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Friedreich Ataxia Mouse Models with Progressive Cerebellar and Sensory Ataxia Reveal Autophagic Neurodegeneration in Dorsal Root Ganglia
The Journal of Neuroscience, February 25, 2004 • 24(8):1987–1995 • 1987 Neurobiology of Disease Friedreich Ataxia Mouse Models with Progressive Cerebellar and Sensory Ataxia Reveal Autophagic Neurodegeneration in Dorsal Root Ganglia Delphine Simon,1 Herve´ Seznec,1 Anne Gansmuller,1 Nade`ge Carelle,1 Philipp Weber,1 Daniel Metzger,1 Pierre Rustin,2 Michel Koenig,1 and He´le`ne Puccio1 1Institut de Ge´ne´tique et de Biologie Mole´culaire et Cellulaire, Centre National de la Recherche Scientifique/Institut National de la Sante´ et de la Recherche Me´dicale (INSERM)/Universite´ Louis Pasteur, 67404 Illkirch cedex, CU de Strasbourg, France, and 2Unite´ de Recherches sur les Handicaps Ge´ne´tiques de l’Enfant, INSERM U393, Hoˆpital Necker-Enfants Malades, 75015 Paris, France Friedreich ataxia (FRDA), the most common recessive ataxia, is characterized by degeneration of the large sensory neurons of the spinal cord and cardiomyopathy. It is caused by severely reduced levels of frataxin, a mitochondrial protein involved in iron–sulfur cluster (ISC) biosynthesis. Through a spatiotemporally controlled conditional gene-targeting approach, we have generated two mouse models for FRDA that specifically develop progressive mixed cerebellar and sensory ataxia, the most prominent neurological features of FRDA. Histological studies showed both spinal cord and dorsal root ganglia (DRG) anomalies with absence of motor neuropathy, a hallmark of the human disease. In addition, one line revealed a cerebellar granule cell loss, whereas both lines had Purkinje cell arborization defects. These lines represent the first FRDA models with a slowly progressive neurological degeneration. We identified an autophagic process as the causative pathological mechanism in the DRG, leading to removal of mitochondrial debris and apparition of lipofuscin deposits. -
Movement Disorders in the Elderly
MOVEMENT DISORDERS IN THE ELDERLY Eugene C. Lai, M.D., Ph.D. Michael E. DeBakey VA Medical Center Baylor College of Medicine Houston, Texas MOVEMENT DISORDERS Neurologic dysfunctions in which there is either a paucity of voluntary and automatic movements (HYPOKINESIA) or an excess of movement (HYPERKINESIA) or uncontrolled movements (DYSKINESIA) typically unassociated with weakness or spasticity HYPOKINESIAS • Parkinson‟s disease • Secondary Parkinsonism • Parkinson‟s plus syndromes HYPERKINESIAS • Akathisia • Hemifacial spasm • Athetosis • Myoclonus • Ballism • Restless leg syndrome • Chorea • Tics • Dystonia • Tremor COMMON MOVEMENT DISORDERS IN THE ELDERLY • Parkinsonism • Tremor • Gait disorder • Restless leg syndrome • Drug-induced syndrome PARKINSONISM • Parkinson‟s disease • Secondary parkinsonism • Drug-induced parkinsonism • Vascular parkinsonism • Parkinson‟s plus syndromes • Multiple system atrophy • Progressive supranuclear palsy PARKINSON’S DISEASE PARKINSON’S DISEASE Classical Clinical Features • Resting Tremor • Cogwheel Rigidity • Bradykinesia • Postural Instability PARKINSON’S DISEASE Associated Clinical Features • Micrographia • Hypophonia • Hypomimia • Shuffling gait / festination • Drooling • Dysphagia NON-MOTOR COMPLICATIONS IN PARKINSON’S DISEASE • Sleep disturbances • Autonomic dysfunctions • Sensory phenomena • Neuropsychiatric manifestations • Cognitive impairment PARKINSON’S DISEASE General Considerations • The second most common progressive neurodegenerative disorder • The most common neurodegenerative movement -
The Clinical Approach to Movement Disorders Wilson F
REVIEWS The clinical approach to movement disorders Wilson F. Abdo, Bart P. C. van de Warrenburg, David J. Burn, Niall P. Quinn and Bastiaan R. Bloem Abstract | Movement disorders are commonly encountered in the clinic. In this Review, aimed at trainees and general neurologists, we provide a practical step-by-step approach to help clinicians in their ‘pattern recognition’ of movement disorders, as part of a process that ultimately leads to the diagnosis. The key to success is establishing the phenomenology of the clinical syndrome, which is determined from the specific combination of the dominant movement disorder, other abnormal movements in patients presenting with a mixed movement disorder, and a set of associated neurological and non-neurological abnormalities. Definition of the clinical syndrome in this manner should, in turn, result in a differential diagnosis. Sometimes, simple pattern recognition will suffice and lead directly to the diagnosis, but often ancillary investigations, guided by the dominant movement disorder, are required. We illustrate this diagnostic process for the most common types of movement disorder, namely, akinetic –rigid syndromes and the various types of hyperkinetic disorders (myoclonus, chorea, tics, dystonia and tremor). Abdo, W. F. et al. Nat. Rev. Neurol. 