Muscle Tone Physiology and Abnormalities
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VIEW Open Access Muscle Spindle Function in Healthy and Diseased Muscle Stephan Kröger* and Bridgette Watkins
Kröger and Watkins Skeletal Muscle (2021) 11:3 https://doi.org/10.1186/s13395-020-00258-x REVIEW Open Access Muscle spindle function in healthy and diseased muscle Stephan Kröger* and Bridgette Watkins Abstract Almost every muscle contains muscle spindles. These delicate sensory receptors inform the central nervous system (CNS) about changes in the length of individual muscles and the speed of stretching. With this information, the CNS computes the position and movement of our extremities in space, which is a requirement for motor control, for maintaining posture and for a stable gait. Many neuromuscular diseases affect muscle spindle function contributing, among others, to an unstable gait, frequent falls and ataxic behavior in the affected patients. Nevertheless, muscle spindles are usually ignored during examination and analysis of muscle function and when designing therapeutic strategies for neuromuscular diseases. This review summarizes the development and function of muscle spindles and the changes observed under pathological conditions, in particular in the various forms of muscular dystrophies. Keywords: Mechanotransduction, Sensory physiology, Proprioception, Neuromuscular diseases, Intrafusal fibers, Muscular dystrophy In its original sense, the term proprioception refers to development of head control and walking, an early im- sensory information arising in our own musculoskeletal pairment of fine motor skills, sensory ataxia with un- system itself [1–4]. Proprioceptive information informs steady gait, increased stride-to-stride variability in force us about the contractile state and movement of muscles, and step length, an inability to maintain balance with about muscle force, heaviness, stiffness, viscosity and ef- eyes closed (Romberg’s sign), a severely reduced ability fort and, thus, is required for any coordinated move- to identify the direction of joint movements, and an ab- ment, normal gait and for the maintenance of a stable sence of tendon reflexes [6–12]. -
A Syndrome-Based Clinical Approach for Clerkship Students General Comments 1. This Is Not an All-Inclusive “Cookbook” for Ev
A Syndrome-Based Clinical Approach for Clerkship Students General Comments 1. This is not an all-inclusive “cookbook” for every Neurology patient, but a set of guidelines to help you rationally approach patients with certain syndromes (sets of signs and symptoms which suggest a lesion in particular parts of the nervous system). 2. As you obtain a history and perform a neurological physical exam, try initially to localize all the patient’s signs and symptoms to one, single lesion in the nervous system. It may be surprising that a variety of signs and symptoms, at first glance apparently unrelated, on second thought can localize accurately to a single lesion. If this approach fails, then consider multiple, separate lesions for the patient’s signs and symptoms. 3. The tempo or rate at which signs and symptoms develop or occur often suggests the underlying pathological process. a. sudden onset---favors stroke (ischemia or hemorrhage), seizure, migraine (or other headache syndromes), and trauma b. subacute onset---favors inflammatory, infectious or immune-mediated disorders c. chronic onset---favors degenerative disorders, tumors Toximetabolic disorders, potentially treatable and reversible, may mimic lesions in the nervous system, and can evolve at variable tempos. Hereditary conditions may be congenital (present at birth) and nonprogressive or static, or develop later in life, with variable rates of progression. Family members affected by the same genetic disorder may be remarkably similar with regards to onset and clinical severity, while some genetic disorders vary widely regarding when and how severely family members are affected. 4. In the central nervous system, “positive symptoms or phenomena,” such as flashes of light, or a tingling sensation, suggest “excitation” or increased activity in the nervous system: migraine or seizure. -
Cramp Fasciculation Syndrome: a Peripheral Nerve Hyperexcitability Disorder Bhojo A
