DOCSLIB.ORG

Introduction to Classification

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Introduction to Classification 12/06/2020 INTRODUCTION TO CLASSIFICATION Dianne Zeller 2020 1 Objectives • Introduce and familiarise participants with commonly used and current Classification systems 2 Classification • The act or process of dividing things into groups according to their type • Classifying children with Cerebral Palsy according to their type of CP 3 12/06/2020 Why do we Classify? • Common language • To assist in treatment planning • To help predict potential abilities and possible associated problems • For research –for consistency and comparisons across groups of children with CP • Epidemiology • To plan current and future service needs • Evaluation of change over time 4 Difficulties with classification • CP is an umbrella term for a heterogeneous groups of disorders • The clinical picture changes • The terminology is confusing • Classification systems have different purposes 5 Classification systems can be based on: • Subtype –quality and distribution of tone • Functional abilities • Pathology • THERE IS NO UNIVERSAL CLASSIFICATION SYSTEM 6 12/06/2020 Current Classification Systems • Bobath/NDT Classification System • SCPE: Surveillance of Cerebral Palsy in Europe • Washington Workshop: 7 Bobath NDT Classification System Based on Quality of Tone and Distribution • 4 Main Groups 1. Spastic Hypertonia-Always increased tone in one or more limbs • Quadriplegia, Diplegia, Hemiplegia • Mild, Moderate, Severe 2. Dyskinaesia- Presence of excess or unwanted movement • Dystonic • Chore-Athetosis • Pure Athetosis 3. Ataxia- Generalised hypotonia with cerebellar signs 4. Hypotonia 8 Continuum of tone Spastic hypertonicity-Always above normal Severe Hypertonia Moderate Mild Pure Choreo Normal Mild Moderate Dystonia with Hypotonia Severe Dystonic spasticity 9 12/06/2020 SCPE: Surveillance of Cerebral Palsy in Europe • Established in Europe in 1998- in 8 European countries • Establish a data base • Monitor specific trends • Provide information for service planning • Collaborative research 10 SCPE Definition of CP (2000) • Cerebral Palsy (CP) is a group of disorders involving movement and posture and of motor function; it is permanent, but not unchanging; it is due to a non- progressive interference, lesion, or abnormality of the developing/immature brain. (SCPE, Dev Med Child Neurol 42 (2000) 816-824) 11 Classification tree of Cerebral Palsy Sub types SCPE classification(Cans C., (2000) DMCN, 42: 816-824) 12 12/06/2020 SCPE Classification(Cans C., (2000) DMCN, 42: 816- 824) • Bilateral Spastic • Unilateral • Dystonic-hypokinesia, hypertonia Dyskinetic • Choreoathetotic-hyperkinesia, hypotonia Ataxic 13 Classification: Clinical subtype Quality of Tone • Hypertonia: • Hypotonia • Fluctuating, varies through a wide range Anatomical distribution • Bilateral- Previously known as Quadruplegia/Diplegia • Hemiplegia-Unilateral • * 14 Spastic Hypertonia Is there persisting increased muscle tone in one or more limbs Y Are both sides of the body involved Y N Spastic Bilateral Spastic Unilateral Spastic CP is characterized by at least two of the following : Abnormal pattern of posture and/or movement Increased tone (not necessarily constant) Pathological reflexes (increased reflexes; hyperreflexia and / or pyramidal signs eg. Babinski response) 15 12/06/2020 Hypertonia- • Tone is defined as “resistance to passive stretch while the patient is attempting to maintain a relaxed state of muscle activity”. It reflects the state of active muscle contraction • Hypertonia is defined as “abnormally increased resistance to externally imposed movement about a joint”. Sanger et al., (2003) Pediatrics 111: 89) • It is measured at rest or during involuntary movement but not on voluntary muscle contraction 16 Hypertonia vs spasticity • Are they the same thing? • Hypertonia has 2 components: • Neural component: Spasticity • Non neural component: Muscle properties, Joint capsule restrictions • The presence of spasticity suggests the presence of hypertonia(Sanger, (2003)) 17 Hypertonia, Upper Motor Neurone Syndrome and Spasticity: Clinical Management and Neurophysiology , Johnson et al 2001 Neural related, eg spasticity Upper motor neuron Increased resistance to syndrome passive movement Non neural related e.g changes in muscle, mechanical properties Figure 1. Neural and non neural mechanisms contributing to increased resistance to passive motion in an upper motor neuron syndrome 18 12/06/2020 Hypertonia • May be caused by spasticity, dystonia, rigidity, or a combination of features Spastic Dystonic Hypertonia Hypertonia Rigid Hypertonia Classification and Definition of Disorders Causing Hypertonia in Childhood Terence D. Sanger, et al Pediatrics, 2003;111;e89, DOI: 10.1542/peds.111.1.e89 19 Spastic Hypertonia. Predominant Lesion in the Brain: Cortex and Corticospinal tracts 20 Theory of Spasticity The exaggeration of the stretch reflex in patients with spasticity could be produced by two factors. 