6, 29–37 (2010); doi:10.1038/nrneurol.2009.196 1 Continuing Medical Education online 85 years. The prevalence of essential tremor—the most common form of tremor—is 4% in people aged over This activity has been planned and implemented in accordance 40 years, increasing to 14% in people over 65 years of with the Essential Areas and policies of the Accreditation Council age.2,3 The prevalence of tics in school-age children and for Continuing Medical Education through the joint sponsorship of 4 MedscapeCME and Nature Publishing Group. -
Ataxia in Children: Early Recognition and Clinical Evaluation Piero Pavone1,6*, Andrea D
Pavone et al. Italian Journal of Pediatrics (2017) 43:6 DOI 10.1186/s13052-016-0325-9 REVIEW Open Access Ataxia in children: early recognition and clinical evaluation Piero Pavone1,6*, Andrea D. Praticò2,3, Vito Pavone4, Riccardo Lubrano5, Raffaele Falsaperla1, Renata Rizzo2 and Martino Ruggieri2 Abstract Background: Ataxia is a sign of different disorders involving any level of the nervous system and consisting of impaired coordination of movement and balance. It is mainly caused by dysfunction of the complex circuitry connecting the basal ganglia, cerebellum and cerebral cortex. A careful history, physical examination and some characteristic maneuvers are useful for the diagnosis of ataxia. Some of the causes of ataxia point toward a benign course, but some cases of ataxia can be severe and particularly frightening. Methods: Here, we describe the primary clinical ways of detecting ataxia, a sign not easily recognizable in children. We also report on the main disorders that cause ataxia in children. Results: The causal events are distinguished and reported according to the course of the disorder: acute, intermittent, chronic-non-progressive and chronic-progressive. Conclusions: Molecular research in the field of ataxia in children is rapidly expanding; on the contrary no similar results have been attained in the field of the treatment since most of the congenital forms remain fully untreatable. Rapid recognition and clinical evaluation of ataxia in children remains of great relevance for therapeutic results and prognostic counseling. Keywords: Ataxia, Diagnostic maneuvers, Acute cerebellitis, Cerebellar syndrome, Cerebellar malformations Background Clinical signs in cerebellar ataxic patients are related to Ataxia in children is a common clinical sign of various impaired localization. -
Ataxia and Tremor in People with Multiple Sclerosis (MS)
for health professionals Ataxia and tremor in people with multiple sclerosis (MS) Freecall: 1800 042 138 www.msaustralia.org.au MS PRACTICE // ATAXIA AND TREMOR IN PEOPLE WITH MULTIPLE SCLEROSIS Ataxia and tremor in people with multiple sclerosis (MS) Ataxia and tremor are common yet difficult symptoms to manage in people with MS ― often requiring the involvement of a multidisciplinary team. Early intervention is important in order to address both the functional and psychological issues associated with these symptoms. Freecall: 1800 042 138 www.msaustralia.org.au MS PRACTICE // ATAXIA AND TREMOR IN PEOPLE WITH MULTIPLE SCLEROSIS 01 Contents Page 1.0 Definitions 02 2.0 Incidence and impact 3.0 Pathophysiology and clinical characteristics 3.1 Ataxia 3.2 Tremor 03 4.0 Assessment 5.0 Management 04 5.1 Physiotherapy 5.2 Pharmacotherapy 05 5.3 Surgical intervention 06 6.0 Summary Freecall: 1800 042 138 www.msaustralia.org.au MS PRACTICE // AtaXIA AND TREMOR IN PEOPLE WITH MULTIPLE SCLEROSIS 02 1.0 Definitions Ataxia Tremor Ataxia is a term used to describe a number of Tremor is defined as a rhythmic, involuntary, oscillating abnormal movements that may occur during the movement of a body part. There are two main execution of voluntary movements. They include, but classifications of tremor ― resting tremor and action are not limited to, incoordination, delay in movement, tremor. Resting tremor is present in a body part that dysmetria (inaccuracy in achieving a target), is completely supported against gravity and is not dysdiadochokinesia (inability -