View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by eCommons@AKU Pakistan Journal of Neurological Sciences (PJNS) Volume 9 | Issue 3 Article 7 7-2014 Cramp fasciculation syndrome: a peripheral nerve hyperexcitability disorder Bhojo A. Khealani Aga Khan University Hospital, Follow this and additional works at: http://ecommons.aku.edu/pjns Part of the Neurology Commons Recommended Citation Khealani, Bhojo A. (2014) "Cramp fasciculation syndrome: a peripheral nerve hyperexcitability disorder," Pakistan Journal of Neurological Sciences (PJNS): Vol. 9: Iss. 3, Article 7. Available at: http://ecommons.aku.edu/pjns/vol9/iss3/7 CASE REPORT CRAMP FASCICULATION SYNDROME: A PERIPHERAL NERVE HYPEREXCITABILITY DISORDER Bhojo A. Khealani Assistant professor, Neurology section, Aga khan University, Karachi Correspondence to: Bhojo A Khealani, Department of Medicine (Neurology), Aga Khan University, Karachi. Email: [email protected] Date of submission: June 28, 2014, Date of revision: August 5, 2014, Date of acceptance:September 1, 2014 ABSTRACT Cramp fasciculation syndrome is mildest among all the peripheral nerve hyperexcitability disorders, which typically presents with cramps, body ache and fasciculations. The diagnosis is based on clinical grounds supported by electrodi- agnostic study. We report a case of young male with two months’ history of body ache, rippling, movements over calves and other body parts, and occasional cramps. His metabolic workup was suggestive of impaired fasting glucose, radio- logic work up (chest X-ray and ultrasound abdomen) was normal, and electrodiagnostic study was significant for fascicu- lation and myokymic discharges. He was started on pregablin and analgesics. To the best of our knowledge this is report first of cramp fasciculation syndrome from Pakistan. -
Joint Contractures and Acquired Deforming Hypertonia in Older People
Annals of Physical and Rehabilitation Medicine 62 (2019) 435–441 Available online at ScienceDirect www.sciencedirect.com Review Joint contractures and acquired deforming hypertonia in older people: Which determinants? a,b, c d,e a Patrick Dehail *, Nathaly Gaudreault , Haodong Zhou , Ve´ronique Cressot , a,f g h Anne Martineau , Julie Kirouac-Laplante , Guy Trudel a Service de me´decine physique et re´adaptation, poˆle de neurosciences cliniques, CHU de Bordeaux, 33000 Bordeaux, France b Universite´ de Bordeaux, EA 4136, 33000 Bordeaux, France c E´cole de re´adaptation, faculte´ de me´decine et des sciences de la sante´, universite´ de Sherbrooke, Sherbrooke, Canada d Department of Biology, Faculty of Science, University of Ottawa, Ottawa, ON, Canada e Bone and Joint Research Laboratory, Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada f De´partement de me´decine, division de physiatrie, universite´ Laval, Que´bec, QC, Canada g De´partement de me´decine, division de ge´riatrie, universite´ Laval, Que´bec, QC, Canada h Department of Medicine, Division of Physical Medicine and Rehabilitation, University of Ottawa, Bone and Joint Research Laboratory, The Ottawa Hospital Research Institute, Ottawa, ON, Canada A R T I C L E I N F O A B S T R A C T Article history: Joint contractures and acquired deforming hypertonia are frequent in dependent older people. The Received 29 March 2018 consequences of these conditions can be significant for activities of daily living as well as comfort and Accepted 29 October 2018 quality of life. They can also negatively affect the burden of care and care costs. -
Interpretation of Sensory Information from Skeletal Muscle Receptors for External Control Milan Djilas
Interpretation of Sensory Information From Skeletal Muscle Receptors For External Control Milan Djilas To cite this version: Milan Djilas. Interpretation of Sensory Information From Skeletal Muscle Receptors For External Control. Automatic. Université Montpellier II - Sciences et Techniques du Languedoc, 2008. English. tel-00333530 HAL Id: tel-00333530 https://tel.archives-ouvertes.fr/tel-00333530 Submitted on 23 Oct 2008 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. UNIVERSITE MONTPELLIER II SCIENCES ET TECHNIQUES DU LANGUEDOC T H E S E pour obtenir le grade de DOCTEUR DE L'UNIVERSITE MONTPELLIER II Formation doctorale: SYSTEMES AUTOMATIQUES ET MICROELECTRONIQUES Ecole Doctorale: INFORMATION, STRUCTURES ET SYSTEMES présentée et soutenue publiquement par Milan DJILAS le 13 octobre 2008 Titre: INTERPRETATION DES INFORMATIONS SENSORIELLES DES RECEPTEURS DU MUSCLE SQUELETTIQUE POUR LE CONTROLE EXTERNE INTERPRETATION OF SENSORY INFORMATION FROM SKELETAL MUSCLE RECEPTORS FOR EXTERNAL CONTROL JURY Jacques LEVY VEHEL Directeur de Recherches, INRIA Rapporteur -