1. An increased excitability of muscle spindles. In this case, passive muscle stretch in a patient with spasticity would induce a greater activation of spindle afferents with respect to that induced in a normal subject, of course considering a similar velocity and amplitude of passive displacements. 2. The second factor is an abnormal processing of sensory inputs from muscle spindles in the spinal cord, leading to an excessive reflex activation of 훼-motoneurons. 21 12/06/2020 22 Definition of Spasticity The pathophysiology of spasticity, G. Sheean, European Journal of Neurology 2002, 9 (Suppl. 1): 3–9 Spasticity ……….is a form of hypertonia due to a velocity-dependent increase in tonic stretch reflexes, which results from abnormal spinal processing of proprioceptive input. Velocity dependent: Resistance increases as speed of lengthening increases • Tested using Ashworth , Tardieu • R1: Range at which a catch is felt when moving the limb quickly • R2 Range at which a catch is felt when moving a limb slowly 23 24 12/06/2020 Dyskinetic CP Is there persisting increased muscle tone in one or more limbs? N Is the tone varying? Y N Dyskinetic CP Unclassifiable Reduced activity-Tone Increased activity –Tone tends to be increased tends to be decreased Dystonic CP Choreo-athetotic CP 25 Dyskinetic CP is dominated by both : Abnormal pattern of posture and/or movement Involuntary, uncontrolled, recurring, occasionally stereotyped movements 26 Predominant lesion in the Brain- Basal Ganglia Basal ganglia are strongly interconnected with the cerebral cortex, thalamus, and brainstem, as well as several other brain areas. The basal ganglia are associated with a variety of functions, including control of voluntary motor movements, procedural learning, habit learning, eye movements, cognition, and emotion. 27 12/06/2020 Dystonia • A movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both (Sanger et al., 2009) • . 28 Sanger article……. • Dystonia could potentially cause hyperkinetic movements by inserting dystonic postures that result in movement of a part of the body away from the intended position. • If sustained dystonic postures are inserted in voluntary movements, this could result in the prevention of movement, substitution of an unwanted posture for an intended posture, or slow movement in an attempt to overcome the inserted dystonic posture. • Thus in our definitions, dystonia is characterized by one or more repeated postures. Chorea is characterized by multiple repeated but not rhythmic movements. Athetosis is characterized by nonrhythmic movements without intervening postures. 29 Dystonic CP Reduced activity (Hypokinesia), tone tends to be increased( Hypertonia) 30 12/06/2020 Choreo-Athetotic CP • Chorea is distinguished from dystonia due to the apparently random, unpredictable, and continuously ongoing nature of the movements, compared with the more predictable and stereotyped movements or postures of dystonia. • Athetosis involves continuous smooth movements that appear random and are not composed of recognizable sub-movements or movement fragments. The term “athetosis” derives from the Greek meaning “without position or place,” reflecting the inability to maintain a stable posture • Chorea is characterized by multiple repeated but not rhythmic movements. Athetosis is characterized by non-rhythmic movements without intervening postures. 