Approach to a Patient with Hemiplegia and Monoplegia
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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. -
Myelopathy—Paresis and Paralysis in Cats
Myelopathy—Paresis and Paralysis in Cats (Disorder of the Spinal Cord Leading to Weakness and Paralysis in Cats) Basics OVERVIEW • “Myelopathy”—any disorder or disease affecting the spinal cord; a myelopathy can cause weakness or partial paralysis (known as “paresis”) or complete loss of voluntary movements (known as “paralysis”) • Paresis or paralysis may affect all four limbs (known as “tetraparesis” or “tetraplegia,” respectively), may affect only the rear legs (known as “paraparesis” or “paraplegia,” respectively), the front and rear leg on the same side (known as “hemiparesis” or “hemiplegia,” respectively) or only one limb (known as “monoparesis” or “monoplegia,” respectively) • Paresis and paralysis also can be caused by disorders of the nerves and/or muscles to the legs (known as “peripheral neuromuscular disorders”) • The spine is composed of multiple bones with disks (intervertebral disks) located in between adjacent bones (vertebrae); the disks act as shock absorbers and allow movement of the spine; the vertebrae are named according to their location—cervical vertebrae are located in the neck and are numbered as cervical vertebrae one through seven or C1–C7; thoracic vertebrae are located from the area of the shoulders to the end of the ribs and are numbered as thoracic vertebrae one through thirteen or T1–T13; lumbar vertebrae start at the end of the ribs and continue to the pelvis and are numbered as lumbar vertebrae one through seven or L1–L7; the remaining vertebrae are the sacral and coccygeal (tail) vertebrae • The brain -
Neuromuscular Disorders Neurology in Practice: Series Editors: Robert A
Neuromuscular Disorders neurology in practice: series editors: robert a. gross, department of neurology, university of rochester medical center, rochester, ny, usa jonathan w. mink, department of neurology, university of rochester medical center,rochester, ny, usa Neuromuscular Disorders edited by Rabi N. Tawil, MD Professor of Neurology University of Rochester Medical Center Rochester, NY, USA Shannon Venance, MD, PhD, FRCPCP Associate Professor of Neurology The University of Western Ontario London, Ontario, Canada A John Wiley & Sons, Ltd., Publication This edition fi rst published 2011, ® 2011 by Blackwell Publishing Ltd Blackwell Publishing was acquired by John Wiley & Sons in February 2007. Blackwell’s publishing program has been merged with Wiley’s global Scientifi c, Technical and Medical business to form Wiley-Blackwell. Registered offi ce: John Wiley & Sons Ltd, The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK Editorial offi ces: 9600 Garsington Road, Oxford, OX4 2DQ, UK The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK 111 River Street, Hoboken, NJ 07030-5774, USA For details of our global editorial offi ces, for customer services and for information about how to apply for permission to reuse the copyright material in this book please see our website at www.wiley.com/wiley-blackwell The right of the author to be identifi ed as the author of this work has been asserted in accordance with the UK Copyright, Designs and Patents Act 1988. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the publisher. -
Absence of Neurobehavioral Disturbance in a Focal Lesion of the Left Paracentral Lobule
Behavioural Neurology (1992), 5,189-191 ICASE REPORTI Absence of neurobehavioral disturbance in a focal lesion of the left paracentral lobule T. Imamura and K. Tsuburaya Department of Neurology, Tohoku Kohseinenkin Hospital, Sendai, Japan Correspondence to: T. Imamura, Department of Neurology, Institute of Brain Diseases, Tohoku University School of Medicine, 1-1, Seiryo-machi, Aoba-Ku, Sendai 980, Japan The case of a right-handed woman with an infarcation confined to the left paracentral lobule and sparing the supplementary motor area (SMA) is reported. She presented with a right leg monoplegia and displayed no mutism. The absence of any associ ated neurobehavioral disturbances (mutism, forced grasping, reduced spontaneous arm activity or aphasia raises the possi bility that the left SMA has discrete neurobehavioral functions. Keywords: Medial frontal lobe - Precentral gyrus - Supplementary motor area - Transcortical motor aphasia INTRODUCTION Various kinds of neurobehavioral disturbances associated medial part of the left precentral gyrus, which is adjacent with left medial frontal lesions involving the supplemen to the SMA, to evaluate its possible neurobehavioral tary motor area (SMA) have been reported. Aphasia due to functions. damage of the left medial frontal lobe is characterized by an initial period of mutism followed by a stage of CASE REPORT decreased verbal output and spontaneous initiation with normal articulation (Stuss and Benson, 1986). Forced A 73-year-old right-handed woman with a history of grasping, compulsive manipulation of tools and decreased hypertension and diabetes mellitus suddenly developed a spontaneous limb movements have also been described gait disturbance. On examination, 24 h later, she was alert (Wise, 1984; Feinberg et al., 1992). -
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, -
Is Hirayama a Gq1b Disease?