Management and Investigation of Neonatal Encephalopathy: 2017 Update Kathryn Martinello,1 Anthony R Hart,2 Sufin Yap,3 Subhabrata Mitra,1 Nicola J Robertson1
Review Arch Dis Child Fetal Neonatal Ed: first published as 10.1136/archdischild-2015-309639 on 6 April 2017. Downloaded from Management and investigation of neonatal encephalopathy: 2017 update Kathryn Martinello,1 Anthony R Hart,2 Sufin Yap,3 Subhabrata Mitra,1 Nicola J Robertson1 1Department of Neonatology, ABSTRACT definite aetiological diagnosis is known, and Institute for Women’s Health, This review discusses an approach to determining the hypoxic-ischaemic encephalopathy (HIE) where University College London, UK 2 cause of neonatal encephalopathy, as well as current clear diagnosis of hypoxia-ischaemia is known to Department of Neonatal and ’ Paediatric Neurology, Sheffield evidence on resuscitation and subsequent management have led to the neonate s clinical state. Children’s Hospital NHS of hypoxic-ischaemic encephalopathy (HIE). Foundation Trust, Sheffield, UK Encephalopathy in neonates can be due to varied 3 DETERMINING THE AETIOLOGY OF NE Department of Inherited aetiologies in addition to hypoxic-ischaemia. A Metabolic Diseases, Sheffield The initial stages of managing NE will be the same Children’s Hospital NHS combination of careful history, examination and the for most babies, with good resuscitation and sup- Foundation Trust, Sheffield, UK judicious use of investigations can help determine the portive management. However, as the picture cause. Over the last 7 years, infants with moderate to evolves and investigations return, clinicians should fi Correspondence to severe HIE have bene ted from the introduction of consider the aetiology of NE as this could lead to Professor Nicola J Robertson, routine therapeutic hypothermia; the number needed to specific treatments, aid with prognosis and recur- Institute for Women’s Health, treat for an additional beneficial outcome is 7 (95% CI University College London, 74 rence risk counselling, and assist with the evalu- Huntley Street, London WC1E 5 to 10). -
Local Control of Human Umbilical Vessel Tone
LOCAL CONTROL OF HUMAN UMBILICAL VESSEL TONE. Thesis submitted to the University of London for the degree of Doctor of Philosophy in the Faculty of Science. by Anita-Jane Sexton B.Sc. (Hons.) Department of Anatomy and Developmental Biology University College London. ProQuest Number: 10017796 All rights reserved INFORMATION TO ALL USERS The quality of this reproduction is dependent upon the quality of the copy submitted. In the unlikely event that the author did not send a complete manuscript and there are missing pages, these will be noted. Also, if material had to be removed, a note will indicate the deletion. uest. ProQuest 10017796 Published by ProQuest LLC(2016). Copyright of the Dissertation is held by the Author. All rights reserved. This work is protected against unauthorized copying under Title 17, United States Code. Microform Edition © ProQuest LLC. ProQuest LLC 789 East Eisenhower Parkway P.O. Box 1346 Ann Arbor, Ml 48106-1346 ABSTRACT This thesis examines the influence of the vascular endothelium on the local control of human feto-placental vascular tone and how these influences may vary with gestation. Electron microscopic studies have revealed that human umbilical vessels are devoid of nerves throughout gestation. Structural changes in the arterial and venous wall could be detected throughout gestation. The presence of a subpopulation of endothelial cells containing nitric oxide synthase (NOS)-like immunoreactivity was identified only in late pregnancy. The umbilical artery and vein, from both early and late pregnancy, did not relax to acetylcholine (ACh), adenosine 5-triphosphate (ATP), substance P (SP) and calcium ionophore A23187, known to be endothelium-dependent vasodilators, but did relax to sodium nitroprusside (SNP). -
Child Neurology: Hereditary Spastic Paraplegia in Children S.T