31 Choreo-athetotic CP Increased activity (Hyperkinesia); tone tends to be decreased (Hypotonia) 32 Ataxia • Is there persisting increased muscle tone in one or more limbs N Is the tone varying? Is there generalised hypotonia with signs of ataxia? Y N Ataxia Unclassifiable 33 12/06/2020 Ataxia • The term “Ataxia” is derived from Greek word meaning ‘lack of order’ • “Ataxia” is defined as an inability to generate a normal or expected voluntary movement trajectory that cannot be attributed to weakness or involuntary muscle activity about the affected joints (Sanger et al, 2003) • Mismatch between intention and action Ataxic CP is characterized by both : Abnormal pattern of posture and / or movement Loss of orderly muscular coordination so that movements are performed with abnormal force, rhythm and accuracy 34 Main body of cerebellum- 3 functional areas Functional area Function Indication of dysfunction Vestibulocerebellum Equilibrium Cannot control eye movements Postural control/movement during head rotation and when Reciprocal innervation standing and walking. (agonist/antagonist) Wide base of support (Low Anticipatory correction COG) Eye movements Inefficient balance
Load more

Recommended publications
  • The Epidemiology of the Cerebral Palsies
    Clinics in Developmental Medicine No. 87 The Epidemiology of the Cerebral Palsies Edited by FIONA STANLEY EVA ALBERMAN 1984 Spastics International Medical Publications OXFORD: Blackwell Scientific Publications Ltd. PHILADELPHIA: J. B. Lippincott Co. CHAPTER 11 Prenatal and Perinatal Risk Factors in a Survey of 681 Swedish Cases BENGT and GUDRUN HAGBERG I, that am curtail'd of this fair proportion, Cheated of feature by dissembling nature, , Deform'd, unfinish'd, sent before my time Into this breathing world, scarce half made up, And that so lamely and unfashionable That dogs bark at me, as I halt by them. Shakespeare, Richard III Introduction The aim of this chapter is to try and shed light on more specific aspects of the main groups of prenatal causes and risk factors for cerebral palsy, their mutual importance, and particularly their relationship to superimposed detrimental perinatal events. This survey is based on an investigation of 681 cases born in Sweden from 1959 to 1976. In our original retrospective analysis of causes of cerebral palsy (Hagberg et al. 1975a), it was found necessary to make certain generalisations about the aetiological groupings. Only the risk factor that was considered to be the predominating possible cause was used for classification. There is no doubt that this oversimplifies the issue as it neglects the complex network of different interacting detrimental risk factors that are present in the majority of cases, and in all probability underlie the development of brain lesions. Definitions For the purpose of the Swedish investigation, the following definition of cerebral palsy was used: a non-progressive 'disorder of movement and posture due to a defect or lesion of the immature brain' (Bax 1964).
    [Show full text]
  • Cerebral Palsy the ABC's of CP
    Cerebral Palsy The ABC’s of CP Toni Benton, M.D. Continuum of Care Project UNM HSC School of Medicine April 20, 2006 Cerebral Palsy Outline I. Definition II. Incidence, Epidemiology and Distribution III. Etiology IV. Types V. Medical Management VI. Psychosocial Issues VII. Aging Cerebral Palsy-Definition Cerebral palsy is a symptom complex, (not a disease) that has multiple etiologies. CP is a disorder of tone, posture or movement due to a lesion in the developing brain. Lesion results in paralysis, weakness, incoordination or abnormal movement Not contagious, no cure. It is static, but it symptoms may change with maturation Cerebral Palsy Brain damage Occurs during developmental period Motor dysfunction Not Curable Non-progressive (static) Any regression or deterioration of motor or intellectual skills should prompt a search for a degenerative disease Therapy can help improve function Cerebral Palsy There are 2 major types of CP, depending on location of lesions: Pyramidal (Spastic) Extrapyramidal There is overlap of both symptoms and anatomic lesions. The pyramidal system carries the signal for muscle contraction. The extrapyramidal system provides regulatory influences on that contraction. Cerebral Palsy Types of brain damage Bleeding Brain malformation Trauma to brain Lack of oxygen Infection Toxins Unknown Epidemiology The overall prevalence of cerebral palsy ranges from 1.5 to 2.5 per 1000 live births. The overall prevalence of CP has remained stable since the 1960’s. Speculations that the increased survival of the VLBW preemies would cause a rise in the prevalence of CP have proven wrong. Likewise the expected decrease in CP as a result of C-section and fetal monitoring has not happened.
