Quiz 42, Understanding Motor Systemssr
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Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia
brain sciences Review Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia Timothy Fullam and Jeffrey Statland * Department of Neurology, University of Kansas Medical Center, Kansas, KS 66160, USA; [email protected] * Correspondence: [email protected] Abstract: Following the exclusion of potentially reversible causes, the differential for those patients presenting with a predominant upper motor neuron syndrome includes primary lateral sclerosis (PLS), hereditary spastic paraplegia (HSP), or upper motor neuron dominant ALS (UMNdALS). Differentiation of these disorders in the early phases of disease remains challenging. While no single clinical or diagnostic tests is specific, there are several developing biomarkers and neuroimaging technologies which may help distinguish PLS from HSP and UMNdALS. Recent consensus diagnostic criteria and use of evolving technologies will allow more precise delineation of PLS from other upper motor neuron disorders and aid in the targeting of potentially disease-modifying therapeutics. Keywords: primary lateral sclerosis; amyotrophic lateral sclerosis; hereditary spastic paraplegia Citation: Fullam, T.; Statland, J. Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper 1. Introduction Motor Neuron Dominant Jean-Martin Charcot (1825–1893) and Wilhelm Erb (1840–1921) are credited with first Amyotrophic Lateral Sclerosis, and describing a distinct clinical syndrome of upper motor neuron (UMN) tract degeneration in Hereditary Spastic Paraplegia. Brain isolation with symptoms including spasticity, hyperreflexia, and mild weakness [1,2]. Many Sci. 2021, 11, 611. https:// of the earliest described cases included cases of hereditary spastic paraplegia, amyotrophic doi.org/10.3390/brainsci11050611 lateral sclerosis, and underrecognized structural, infectious, or inflammatory etiologies for upper motor neuron dysfunction which have since become routinely diagnosed with the Academic Editors: P. -
Corticospinal Fibers
151 Brain stem Pyramids/Corticospinal Tract 1 PYRAMIDS - CORTICOSPINAL FIBERS The pyramids are two elongated swellings on the ventral aspect of the medulla. Each pyramid contains approximately 1,000,000 CORTICOSPINAL AXONS. As the name suggests, these axons arise from the cerebral cortex and descend to terminate within the spinal cord. The cortical cells that give rise to corticospinal axons are called Betz cells. As corticospinal axons descend from the cortex, they course through the internal capsule, the cerebral peduncle of the midbrain, and the ventral pons (you will learn about these structures later in the course so don’t worry about them now) and onto the ventral surface of the medulla as the pyramids (see below). When corticospinal axons reach the medulla they lie within the pyramids. The pyramids are just big fiber bundles that lie on the ventral surface of the caudal medulla. The fibers in the pyramids are corticospinal. It is important to REMEMBER: THERE HAS BEEN NO CROSSING YET! in this system. The cell bodies of corticospinal axons within the pyramids lie within the IPSILATERAL cerebral cortex. Brain stem 152 Pyramids/Corticospinal Tract At the most caudal pole of the pyramids the corticospinal axons cross over the midline and now continue their descent on the contralateral (to the cell of origin) side. This crossover point is called the PYRAMIDAL DECUSSATION. The crossing fibers enter the lateral funiculus of the spinal cord where they are called the LATERAL CORTICOSPINAL TRACT (“corticospinal” is not good enough, you have to call them lateral corticospinal; LCST - remember this one??). LCST axons exit the tract to terminate upon neurons in the spinal cord gray matter along its entire length. -
Spinal Tracts.Pdf