RESIDENT & FELLOW SECTION Child Neurology: Section Editor Hereditary spastic paraplegia in children Mitchell S.V. Elkind, MD, MS S.T. de Bot, MD Because the medical literature on hereditary spastic clinical feature is progressive lower limb spasticity B.P.C. van de paraplegia (HSP) is dominated by descriptions of secondary to pyramidal tract dysfunction. HSP is Warrenburg, MD, adult case series, there is less emphasis on the genetic classified as pure if neurologic signs are limited to the PhD evaluation in suspected pediatric cases of HSP. The lower limbs (although urinary urgency and mild im- H.P.H. Kremer, differential diagnosis of progressive spastic paraplegia pairment of vibration perception in the distal lower MD, PhD strongly depends on the age at onset, as well as the ac- extremities may occur). In contrast, complicated M.A.A.P. Willemsen, companying clinical features, possible abnormalities on forms of HSP display additional neurologic and MRI abnormalities such as ataxia, more significant periph- MD, PhD MRI, and family history. In order to develop a rational eral neuropathy, mental retardation, or a thin corpus diagnostic strategy for pediatric HSP cases, we per- callosum. HSP may be inherited as an autosomal formed a literature search focusing on presenting signs Address correspondence and dominant, autosomal recessive, or X-linked disease. reprint requests to Dr. S.T. de and symptoms, age at onset, and genotype. We present Over 40 loci and nearly 20 genes have already been Bot, Radboud University a case of a young boy with a REEP1 (SPG31) mutation. Nijmegen Medical Centre, identified.1 Autosomal dominant transmission is ob- Department of Neurology, PO served in 70% to 80% of all cases and typically re- Box 9101, 6500 HB, Nijmegen, CASE REPORT A 4-year-old boy presented with 2 the Netherlands progressive walking difficulties from the time he sults in pure HSP. -
Treatment of Autonomic Dysreflexia for Adults & Adolescents with Spinal
Treatment of Autonomic Dysreflexia for Adults & Adolescents with Spinal Cord Injuries Authors: Dr James Middleton, Director, State Spinal Cord Injury Service, NSW Agency for Clinical Innovation. Dr Kumaran Ramakrishnan, Honorary Fellow, Rehabilitation Studies Unit, Sydney Medical School Northern, The University of Sydney, and Consultant Rehabilitation Physician & Senior Lecturer, Department of Rehabilitation Medicine, University Malaya. Dr Ian Cameron, Head of the Rehabilitation Studies Unit, Sydney Medical School Northern, The University of Sydney. Reviewed and updated in 2013 by the authors. AGENCY FOR CLINICAL INNOVATION Level 4, Sage Building 67 Albert Avenue Chatswood NSW 2067 PO Box 699 Chatswood NSW 2057 T +61 2 9464 4666 | F +61 2 9464 4728 E [email protected] | www.aci.health.nsw.gov.au Produced by the NSW State Spinal Cord Injury Service. SHPN: (ACI) 140038 ISBN: 978-1-74187-972-8 Further copies of this publication can be obtained from the Agency for Clinical Innovation website at: www.aci.health.nsw.gov.au Disclaimer: Content within this publication was accurate at the time of publication. This work is copyright. It may be reproduced in whole or part for study or training purposes subject to the inclusion of an acknowledgment of the source. It may not be reproduced for commercial usage or sale. Reproduction for purposes other than those indicated above, requires written permission from the Agency for Clinical Innovation. © Agency for Clinical Innovation 2014 Published: February 2014 HS13-136 ACKNOWLEDGEMENTS This document was originally published as a fact sheet for the Rural Spinal Cord Injury Project (RSCIP), a pilot healthcare program for people with a spinal cord injury (SCI) conducted within New South Wales involving the collaboration of Prince Henry & Prince of Wales Hospitals, Royal North Shore Hospital, Royal Rehabilitation Centre Sydney, Spinal Cord Injuries Australia and the Paraplegic & Quadriplegic Association of NSW. -
Scienti®C Review Spastic Movement Disorder
Spinal Cord (2000) 38, 389 ± 393 ã 2000 International Medical Society of Paraplegia All rights reserved 1362 ± 4393/00 $15.00 www.nature.com/sc Scienti®c Review Spastic movement disorder V Dietz*,1 1Paracare, Paraplegic Centre of the University Hospital Balgrist, ZuÈrich, Switzerland This review deals with the neuronal mechanisms underlying spastic movement disorder, assessed by electrophysiological means with the aim of ®rst, a better understanding of the underlying pathophysiology and second, the selection of an adequate treatment. For the patient usually one of the ®rst symptoms of a lesion within the central motor system represents the movement disorder, which is most characteristic during locomotion in patients with spasticity. The clinical examination reveals exaggerated tendon tap re¯exes and increased muscle tone typical of the spastic movement disorder. However, today we know that there exists only a weak relationship between the physical signs obtained during the clinical examination in a passive motor condition and the impaired neuronal mechanisms being in operation during an active movement. By the recording and analysis of