    [Show full text]
  • ICD9 & ICD10 Neuromuscular Codes
    ICD-9-CM and ICD-10-CM NEUROMUSCULAR DIAGNOSIS CODES ICD-9-CM ICD-10-CM Focal Neuropathy Mononeuropathy G56.00 Carpal tunnel syndrome, unspecified Carpal tunnel syndrome 354.00 G56.00 upper limb Other lesions of median nerve, Other median nerve lesion 354.10 G56.10 unspecified upper limb Lesion of ulnar nerve, unspecified Lesion of ulnar nerve 354.20 G56.20 upper limb Lesion of radial nerve, unspecified Lesion of radial nerve 354.30 G56.30 upper limb Lesion of sciatic nerve, unspecified Sciatic nerve lesion (Piriformis syndrome) 355.00 G57.00 lower limb Meralgia paresthetica, unspecified Meralgia paresthetica 355.10 G57.10 lower limb Lesion of lateral popiteal nerve, Peroneal nerve (lesion of lateral popiteal nerve) 355.30 G57.30 unspecified lower limb Tarsal tunnel syndrome, unspecified Tarsal tunnel syndrome 355.50 G57.50 lower limb Plexus Brachial plexus lesion 353.00 Brachial plexus disorders G54.0 Brachial neuralgia (or radiculitis NOS) 723.40 Radiculopathy, cervical region M54.12 Radiculopathy, cervicothoracic region M54.13 Thoracic outlet syndrome (Thoracic root Thoracic root disorders, not elsewhere 353.00 G54.3 lesions, not elsewhere classified) classified Lumbosacral plexus lesion 353.10 Lumbosacral plexus disorders G54.1 Neuralgic amyotrophy 353.50 Neuralgic amyotrophy G54.5 Root Cervical radiculopathy (Intervertebral disc Cervical disc disorder with myelopathy, 722.71 M50.00 disorder with myelopathy, cervical region) unspecified cervical region Lumbosacral root lesions (Degeneration of Other intervertebral disc degeneration,
    [Show full text]
  • Acute Flaccid Paralysis Field Manual
    Republic of Iraq Ministry of Health Expanded program of immunization Acute Flaccid Paralysis Field Manual For Communicable Diseases Surveillance Staff With Major funding from EU 2009 1 C o n t e n t 5- Forms 35 1- Introduction 6 A form for immediate notification of “acute flaccid paralysis”, FORM (1) 37 2-Acute poliomyelitis 10 A case investigation form for acute flaccid paralysis, FORM (2 28 Poliovirus 10 A laboratory request reporting form for submission of stool specimen, FORM (3) 40 Epidemiology 10 A form for 60-day follow-up examination of AFP case, FORM (4) 41 Pathogenesis 11 A form for final classification of AFP case, FORM (5) 41 Clinical features 11 A form for AFP case’s contacts examination, FORM (6) 42 Laboratory diagnosis 12 A line listing form for all reported AFP cases, FORM (7) 43 Differential diagnosis 12 A line listing form for AFP cases undergoing “expert review”, FORM (8) 44 Poliovirus vaccine 13 A weekly reporting form, including “acute flaccid paralysis “, FORM (9) 45 A monthly reporting forms, including “acute flaccid paralysis and polio cases”, FORM (10) 46 3-Surveillance 14 A weekly active surveillance form, FORM (11) 47 Purpose of disease surveillance 14 A form to monitor completeness and timeliness of weekly reports received, FORM (12) 49 Attributes of disease surveillance 14 6- Tables 50 4-Acute Flaccid Paralysis Surveillance 15 Table (1) Annual reported polio cases 1955-2003 Iraq 50 The role of AFP surveillance 15 Table (2) Differential diagnosis of poliomyelitis 50 The role of laboratory in AFP surveillance 16 Types of AFP surveillance 16 7- Figures 53 Steps to develop AFP surveillance 17 Figure (1) Annual reported polio cases, 1955-2000 Iraq 53 How to initiate AFP surveillance 22 Figure (2) Phases of occurrence of symptoms in polio infection 53 AFP surveillance in risk areas and population 22 Figure (3) Classification of AFP cases.