Neurological Sciences (2019) 40:1743–1747 https://doi.org/10.1007/s10072-019-03758-x LETTER TO THE EDITOR Is Hirayama a Gq1b disease? Sezin AlpaydınBaslo1 & Mücahid Erdoğan1 & Zeynep Ezgi Balçık1 & Oya Öztürk 1 & Dilek Ataklı1 Received: 13 November 2018 /Accepted: 7 February 2019 /Published online: 23 February 2019 # Fondazione Società Italiana di Neurologia 2019 Introduction knowledge, the association between HD and anti-ganglioside antibodies has not been reported before. Hirayama disease (HD) or juvenile muscular atrophy of We herein, present a young male patient who got the diag- distal upper extremity is a rare benign disease of motor nosis of HD, with unilateral complains but bilateral asymmet- neurons that commonly affects the cervical spinal seg- rical hand muscle weakness and atrophy accompanied by bi- ments. It is more prevalent in males and mostly seen in lateral electrophysiology and typical MRI findings. His serum teens and early 20s. Slowly progressive unilateral or was strongly positive (138%) for anti-GQ1b IgG antibody that asymmetrically bilateral weakness of hands and fore- is worth to be mentioned as a notable bystander. arms is typical. Sensory disturbances, autonomic and Informed consent was obtained from the patient included in upper motor neuron signs are extremely rare [1]. As the study. the synonym Bbenign focal amyotrophy^ implies, it reaches a plateauafterafewyears.Electromyographyrevealsasymmetri- cal chronic denervation in C7, C8, and T1 myotomes. Case Preservation of C6 myotomes is remarkable. Supportive MRI findings are anterior shift of posterior dura, enlargement of epi- A 17-year-old male was admitted with a 1 year’shistoryof dural space, and venous congestion under neck flexion. -
Motor Neuron Disease Motor Neuron Disease
Motor Neuron Disease Motor Neuron Disease • Incidence: 2-4 per 100 000 • Onset: usually 50-70 years • Pathology: – Degenerative condition – anterior horn cells and upper motor neurons in spinal cord, resulting in mixed upper and lower motor neuron signs • Cause unknown – 10% familial (SOD-1 mutation) – ? Related to athleticism Presentation • Several variations in onset, but progress to the same endpoint • Motor nerves only affected • May be just UMN or just LMN at onset, but other features will appear over time • Main patterns: – Amyotrophic lateral sclerosis – Bulbar presentaion – Primary lateral sclerosis (UMN onset) – Progressive muscular atrophy (LMN onset) Questions Wasting Classification • Amyotrophic Lateral Sclerosis • Progressive Bulbar Palsy • Progressive Muscular Atrophy • Primary Lateral Sclerosis • Multifocal Motor Neuropathy • Spinal Muscular Atrophy • Kennedy’s Disease • Monomelic Amyotrophy • Brachial Amyotrophic Diplegia El Escorial Criteria for Diagnosis Tongue fasiculations Amyotrophic lateral sclerosis • ‘Typical’ presentation (60%+) • Usually one limb initially – Foot drop – Clumsy weak hand – May complain of cramps • Gradual progression over months • May be some wasting at presentation • Usually fasiculations (often more widespread) • Brisk reflexes, extensor plantars • No sensory signs; MAY occasionally be mild symptoms • Relentless progression, noticable over weeks/ months Bulbar MND • Approximately 30% of cases • Onset with dysarthria, dysphagia • Bulbar and pseudobulbar symptoms • On examination – Dysarthria – -
Hemiballismus: /Etiology and Surgical Treatment by Russell Meyers, Donald B