electrophysiological and biomechanical parameters during a functional movement such as locomotion, the signi®cance of, for example, impaired re¯ex behaviour or pathophysiology of muscle tone and its contribution to the movement disorder can reliably be assessed. Consequently, an adequate treatment should not be restricted to the cosmetic therapy and correction of an isolated clinical parameter but should be based on the pathophysiology and signi®cance of the mechanisms underlying the disorder of functional movement which impairs the patient. Spinal Cord (2000) 38, 389 ± 393 Keywords: spinal cord injury; spasticity; electrophysiological recordings; treatment Introduction Movement disorders are prominent features of impaired strength of electromyographic (EMG) activation of function of the motor systems and are frequently best antagonistic leg muscles as well as intrinsic and passive re¯ected during gait. -
Vet Oracle Teleneurology: Client Factsheet
Client Factsheet Paroxysmal Dyskinesia Paroxysmal Dyskinesia: A little bit of background Paroxysmal dyskinesias (PDs) are episodic movement disorders in which abnormal movements are present only during attacks. Although increasingly being recognised, they are often poorly characterised in veterinary literature and are commonly mistaken for an epileptic seizure, both by owners and by vets. The term ‘paroxysmal’ indicates that the signs occur suddenly against a background of normality. The term ‘dyskinesia’ broadly refers to a movement of the body that is involuntary, which means that the animal has no control over the movement and remains fully aware of its surroundings. Between attacks, affected animals are totally normal and there is no loss of consciousness during the attacks, though some animals may find the episodes disconcerting and do not respond normally. The attacks can last anything from a few minutes to a couple of hours and can sometime occur multiple times in a day. What causes Paroxysmal Dyskinesia? Most neurologists consider that PD results from dysfunction an area of the brain called the basal nuclei (often call the basal ganglia) and the cerebellum which is a fundamental part of the brain that involves in coordinating movement. Nerve cells in the basal nuclei play an important role in initiating and controlling movement. It is thought that abnormal signal from the cerebellum causes abnormal activity of the basal nuclei, which results in spontaneous and uncontrolled muscle activity and, therefore, movement and posture. The underlying cause of many PDs is unknown, with the majority being described as idiopathic (meaning of unknown cause) and presumed to be related to abnormal brain signalling between different parts involved with movement or its feedback control. -
Hypotonia and Lethargy in a Two-Day-Old Male Infant Adrienne H
Hypotonia and Lethargy in a Two-Day-Old Male Infant Adrienne H. Long, MD, PhD,a,b Jennifer G. Fiore, MD,a,b Riaz Gillani, MD,a,b Laurie M. Douglass, MD,c Alan M. Fujii, MD,d Jodi D. Hoffman, MDe A 2-day old term male infant was found to be hypotonic and minimally abstract reactive during routine nursing care in the newborn nursery. At 40 hours of life, he was hypoglycemic and had intermittent desaturations to 70%. His mother had an unremarkable pregnancy and spontaneous vaginal delivery. The mother’s prenatal serology results were negative for infectious risk factors. Apgar scores were 9 at 1 and 5 minutes of life. On day 1 of life, he fed, stooled, and voided well. Our expert panel discusses the differential diagnosis of hypotonia in a neonate, offers diagnostic and management recommendations, and discusses the final diagnosis. DRS LONG, FIORE, AND GILLANI, birth weight was 3.4 kg (56th PEDIATRIC RESIDENTS percentile), length was 52 cm (87th aDepartment of Medicine, Boston Children’s Hospital, d e percentile), and head circumference Boston, Massachusetts; and Neonatology Section, Medical A 2-day old male infant born at Genetics Section, cDivision of Child Neurology, and 38 weeks and 4 days was found to be was 33 cm (12th percentile). His bDepartment of Pediatrics, Boston Medical Center, Boston, limp and minimally reactive during physical examination at birth was Massachusetts routine care in the newborn nursery. normal for gestational age, with Drs Long, Fiore, and Gillani conceptualized, drafted, Just 5 hours before, he had an appropriate neurologic, cardiac, and and edited the manuscript; Drs Douglass, Fujii, and appropriate neurologic status when respiratory components.