Spinal Tracts Andreas Talgø Lie Illustrations by: Peder Olai Skjeflo Holman Previous material: Maja Solbakken Definitions to bring home Nerve Ganglion Neuron Nucleus Tract Collection neurons Collection of nerve A single cell Collection of Collection of axons that transmits cell bodies in the transmitting nerve cell bodies in traveling up or sensation or motor PNS, typically linked electrical impluses. the CNS, typically down the spinal impulses depending by synapses. linked by synapses. cord, depending on the function and Location: both on function destination. Location: PNS Location: CNS and destination. Location: PNS Location: CNS - does it matter? Tract - highway to pass anatomy exam? • Highway = Tract • Lane = Neuron • Car = Signal Slow... Fast!! Just to make sure... • Ipsilateral or contralateral • Ventral = anterior • Dorsal = posterior High yield points to understand • What does the tract transmit - Motor or sensory? If sensory: which sensation? • Where does the neurones synapse - 1.st, 2nd and 3rd order neron? Which ganglion/nuclei? • Where there are decussations - If there are any decussations at all? ASCENDING / SENSORY TRACTS Sensations * Temperature NOT transmitted by the tracts: * Pressure * Visualization * Pain * Audition * Fine touch * Olfaction * Crude touch * Gustation * Proprioception * Vibration Sensations Precise sensation Primitive sensation Fine touch Crude touch Pressure Pain Vibration Temperature Proprioception Other: sexual, itching, tickling Ascending tracts / Sensory tracts Dorsal coulmn Lat. Spinothalamic Ant. -
Toward a Common Terminology for the Gyri and Sulci of the Human Cerebral Cortex Hans Ten Donkelaar, Nathalie Tzourio-Mazoyer, Jürgen Mai
Toward a Common Terminology for the Gyri and Sulci of the Human Cerebral Cortex Hans ten Donkelaar, Nathalie Tzourio-Mazoyer, Jürgen Mai To cite this version: Hans ten Donkelaar, Nathalie Tzourio-Mazoyer, Jürgen Mai. Toward a Common Terminology for the Gyri and Sulci of the Human Cerebral Cortex. Frontiers in Neuroanatomy, Frontiers, 2018, 12, pp.93. 10.3389/fnana.2018.00093. hal-01929541 HAL Id: hal-01929541 https://hal.archives-ouvertes.fr/hal-01929541 Submitted on 21 Nov 2018 HAL is a multi-disciplinary open access L’archive ouverte pluridisciplinaire HAL, est archive for the deposit and dissemination of sci- destinée au dépôt et à la diffusion de documents entific research documents, whether they are pub- scientifiques de niveau recherche, publiés ou non, lished or not. The documents may come from émanant des établissements d’enseignement et de teaching and research institutions in France or recherche français ou étrangers, des laboratoires abroad, or from public or private research centers. publics ou privés. REVIEW published: 19 November 2018 doi: 10.3389/fnana.2018.00093 Toward a Common Terminology for the Gyri and Sulci of the Human Cerebral Cortex Hans J. ten Donkelaar 1*†, Nathalie Tzourio-Mazoyer 2† and Jürgen K. Mai 3† 1 Department of Neurology, Donders Center for Medical Neuroscience, Radboud University Medical Center, Nijmegen, Netherlands, 2 IMN Institut des Maladies Neurodégénératives UMR 5293, Université de Bordeaux, Bordeaux, France, 3 Institute for Anatomy, Heinrich Heine University, Düsseldorf, Germany The gyri and sulci of the human brain were defined by pioneers such as Louis-Pierre Gratiolet and Alexander Ecker, and extensified by, among others, Dejerine (1895) and von Economo and Koskinas (1925). -
ALS and Other Motor Neuron Diseases Can Represent Diagnostic Challenges
Review Article Address correspondence to Dr Ezgi Tiryaki, Hennepin ALS and Other Motor County Medical Center, Department of Neurology, 701 Park Avenue P5-200, Neuron Diseases Minneapolis, MN 55415, [email protected]. Ezgi Tiryaki, MD; Holli A. Horak, MD, FAAN Relationship Disclosure: Dr Tiryaki’s institution receives support from The ALS Association. Dr Horak’s ABSTRACT institution receives a grant from the Centers for Disease Purpose of Review: This review describes the most common motor neuron disease, Control and Prevention. ALS. It discusses the diagnosis and evaluation of ALS and the current understanding of its Unlabeled Use of pathophysiology, including new genetic underpinnings of the disease. This article also Products/Investigational covers other motor neuron diseases, reviews how to distinguish them from ALS, and Use Disclosure: Drs Tiryaki and Horak discuss discusses their pathophysiology. the unlabeled use of various Recent Findings: In this article, the spectrum of cognitive involvement in ALS, new concepts drugs for the symptomatic about protein synthesis pathology in the etiology of ALS, and new genetic associations will be management of ALS. * 2014, American Academy covered. This concept has changed over the past 3 to 4 years with the discovery of new of Neurology. genes and genetic processes that may trigger the disease. As of 2014, two-thirds of familial ALS and 10% of sporadic ALS can be explained by genetics. TAR DNA binding protein 43 kDa (TDP-43), for instance, has been shown to cause frontotemporal dementia as well as some cases of familial ALS, and is associated with frontotemporal dysfunction in ALS. Summary: The anterior horn cells control all voluntary movement: motor activity, res- piratory, speech, and swallowing functions are dependent upon signals from the anterior horn cells. -