GAIT DISORDERS, FALLS, IMMOBILITY Mov7 (1)
GAIT DISORDERS, FALLS, IMMOBILITY Mov7 (1) Gait Disorders, Falls, Immobility Last updated: April 17, 2019 GAIT DISORDERS ..................................................................................................................................... 1 CLINICAL FEATURES .............................................................................................................................. 1 CLINICO-ANATOMICAL SYNDROMES .............................................................................................. 1 CLINICO-PHYSIOLOGICAL SYNDROMES ......................................................................................... 1 Dyssynergy Syndromes .................................................................................................................... 1 Frontal gait ............................................................................................................................ 2 Sensory Gait Syndromes .................................................................................................................. 2 Sensory ataxia ........................................................................................................................ 2 Vestibular ataxia .................................................................................................................... 2 Visual ataxia .......................................................................................................................... 2 Multisensory disequilibrium ................................................................................................. -
Handbook on Clinical Neurology and Neurosurgery
Alekseenko YU.V. HANDBOOK ON CLINICAL NEUROLOGY AND NEUROSURGERY FOR STUDENTS OF MEDICAL FACULTY Vitebsk - 2005 УДК 616.8+616.8-089(042.3/;4) ~ А 47 Алексеенко Ю.В. А47 Пособие по неврологии и нейрохирургии для студентов факуль тета подготовки иностранных граждан: пособие / составитель Ю.В. Алексеенко. - Витебск: ВГМ У, 2005,- 495 с. ISBN 985-466-119-9 Учебное пособие по неврологии и нейрохирургии подготовлено в соответствии с типовой учебной программой по неврологии и нейрохирургии для студентов лечебного факультетов медицинских университетов, утвержденной Министерством здравоохра нения Республики Беларусь в 1998 году В учебном пособии представлены ключевые разделы общей и частной клиниче ской неврологии, а также нейрохирургии, которые имеют большое значение в работе врачей общей медицинской практики и системе неотложной медицинской помощи: за болевания периферической нервной системы, нарушения мозгового кровообращения, инфекционно-воспалительные поражения нервной системы, эпилепсия и судорожные синдромы, демиелинизирующие и дегенеративные поражения нервной системы, опу холи головного мозга и черепно-мозговые повреждения. Учебное пособие предназначено для студентов медицинского университета и врачей-стажеров, проходящих подготовку по неврологии и нейрохирургии. if' \ * /’ L ^ ' i L " / УДК 616.8+616.8-089(042.3/.4) ББК 56.1я7 б.:: удгритний I ISBN 985-466-119-9 2 CONTENTS Abbreviations 4 Motor System and Movement Disorders 5 Motor Deficit 12 Movement (Extrapyramidal) Disorders 25 Ataxia 36 Sensory System and Disorders of Sensation -
Clinical Management of Friedreich's Ataxia: a Report of Two Cases
Spinal Cord Series and Cases (2018) 4:38 https://doi.org/10.1038/s41394-018-0071-x CASE REPORT Clinical management of Friedreich’s Ataxia: a report of two cases 1,2 3 2 4 4 Yannis Dionyssiotis ● Athina Kapsokoulou ● Anna Danopoulou ● Maria Kokolaki ● Athina Vadalouka Received: 3 March 2018 / Revised: 24 March 2018 / Accepted: 25 March 2018 © International Spinal Cord Society 2018 Abstract Introduction Friedreich’s ataxia (FDRA) is the most common autosomal recessive, early-onset ataxia. FDRA is a pro- gressive neurodegenerative disease that mainly affects the posterior (dorsal) columns of the spinal cord resulting in sensory ataxia. It manifests in initial