    [Show full text]
  • Miller Fisher Syndrome, Facial Diplegia and Multiple Cranial Nerve Palsies Ashfaq Shuaib and Werner J
    LE JOURNAL CANADIEN DES SCIENCES NEUROLOGIQUES Variants of Guillain-Barre Syndrome: Miller Fisher Syndrome, Facial Diplegia and Multiple Cranial Nerve Palsies Ashfaq Shuaib and Werner J. Becker ABSTRACT: We report the experience at a large teaching hospital over a 10 year period with Miller Fisher Syndrome, facial diplegia, and multiple cranial nerve palsies. In these patients, absence of drowsiness on examination, normal cranial CT scans, albumino-cytological dissociation on CSF examination and slowing of nerve conduction, all suggest that a peripheral nerve dysfunction is the underlying mechanism. Pertinent literature is reviewed, in an attempt to separate these probable variants of Guillain-Barre' Syndrome from brainstem encephalitis, with which they may be confused. RESUME: Variantes du syndrome de Guillain-Barre: le syndrome de Miller-Fisher, la diplegie faciale et les paralysies multiples des nerfs craniens. Nous rapportons l'observation de patients atteints du syndrome de Miller-Fisher, de diplegie faciale et de paralysies multiples des nerfs craniens, ayant consulte dans un hopital d'enseignement sur une p6riode de dix ans. Chez ces patients, l'absence de somnolence a l'examen, un CT-scan cranien normal, une dissociation albumino-cytologique a l'examen du LCR et une conduction lente a l'etude de la conduction nerveuse, suggerent que la dysfonction au niveau du nerf p6ripherique en est le m6canisme sous-jacent. Nous revoyons la literature pertinente, en tentant de separer ces variantes probables du syndrome de Guillain-Barr6 de l'enc6phalite du tronc cerebral, entite avec laquelle elles peuvent etre confondues. Can. J. Neurol. Sci. 1987; 14:611-616 The syndrome of ataxia, areflexia and ophthalmoplegia (AAO), We report here our analysis of patients admitted over a ten initially described by Bogaert et al in 1938,' became widely year period with AAO, multiple cranial nerve dysfunction and recognized after Miller Fisher's classic description of three facial diplegia.
    [Show full text]
  • Research Journal of Pharmaceutical, Biological and Chemical Sciences
    ISSN: 0975-8585 Research Journal of Pharmaceutical, Biological and Chemical Sciences The Ability to Reduce the Severity of Motor Disorders in Children With Cerebral Palsy. Makhov AS, and Medvedev IN*. Russian State Social University, st. V. Pika, 4, Moscow, Russia, 129226 ABSTRACT Children's cerebral palsy remains a frequent pathology in children. The effectiveness of the schemes used for the rehabilitation of these children remains low. Purpose: to evaluate the effectiveness of the author's technique, including therapeutic gymnastics and technical means, in children 7-9 years old with infantile cerebral palsy. The study was performed on 35 children aged 7-9 years with infantile cerebral palsy: 17 of them made up a control group, 18 - experimental. In the children studied, spastic diplegia or spastic tetra paresis was noted. The children of the experimental group used the author's complex correction, which includes curative gymnastics, the use of strength training, orthoses and verticalizers. Children of the control group are prescribed a traditional correction of infantile cerebral palsy. All children are assessed dynamics of motor skills on the scale of Cheylie, goniometry of knee joints, spasticity of muscles, and strength of the quadriceps muscle of the thigh. The results are processed by Student's test. As a result of the lessons, all children have achieved a positive dynamics of the indicators taken into account. A more pronounced positive dynamics of the strength of the quadriceps muscle (by 19.2%), the level of the motor skill (from 9.3 to 27.8%) and spasticity of the limb (by 26.5%) was achieved in the children of the experimental group.
    [Show full text]
  • Cerebral Palsy
    Cerebral Palsy What is Cerebral Palsy? Doctors use the term cerebral palsy to refer to any one of a number of neurological disorders that appear in infancy or early childhood and permanently affect body movement and muscle coordination but are not progressive, in other words, they do not get worse over time. • Cerebral refers to the motor area of the brain’s outer layer (called the cerebral cortex), the part of the brain that directs muscle movement. • Palsy refers to the loss or impairment of motor function. Even though cerebral palsy affects muscle movement, it is not caused by problems in the muscles or nerves. It is caused by abnormalities inside the brain that disrupt the brain’s ability to control movement and posture. In some cases of cerebral palsy, the cerebral motor cortex has not developed normally during fetal growth. In others, the damage is a result of injury to the brain either before, during, or after birth. In either case, the damage is not repairable and the disabilities that result are permanent. Patients with cerebral palsy exhibit a wide variety of symptoms, including: • Lack of muscle coordination when performing voluntary movements (ataxia); • Stiff or tight muscles and exaggerated reflexes (spasticity); • Walking with one foot or leg dragging; • Walking on the toes, a crouched gait, or a “scissored” gait; • Variations in muscle tone, either too stiff or too floppy; • Excessive drooling or difficulties swallowing or speaking; • Shaking (tremor) or random involuntary movements; and • Difficulty with precise motions, such as writing or buttoning a shirt. The symptoms of cerebral palsy differ in type and severity from one person to the next, and may even change in an individual over time.