L4-Physiology of Motor Tracts.Pdf
: chapter 55 page 667 Objectives (1) Describe the upper and lower motor neurons. (2) Understand the pathway of Pyramidal tracts (Corticospinal & corticobulbar tracts). (3) Understand the lateral and ventral corticospinal tracts. (4) Explain functional role of corticospinal & corticobulbar tracts. (5) Describe the Extrapyramidal tracts as Rubrospinal, Vestibulospinal, Reticulospinal and Tectspinal Tracts. The name of the tract indicate its pathway, for example Corticobulbar : Terms: - cortico: cerebral cortex. Decustation: crossing. - Bulbar: brainstem. Ipsilateral : same side. *So it starts at cerebral cortex and Contralateral: opposite side. terminate at the brainstem. CNS influence the activity of skeletal muscle through two set of neurons : 1- Upper motor neurons (UMN) 2- lower motor neuron (LMN) They are neurons of motor cortex & their axons that pass to brain stem and They are Spinal motor neurons in the spinal spinal cord to activate: cord & cranial motor neurons in the brain • cranial motor neurons (in brainstem) stem which innervate muscles directly. • spinal motor neurons (in spinal cord) - These are the only neurons that innervate - Upper motor neurons (UMN) are the skeletal muscle fibers, they function as responsible for conveying impulses for the final common pathway, the final link voluntary motor activity through between the CNS and skeletal muscles. descending motor pathways that make up by the upper motor neurons. Lower motor neurons are classified based on the type of muscle fiber the innervate: There are two UMN Systems through which 1- alpha motor neurons (UMN) control (LMN): 2- gamma motor neurons 1- Pyramidal system (corticospinal tracts ). 2- Extrapyramidal system The activity of the lower motor neuron (LMN, spinal or cranial) is influenced by: 1. -
01 05 Lateral Surface of the Brain-NOTES.Pdf
Lateral Surface of the Brain Medical Neuroscience | Tutorial Notes Lateral Surface of the Brain 1 MAP TO NEUROSCIENCE CORE CONCEPTS NCC1. The brain is the body's most complex organ. LEARNING OBJECTIVES After study of the assigned learning materials, the student will: 1. Demonstrate the four paired lobes of the cerebral cortex and describe the boundaries of each. 2. Sketch the major features of each cerebral lobe, as seen from the lateral view, identifying major gyri and sulci that characterize each lobe. NARRATIVE by Leonard E. WHITE and Nell B. CANT Duke Institute for Brain Sciences Department of Neurobiology Duke University School of Medicine Overview When you view the lateral aspect of a human brain specimen (see Figures A3A and A102), three structures are usually visible: the cerebral hemispheres, the cerebellum, and part of the brainstem (although the brainstem is not visible in the specimen photographed in lateral view for Fig. 1 below). The spinal cord has usually been severed (but we’ll consider the spinal cord later), and the rest of the subdivisions are hidden from lateral view by the hemispheres. The diencephalon and the rest of the brainstem are visible on the medial surface of a brain that has been cut in the midsagittal plane. Parts of all of the subdivisions are also visible from the ventral surface of the whole brain. Over the next several tutorials, you will find video demonstrations (from the brain anatomy lab) and photographs (in the tutorial notes) of these brain surfaces, and sufficient detail in the narrative to appreciate the overall organization of the parts of the brain that are visible from each perspective. -
(Corticobulbar) Tract