symptoms of poor coordination and gait disturbance. Case presentation We present two cases, a brother (54 years old) and sister (56 years old), with FDRA that are chronically institutionalized for incomplete quadriplegia without spasticity. Gait and postural ataxia, cerebellar dysarthria, oculomotor dysfunction, musculoskeletal deformities, hearing impairment, hypertrophic cardiomyopathy, and diabetes mellitus are also present. Neurological examination reveals extensor plantar responses and diminished to absent tendon reflexes. Both are wheelchair bound, cannot perform daily tasks and need assistance. Discussion Although there is no cure that can alter the natural course of the disease physiotherapy, management of spasticity 1234567890();,: 1234567890();,: and neuropathic pain, symptomatic treatment of heart failure and diabetes and nursing care can grant the patients quality of life. Introduction remains unclear it seems that it is involved with respiration, iron–sulfur cluster assembly, iron homeostasis, and Friedreich’s ataxia (FRDA), which was first described maintenance of a redox status. Frataxin deficiency results in in 1863, is a hereditary early-onset neurodegenerative dis- mitochondrial iron accumulation and renders neurons ease affecting mainly the central and to a lesser extent prone to oxidative stress and Wallerian degeneration. -
Ataxias of Childhood
ATAXIAS OF CHILDHOOD Jayaprakash A. Gosalakkal, MD, FAAP BACKGROUND– This review examines the causes of ataxias in children. It is a relatively common manifestation of neurological diseases in children. The etiology of ataxia covers a broad range, from infections to rare hereditary metabolic diseases. A systematic approach is required to make the correct diagnosis. REVIEW SUMMARY– The more common causes of ataxias in children are discussed in detail. The importance of recognizing potentially reversible conditions such as vitamin E deficiency and Refsum’s disease is stressed. Recent advances in molecular genetics of some of the chronic ataxias are discussed. The importance of categorizing diseases based on the duration of symptoms and associated signs is discussed. Treatment options are mentioned. CONCLUSION– Ataxia is a common mode of presentation of cerebellar, posterior column, and vestibular disease in children. Awareness of the spectrum of disease entities that cause ataxia in childhood may lead to better diagnosis. KEY WORDS ataxia, cerebellar, childhood (THE NEUROLOGIST 7:300–306, 2001) The term ataxia refers to a special disorder of movement tected early in life in conditions such as Dejerine-Sottas resulting from lesions of the cerebellum or spinocerebellar disease by the ability to correct posture with eyes open but tracts. The disorders, which result in ataxia in children, are not with eyes closed. somewhat different from those in adults. Signs of ataxia in children may be detected as early as the first year of life. This ACUTE AND ACUTE EPISODIC ATAXIA is especially apparent when the child sits down and reaches for objects. Ataxias of childhood can be categorized initially Infectious and Postinfectious by ascertaining the following: 1) whether this is an acute, Acute cerebellar ataxia in children most often follows an acute recurrent, or chronic disorder; 2) if chronic, whether it infectious process. -
CASE STUDY: SENSORY £ Differences from Cerebellar Ataxia ATAXIA § No Tremors § No Central Signs (EOM Normal, No Nystagmus, Etc.) Geoff Mosley, PT, NCS
Sensory Ataxia £ Uncoordinated movement due to lack of peripheral sensation £ Distinguishing characteristics: § Near normal coordination with visual guidance, but severe degradation when eyes are closed § Pseudoathetosis—writhing uncoordinated movement when visual guidance is absent CASE STUDY: SENSORY £ Differences from cerebellar ataxia ATAXIA § No tremors § No central signs (EOM normal, no nystagmus, etc.) Geoff Mosley, PT, NCS Sensory Ataxia Sensory Ataxia £ Causes £ Case #1 § Peripheral neuropathies § 58 yom c multiple myeloma who had a decompressive Diabetic laminectomy for a spinal tumor (C7 to T12) Alcoholism § MMT mostly in 4/5 range except 3/5 range in ankles Metabolic and hip extensors § Dorsal column disease § Sensation was impaired below T4 distally, with Spinal cord injury (trauma, stenosis, spinal stroke) proprioception being especially impaired