    [Show full text]
  • A Dictionary of Neurological Signs
    FM.qxd 9/28/05 11:10 PM Page i A DICTIONARY OF NEUROLOGICAL SIGNS SECOND EDITION FM.qxd 9/28/05 11:10 PM Page iii A DICTIONARY OF NEUROLOGICAL SIGNS SECOND EDITION A.J. LARNER MA, MD, MRCP(UK), DHMSA Consultant Neurologist Walton Centre for Neurology and Neurosurgery, Liverpool Honorary Lecturer in Neuroscience, University of Liverpool Society of Apothecaries’ Honorary Lecturer in the History of Medicine, University of Liverpool Liverpool, U.K. FM.qxd 9/28/05 11:10 PM Page iv A.J. Larner, MA, MD, MRCP(UK), DHMSA Walton Centre for Neurology and Neurosurgery Liverpool, UK Library of Congress Control Number: 2005927413 ISBN-10: 0-387-26214-8 ISBN-13: 978-0387-26214-7 Printed on acid-free paper. © 2006, 2001 Springer Science+Business Media, Inc. All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, Inc., 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dis- similar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to propri- etary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omis- sions that may be made.
    [Show full text]
  • The Ultimate Poker Face: a Case Report of Facial Diplegia, a Guillain-Barré Variant
    CASE REPORT The Ultimate Poker Face: A Case Report of Facial Diplegia, a Guillain-Barré Variant Joshua Lowe, MD Brooke Army Medical Center, Department of Emergency Medicine, Fort Sam Houston, Texas James Pfaff, MD Section Editor: Christopher Sampson, MD Submission history: Submitted October 14, 2019; Revision received February 4, 2020; Accepted February 7, 2020 Electronically published April 23, 2020 Full text available through open access at http://escholarship.org/uc/uciem_cpcem DOI: 10.5811/cpcem.2020.2.45556 Introduction: Facial diplegia, a rare variant of Guillain-Barré syndrome (GBS), is a challenging diagnosis to make in the emergency department due to its resemblance to neurologic Lyme disease. Case report: We present a case of a 27-year-old previously healthy man who presented with bilateral facial paralysis. Discussion: Despite the variance in presentation, the recommended standard of practice for diagnostics (cerebrospinal fluid albumin-cytological dissociation) and disposition (admission for observation, intravenous immunoglobulin, and serial negative inspiratory force) of facial diplegia are the same as for other presentations of GBS. Conclusion: When presented with bilateral facial palsy emergency providers should consider autoimmune, infectious, idiopathic, metabolic, neoplastic, neurologic, and traumatic etiologies in addition to the much more common neurologic Lyme disease. [Clin Pract Cases Emerg Med. 2020;4(2):150–153.] Keywords: Facial Diplegia; Guillain-Barré variant. INTRODUCTION patient had recently traveled to San Antonio, Texas, for Facial diplegia is a rare variant of Guillain-Barré military exercises and received multiple vaccines five days syndrome (GBS) where patients present with bilateral facial prior to onset of symptoms. He began experiencing mild paralysis and paresthesia.
