND: Mitochondrial membrane protein associated neurodegeneration (MPAN) is the third most common subtype of neurodegeneration with brain iron accumulation (NBIA) and caused by mutations of the orphan gene C19ORF12 encoding a transmembrane mitochondrial protein. Like other NBIA disorders, the hallmark of neuropathology is iron deposition in the basal ganglia, but the clinical presentation is highly variable. METHODS: We present the relevant clinical history, neurological examination, electrophysiological and neuroimaging tests of a currently ten-year-old patient are presented...
August 24, 2016: Pediatric Neurology
Lauren N Bowen, Richa Tyagi, Wenxue Li, Tariq Alfahad, Bryan Smith, Mary Wright, Elyse J Singer, Avindra Nath
OBJECTIVE: To determine whether there is activation of human endogenous retrovirus K (HERV-K) in amyotrophic lateral sclerosis in HIV infection and whether it might respond to treatment with antiretroviral drugs. METHODS: In this case series, we present 5 patients with HIV infection who subsequently developed motor neuron disease involving both upper and lower motor neurons. We monitored HERV-K levels in plasma of 4 of these patients. RESULTS: Three patients who received antiretroviral therapy had reversal of symptoms within 6 months of onset of neurologic symptoms and the other 2 had slow neurologic progression over several years...
September 24, 2016: Neurology
Ammar Al-Chalabi, Orla Hardiman, Matthew C Kiernan, Adriano Chiò, Benjamin Rix-Brooks, Leonard H van den Berg
Amyotrophic lateral sclerosis is a progressive adult-onset neurodegenerative disease that primarily affects upper and lower motor neurons, but also frontotemporal and other regions of the brain. The extent to which each neuronal population is affected varies between individuals. The subsequent patterns of disease progression form the basis of diagnostic criteria and phenotypic classification systems, with considerable overlap in the clinical terms used. This overlap can lead to confusion between diagnosis and phenotype...
October 2016: Lancet Neurology
Stéphane Mathis, Philippe Couratier, Adrien Julian, Jean-Michel Vallat, Philippe Corcia, Gwendal Le Masson
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder affecting both upper and lower motor neurons. Despite much research and effort, no clear insights into a unifying hypothesis for the pathogenesis has so far emerged for this disease. AREAS COVERED: We review the main pathophysiological hypotheses and the potential therapeutic targets in ALS, as well as the management of these patients (in order to improve their survival and quality of life)...
September 20, 2016: Expert Review of Neurotherapeutics
B Mark Keegan, Timothy J Kaufmann, Brian G Weinshenker, Orhun H Kantarci, William F Schmalstieg, M Mateo Paz Soldan, Eoin P Flanagan
OBJECTIVE: To report patients with progressive motor impairment resulting from an isolated CNS demyelinating lesion in cerebral, brainstem, or spinal cord white matter that we call progressive solitary sclerosis. METHODS: Thirty patients were identified with (1) progressive motor impairment for over 1 year with a single radiologically identified CNS demyelinating lesion along corticospinal tracts, (2) absence of other demyelinating CNS lesions, and (3) no history of relapses affecting other CNS pathways...
October 18, 2016: Neurology
M A Akulov, S E Khat'kova, O A Mokienko, O R Orlova, D Yu Usachev, V O Zakharov, A S Orlova, A A Tomsky
Spasticity is a type of muscle hyperactivity that occurs in patients after focal lesions of the Central nervous system due to various diseases: stroke, traumatic brain injury or spinal cord injury, neurosurgical intervention, as well as multiple sclerosis and other diseases of the Central nervous system and is the most disability manifestation of the syndrome of upper motor neuron (UMNS). Focal spasticity of the upper limb requires a complex treatment. Botulinum toxin therapy is an effective treatment for focal/multifocal spasticity in reducing muscle tone and improving function with the highest level of evidence according to the latest American and European guidelines for treatment of spasticity...
2016: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
Angela Rosenbohm, Hans-Peter Müller, Annemarie Hübers, Albert C Ludolph, Jan Kassubek
Criteria for assessing upper motor neuron pathology in lower motor neuron disease (LMND) still remain major issues in clinical diagnosis. This study was designed to investigate patients with the clinical diagnosis of adult pure LMND by use of whole brain based diffusion tensor imaging (DTI) to delineate alterations of corticoefferent pathways in vivo. Comparison of fractional anisotropy (FA) maps was performed by whole brain-based spatial statistics for 37 LMND patients vs. 53 matched controls to detect white matter structural alterations...
September 13, 2016: Journal of Neurology
Richard Baker, Alberto Esquenazi, Maria G Benedetti, Kaat Desloovere
Gait analysis is a well-established tool for the quantitative assessment of gait disturbances providing functional diagnosis, assessment for treatment planning, and monitoring of disease progress. There is a large volume of literature on the research use of gait analysis, but evidence on its clinical routine use supports a favorable cost-benefit ratio in a limited number of conditions. Initially gait analysis was introduced to clinical practice to improve the management of children with cerebral palsy. However, there is good evidence to extend its use to patients with various upper motor neuron diseases, and to lower limb amputation...
August 2016: European Journal of Physical and Rehabilitation Medicine
Eric Diaz, Humberto Morales
We review the anatomy of the spinal cord, providing correlation with key functional and clinically relevant neural pathways, as well as magnetic resonance imaging. Peripherally, the main descending (corticospinal tract) and ascending (gracilis or cuneatus fasciculi and spinothalamic tracts) pathways compose the white matter. Centrally, the gray matter can be divided into multiple laminae. Laminae 1-5 carry sensitive neuron information in the posterior horn, and lamina 9 carries most lower motor neuron information in the anterior horn...
October 2016: Seminars in Ultrasound, CT, and MR
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