Cor$cospinal (cor$cobulbar) Tract 6/18/12 1. Appreciate the functions of the corticospinal & corticobulbar tracts. 2. Distinguish corticospinal and corticobulbar tracts. Theodore Tzavaras MD2015 3. Identify the locations of both Laurie L. Wellman Ph.D. tracts through the forebrain, Eastern Virginia Medical School brainstem and spinal cord. 4. Identify the level of decussation of the corticospinal tract, and it’s clinical importance. Dr. Craig Goodmurphy Anatomy Guy Pathway Overview Pathway Overview Corticospinal Corticobulbar ² Begin in primary motor cortex. ² Begin in primary motor cortex. ² Upper ² Travel through the posterior limb Travel through the posterior limb Upper of the internal capsule (somatotopic). Motor of the internal capsule (somatotopic). Motor ² Descend through the middle 3/5 of Neuron ² Descend through the middle 3/5 of the crus cerebri (somatotopic). the crus cerebri (somatotopic). Neuron ² Travel through the brainstem as ² Travel through the brainstem and the descending pyramidal system. synapse on brainstem nuclei. Crosses Lower ² Decussate at the caudal medulla in Midline ² α-Motor neurons project along the pyramidal decussation. cranial nerves for facial movements Motor ² Descend in the spinal cord as the and voice production. Neuron corticospinal tract Lower ² All cranial nerve nuclei receive ² Synapse on α-motor neurons Motor bilateral UMN input* ² Exit spinal cord in ventral rami Neuron *Part of CN VI is the exception AnatomyGuy.com 1 Cor$cospinal (cor$cobulbar) Tract 6/18/12 Overview Corticospinal ² Primary motor cortex Don’t ü ² Try to learn all the details on your first Posterior limb of the internal pass. capsule (somatotopic). Do ü Try to sketch out each brain, ² Middle 3/5 of the crus cerebri brainstem, and cord level we showed (somatotopic). -
Identification of the Corticobulbar Tracts of the Tongue and Face Using Deterministic and Probabilistic DTI Fiber Tracking in Pa
Published August 6, 2015 as 10.3174/ajnr.A4430 ORIGINAL RESEARCH ADULT BRAIN Identification of the Corticobulbar Tracts of the Tongue and Face Using Deterministic and Probabilistic DTI Fiber Tracking in Patients with Brain Tumor M. Jenabi, X K.K. Peck, X R.J. Young, N. Brennan, and X A.I. Holodny ABSTRACT BACKGROUND AND PURPOSE: The corticobulbar tract of the face and tongue, a critical white matter tract connecting the primary motor cortex and the pons, is rarely detected by deterministic DTI fiber tractography. Detection becomes even more difficult in the presence of a tumor. The purpose of this study was to compare identification of the corticobulbar tract by using deterministic and probabilistic tractography in patients with brain tumor. MATERIALS AND METHODS: Fifty patients with brain tumor who underwent DTI were studied. Deterministic tractography was per- formed by using the fiber assignment by continuous tractography algorithm. Probabilistic tractography was performed by using a Monte Carlo simulation method. ROIs were drawn of the face and tongue motor homunculi and the pons in both hemispheres. RESULTS: In all subjects, fiber assignment by continuous tractography was ineffectual in visualizing the entire course of the corticobulbar tract between the face and tongue motor cortices and the pons on either side. However, probabilistic tractography successfully visualized the corticobulbar tract from the face and tongue motor cortices in all patients on both sides. No significant difference (P Ͻ .08) was found between both sides in terms of the number of voxels or degree of connectivity. The fractional anisotropy of both the face and tongue was significantly lower on the tumor side (P Ͻ .03). -
Visualization of the Pyramidal Decussation Utilizing Diffusion
OPEN ACCESS RESEARCH Visualization of the pyramidal decussation utilizing diffusion tensor imaging: a feasibility study utilizing generalized q-sampling imaging Erik H Middlebrooks1,*, Jeffrey A Bennett1, Sharatchandra Bidari1, and Alissa Old Crow1 1Department of Radiology, University of Florida College of Medicine, Gainesville, Florida, USA *corresponding author ([email protected]) Abstract Background: The delineating point of the end of the brainstem and beginning of the spinal cord is known as the cervicomedullary junction (CMJ). This point is defined by the decussation of the pyramidal tracts. Abnormal configuration and location of the CMJ have both been implicated in disease processes such as Chiari malformation. Unfortunately, the CMJ is not directly visualized on contemporary imaging techniques. Diffusion tensor imaging (DTI) has given us the ability to directly visualize white matter tracts, but suffers from difficulties with visualizing crossing fibers. Many advanced techniques for visualizing crossing fibers utilize substantially long imaging times or non-clinical magnet strengths making clinical applicability limited. Objective: This study inves- tigates the use of generalized q-sampling imaging with diffusion decomposition on standard DTI acquisitions at 3 Tesla to