J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from J. Neurol. Neurosurg. Psychiat., 1950, 13, 115. HEMIBALLISMUS: /ETIOLOGY AND SURGICAL TREATMENT BY RUSSELL MEYERS, DONALD B. SWEENEY, and JESS T. SCHWIDDE From the Division of Neurosurgery, State University of Iowa, College ofMedicine, Iowa City, Iowa Hemiballismus is a relatively uncommon hyper- 1949; Whittier). A few instances are on record in kinesia characterized by vigorous, extensive, and which the disorder has run an extended chronic rapidly executed, non-patterned, seemingly pur- course (Touche, 1901 ; Marcus and Sjogren, 1938), poseless movements involving one side of the body. while in one case reported by Lea-Plaza and Uiberall The movements are almost unceasing during the (1945) the abnormal movements are said to have waking state and, as with other hyperkinesias con- ceased spontaneously after seven weeks. Hemi- sidered to be of extrapyramidal origin, they cease ballismus has also been known to cease following during sleep. the supervention of a haemorrhagic ictus. Clinical Aspects Terminology.-There appears to be among writers on this subject no agreement regarding the precise Cases are on record (Whittier, 1947) in which the Protected by copyright. abnormal movements have been confined to a single features of the clinical phenomena to which the limb (" monoballismus ") or to both limbs of both term hemiballismus may properly be applied. sides (" biballismus ") (Martin and Alcock, 1934; Various authors have credited Kussmaul and Fischer von Santha, 1932). In a majority of recorded (1911) with introducing the term hemiballismus to instances, however, the face, neck, and trunk as well signify the flinging or flipping character of the limb as the limbs appear to have been involved. -
HCC Risk Adjustment Terminology: • HCC • CCV • AHA • RAF Score / Risk Score What Is Risk Adjustment?
HCC Risk Adjustment Terminology: • HCC • CCV • AHA • RAF Score / Risk Score What is Risk Adjustment? Who? What? Why? How? Redistributes Actuarial risk funds from plans Medicare To protect against based on with lower-risk Advantage Plans, adverse selection enrollees’ enrollees to plans Health Insurance and risk selection individual risk with higher-risk Exchanges, scores others enrollees RAF Calculation Demographic Health RAF Information StatusHealth Status Score Risk Score Higher risk scores represent members with a higher disease burden resulting in appropriately higher reimbursement. Lower risk scores represent a healthier population, but may also be due to: • Poor chart documentation • Poor diagnosis coding Why is this important? • Results in appropriate reimbursement • Augments the overall patient evaluation process • Allows us to stratify patients by risk • Metric for considering panel size, productivity, access, and coding education efforts Current Scope: Attributed Downside Plan Gain Share Lives Risk NGACO 24,876 80%/6.5 80%/6.5 91.5%- MCW 3,785 100% 100.65% Healthy 91.5%- 4,163 100% Saver 100.65% MA 16,561 50% TBD Current State: HCP-LAN APM Framework Category 3A: 83% Category 3B: 17% FFS APM HCC Risk Model • Diagnosis codes with RAF values are grouped into Condition Categories. • Diseases within a Condition Category are clinically related and are similar in respect to cost patterns. HCC Description Applicable Diagnoses to HCC Category RAF 111 Chronic Obstructive COPD Emphysema 0.346 Pulmonary Disease Chronic Bronchitis 108 Vascular Disease Atherosclerosis (of native or PVD 0.299 bypass grafts with or without Phlebitis and Thrombophlebitis complications [rest pain, (acute or chronic) claudication, etc]) Embolism of Veins (acute or Aortic Aneurysm chronic) (nonruptured) Diabetic Peripheral Angiopathy Arterial Aneurysm • There are 79 identified Condition Categories in 2018. -