    [Show full text]
  • Mechanisms of Injury in Dyskinetic Cerebral Palsy
    Mechanisms of Injury in Dyskinetic Cerebral Palsy Alec Hoon, MD Associate Professor of Pediatrics Johns Hopkins University School of Medicine Director, Phelps Center for Cerebral Palsy Kennedy Krieger Institute Neurodevelopmental Evaluation Structure‐Function Genetic, Epigenetic, Clinical Phenotype Abnormalities Environmental Risk Factors CP Diagnosis Etiologic Diagnosis Child‐Family Rehabilitative Characteristics Management Medical Surgical Measurement CAM Cell‐Based therapy Techniques Outcome Key Concepts in Cerebral Palsy • Motor control‐ tone, posture, movement • 2‐3/1000 children • Risk factors include infection, inflammation, low birth weight, prematurity, genetic • Secondary to brain dysgenesis or injury • “Non‐progressive”‐ manifestations can change • Unilateral and bilateral phenotypes • A range of associated disorders • Etiology links to phenotype links to treatment 1 Cerebral Palsy‐ Clinical Phenotypes CEREBRAL PALSY Spastic Dyskinetic Ataxic Hypotonia Bilateral Unilateral Hypertonic Hyperkinetic Spastic Diplegia Hemiplegia Dystonic Rigid Dystonic Tetraplegia Quadriplegia Athetosis Chorea Hemiballismus Cascade of events in brain injury 1. Prenatal antecedents may be suspected‐(Freud) 2. Final common pathways often involve hypoxia‐ ischemia and infection‐inflammation 3. Injury may have a protracted time course 4. Injury may lead to myelin abnormalities, reduced plasticity and decreased cell number 5. Injury changes both developmental trajectory and may sensitive brain to later injury The Evolving Nature of Injury TIME
    [Show full text]
  • Treatable Inborn Errors of Metabolism Presenting As Cerebral Palsy Mimics
    Leach et al. Orphanet Journal of Rare Diseases 2014, 9:197 http://www.ojrd.com/content/9/1/197 RESEARCH Open Access Treatable inborn errors of metabolism presenting as cerebral palsy mimics: systematic literature review Emma L Leach1,2, Michael Shevell3,4,KristinBowden2, Sylvia Stockler-Ipsiroglu2,5,6 and Clara DM van Karnebeek2,5,6,7,8* Abstract Background: Inborn errors of metabolism (IEMs) have been anecdotally reported in the literature as presenting with features of cerebral palsy (CP) or misdiagnosed as ‘atypical CP’. A significant proportion is amenable to treatment either directly targeting the underlying pathophysiology (often with improvement of symptoms) or with the potential to halt disease progression and prevent/minimize further damage. Methods: We performed a systematic literature review to identify all reports of IEMs presenting with CP-like symptoms before 5 years of age, and selected those for which evidence for effective treatment exists. Results: We identified 54 treatable IEMs reported to mimic CP, belonging to 13 different biochemical categories. A further 13 treatable IEMs were included, which can present with CP-like symptoms according to expert opinion, but for which no reports in the literature were identified. For 26 of these IEMs, a treatment is available that targets the primary underlying pathophysiology (e.g. neurotransmitter supplements), and for the remainder (n = 41) treatment exerts stabilizing/preventative effects (e.g. emergency regimen). The total number of treatments is 50, and evidence varies for the various treatments from Level 1b, c (n = 2); Level 2a, b, c (n = 16); Level 4 (n = 35); to Level 4–5 (n = 6); Level 5 (n = 8).
    [Show full text]
  • Spastic Diplegia with Nonperinatal Asphyxia in Pediatric Population
    International Physical Medicine & Rehabilitation Journals Case Report Open Access Spastic diplegia with nonperinatal asphyxia in pediatric population Abstract Volume 1 Issue 4 - 2017 Spastic diplegia is a condition where patients have spasticity mainly in the lower Keisuke Ueda,1 Huiyuan Jiang2 extremities and highly associated with cerebral palsy. However, there exist other 1Department of Pediatrics, Children’s Hospital of Michigan, USA entities causing spastic diplegia in a pediatric population some of which are treatable 2Wayne State University, USA and preventable. In this review, we describe disorders causing spastic diplegia which occur due to non-perinatal asphyxia. Correspondence: Huiyuan Jiang, Wayne State University, School of Medicine, Detroit, MI, USA, Tel 313-832-9620, Fax Keywords: spastic diplegia, diagnosis, children 313-745-3012, Email [email protected] Received: April 07, 2017 | Published: July 14, 2017 Abbreviations: CP, cerebralpPalsy; HSP, hereditary spastic Leukodystrophies paraplegia; IEM, inborn errors of metabolism; DRD, dopa-responsive The leukodystrophies are genetic disorders affecting the white dystonia matter of the brain, potentially leading to motor impairments Introduction such as hypotonia in early life and spasticity over time. Several leukodystrophies including Canavan disease,9 Krabbe disease,10 Spastic diplegia is characterized by spasticity in the upper or lower Pelizaeus-Merzbacher disease,11 metachromatic leukodystrophy,12 extremities whereby the lower extremities usually are more affected.
    [Show full text]