in both Vitamin deficiency (most commonly B12) ankles and feet (T4 ASIA D) Tabes dorsalis Sensory Ataxia Sensory Ataxia £ Case #1 £ Case #2 § Functionally he required mod-max assist for standing § 59 yom who suffered a STEMI, had syncopal episode and mod assist for transfers and ambulation with a and fell, causing C4-5 spinal cord compression and rolling walker 28’ syringomyelia. He initially transferred to rehab under another PT’s care but had sepsis and so went back to § Gait was ataxic with difficulty stepping accurately and one occasion of right knee buckling main hospital for 1 week for treatment. § MMT: decreased hand strength and dexterity, LEs 4 to § He also c/o back pain 7/10 and had low endurance 4+/5 x right hip and knee ext 4-/5 § He was able to propel a wheelchair 200+’ with UEs without assist § Sensation: Diminished in UEs especially hands, diminished in both LEs especially distally, proprioception included (C5 ASIA D) 1 Sensory Ataxia Sensory Ataxia £ Case #2 £ Case #3 § Tone: 1+ to 2 spasticity in both UEs and LEs c right § 65 yom who had a right parietal lobe CVA. -
An Approach to Late Onset Cerebellar Ataxia
Sixth Annual Intensive Update in Neurology 9/15-16/2016 THE WOBBLY PATIENT: ADULT ONSET ATAXIA Rosalind Chuang, M.D. Movement Disorders Swedish Neuroscience Institute September 15, 2016 Disclosure: none 1 Sixth Annual Intensive Update in Neurology 9/15-16/2016 Objectives • Review algorithm for work up of patients with ataxia • Review common forms of genetic ataxia • Understand which genetic tests to obtain and rationale for genetic testing 2 Sixth Annual Intensive Update in Neurology 9/15-16/2016 What is ataxia? • Dysfunction of the cerebellum or cerebellar pathways • Core symptoms: • Balance & gait • Hand incoordination • Dysarthria • Oculo-motor abnormality 3 Sixth Annual Intensive Update in Neurology 9/15-16/2016 APPROACH TO THE ATAXIC PATIENT A detailed history and exam are always free! 4 Sixth Annual Intensive Update in Neurology 9/15-16/2016 Question to self: “Is it ataxia?” Other causes of imbalance Visual: blindness Vestibular: BPPV, Ménière syndrome Cortical: dementia, medications Musculoskeletal: muscle weakness, hip/knee joint problems Proprioception/Sensory: sensory ataxia 5 Sixth Annual Intensive Update in Neurology 9/15-16/2016 Drugs & Dementia are common cause of falls in elderly 6 Sixth Annual Intensive Update in Neurology 9/15-16/2016 Cerebellar Exam: Scale for the assessment and rating of ataxia (SARA) 1. Gait 2. Stance 3. Sitting 4. Speech 5. Finger chase 6. Nose-finger 7. Fast alternating hand movements 8. Heel shin slide 7 Sixth Annual Intensive Update in Neurology 9/15-16/2016 8 Sixth Annual Intensive Update in Neurology 9/15-16/2016 Questions to ask 1. Age of onset 2. Rate of disease progression 3. -
Acute Or Recurrent Ataxia
Stephen Nelson, MD, PhD, FAAP Section Head, Pediatric Neurology Assoc Prof of Pediatrics, Neurology, Neurosurgery and Psychiatry Tulane University School of Medicine ACUTE ATAXIA IN CHILDREN Disclosures . No financial disclosures . My opinions Based on experience and literature . Images May be copyrighted, from variety of sources Used under Fair Use law for educators Defining Ataxia . From the Greek “a taxis” Lack of order . Disturbance in fine control of posture and movement . Can result from cerebellar, sensory or vestibular problems Defining Ataxia . Not attributable to weakness or involuntary movements: Chorea, dystonia, myoclonus, tremor Distinguish between ataxic and “clumsy” . From impairment of one or both: Spatial pattern of muscle activity Timing of muscle activity Brainstem anatomy Cerebellar function/Ataxia . Vestibulocerebellum (flocculonodular lobe) Balance, reflexive head/eye movements . Spinocerebellum (vermis, paravermis) Posture and limb movements . Cerebrocerebellum Movement planning and motor learning Cerebellar Anatomy (Function) Vestibulocerellum - Archicerebellum . Abnormal gate Abasia - wide based, lurching, staggering Alcohol impairs cerebellum . Titubations – Trunk/head tremor -Vermis lesions . Tandem gait Fall or deviate toward lesion - Hemisphere lesions Vestibulocerellum . Ocular dysmetria Saccades over/undershoot target Jerky saccadic movements during smooth pursuit . Nystagmus with peripheral gaze Slow toward primary, fast toward target Horizontal or vertical May change direction Does