demonstrate the pyramidal decussation. Methods: Three differing DTI protocols were analyzed in vivo with scan times of 17 minutes, 10 minutes, and 5.5 minutes. Data was processed with standard DTI post-processing, as well as generalized q-sampling imaging with diffusion decomposition. Results: The results of the study show that the pyramidal decussation can be reliably visualized using generalized q-sampling imaging and diffusion decomposition with scan times as low at 10 minutes. Conclusion: Utilizing generalized q-sampling post-processing, the pyramidal decussation can be reliably visualized using clinically feasible DTI sequences with scan times as low as 10 minutes. -
Auditory and Vestibular Systems Objective • to Learn the Functional
Auditory and Vestibular Systems Objective • To learn the functional organization of the auditory and vestibular systems • To understand how one can use changes in auditory function following injury to localize the site of a lesion • To begin to learn the vestibular pathways, as a prelude to studying motor pathways controlling balance in a later lab. Ch 7 Key Figs: 7-1; 7-2; 7-4; 7-5 Clinical Case #2 Hearing loss and dizziness; CC4-1 Self evaluation • Be able to identify all structures listed in key terms and describe briefly their principal functions • Use neuroanatomy on the web to test your understanding ************************************************************************************** List of media F-5 Vestibular efferent connections The first order neurons of the vestibular system are bipolar cells whose cell bodies are located in the vestibular ganglion in the internal ear (NTA Fig. 7-3). The distal processes of these cells contact the receptor hair cells located within the ampulae of the semicircular canals and the utricle and saccule. The central processes of the bipolar cells constitute the vestibular portion of the vestibulocochlear (VIIIth cranial) nerve. Most of these primary vestibular afferents enter the ipsilateral brain stem inferior to the inferior cerebellar peduncle to terminate in the vestibular nuclear complex, which is located in the medulla and caudal pons. The vestibular nuclear complex (NTA Figs, 7-2, 7-3), which lies in the floor of the fourth ventricle, contains four nuclei: 1) the superior vestibular nucleus; 2) the inferior vestibular nucleus; 3) the lateral vestibular nucleus; and 4) the medial vestibular nucleus. Vestibular nuclei give rise to secondary fibers that project to the cerebellum, certain motor cranial nerve nuclei, the reticular formation, all spinal levels, and the thalamus. -
A System for Studying Mechanisms of Neuromuscular Junction Development and Maintenance Valérie Vilmont1,‡, Bruno Cadot1, Gilles Ouanounou2 and Edgar R
© 2016. Published by The Company of Biologists Ltd | Development (2016) 143, 2464-2477 doi:10.1242/dev.130278 TECHNIQUES AND RESOURCES RESEARCH ARTICLE A system for studying mechanisms of neuromuscular junction development and maintenance Valérie Vilmont1,‡, Bruno Cadot1, Gilles Ouanounou2 and Edgar R. Gomes1,3,*,‡ ABSTRACT different animal models and cell lines (Chen et al., 2014; Corti et al., The neuromuscular junction (NMJ), a cellular synapse between a 2012; Lenzi et al., 2015) with the hope of recapitulating some motor neuron and a skeletal muscle fiber, enables the translation of features of neuromuscular diseases and understanding the triggers chemical cues into physical activity. The development of this special of one of their common hallmarks: the disruption of the structure has been subject to numerous investigations, but its neuromuscular junction (NMJ). The NMJ is one of the most complexity renders in vivo studies particularly difficult to perform. studied synapses. It is formed of three key elements: the lower motor In vitro modeling of the neuromuscular junction represents a powerful neuron (the pre-synaptic compartment), the skeletal muscle (the tool to delineate fully the fine tuning of events that lead to subcellular post-synaptic compartment) and the Schwann cell (Sanes and specialization at the pre-synaptic and post-synaptic sites. Here, we Lichtman, 1999). The NMJ is formed in a step-wise manner describe a novel heterologous co-culture in vitro method using rat following a series of cues involving these three cellular components spinal cord explants with dorsal root ganglia and murine primary and its role is basically to ensure the skeletal muscle functionality.