Jemds.Com Case Report
Jemds.com Case Report HIRAYAMA DISEASE Shaik Sulaiman Meeron1, Senthilkumaran Arjunan2, Murugarajan Singaram3 1Associate Professor, Department of Medicine, Chengalpattu Medical College. 2Assistant Professor, Department of Medicine, Chengalpattu Medical College. 3Junior Resident, Department of Medicine, Chengalpattu Medical College. ABSTRACT Hirayama’s disease, also known as Monomelic Amyotrophy (MMA), juvenile non-progressive amyotrophy, Sobue disease. It is rare and benign condition. It is a focal, lower motor neuron type of disorder, which occurs mainly in young males. Age of onset, it is first seen most commonly in people in their second and third decades. Geographically, it is seen most commonly in Asian countries like India and Japan. Cause of this disease is unknown in most cases. MRI of cervical spine in flexion is the investigation of choice, which will reveal the cardinal features of Hirayama disease. CASE REPORT 20 years old male came with the complaints of tremors of both hands more of right hand and weakness and wasting of right hand, which is slowly progressive for past 6 months. Lower limbs had no abnormality with normal deep tendon reflexes. On examination, there was wasting and weakness of hypothenar and interosseous muscles of right hand. MRI showed thinning of cord from C5 to C7 level. Proximal epidural fat and tiny flow voids with anterior migration of the posterior dural layer at C5-7 level on flexion MRI. Based on these features a diagnosis of focal amyotrophy was made. A cervical collar was prescribed and patient is under regular follow-up. CONCLUSION Hirayama disease is a rare self-limiting disease. Early diagnosis is necessary as the use of a simple cervical collar which will prevent neck flexion, has been shown to stop the progression. -
Assessment of Acute Motor Deficit in the Pediatric Emergency Room
J Pediatr (Rio J). 2017;93(s1):26---35 www.jped.com.br REVIEW ARTICLE Assessment of acute motor deficit in the pediatric ଝ emergency room a,∗ b a Marcio Moacyr Vasconcelos , Luciana G.A. Vasconcelos , Adriana Rocha Brito a Universidade Federal Fluminense (UFF), Hospital Universitário Antônio Pedro, Departamento Materno Infantil, Niterói, RJ, Brazil b Associac¸ão Brasileira Beneficente de Reabilitac¸ão (ABBR), Divisão de Pediatria, Rio de Janeiro, RJ, Brazil Received 21 May 2017; accepted 28 May 2017 Available online 27 July 2017 KEYWORDS Abstract Objectives: This review article aimed to present a clinical approach, emphasizing the diagnostic Acute weakness; investigation, to children and adolescents who present in the emergency room with acute-onset Motor deficit; Guillain---Barré muscle weakness. syndrome; Sources: A systematic search was performed in PubMed database during April and May 2017, using the following search terms in various combinations: ‘‘acute,’’ ‘‘weakness,’’ ‘‘motor Transverse myelitis; Child deficit,’’ ‘‘flaccid paralysis,’’ ‘‘child,’’ ‘‘pediatric,’’ and ‘‘emergency’’. The articles chosen for this review were published over the past ten years, from 1997 through 2017. This study assessed the pediatric age range, from 0 to 18 years. Summary of the data: Acute motor deficit is a fairly common presentation in the pedi- atric emergency room. Patients may be categorized as having localized or diffuse motor impairment, and a precise description of clinical features is essential in order to allow a complete differential diagnosis. The two most common causes of acute flaccid paralysis in the pediatric emergency room are Guillain---Barré syndrome and transverse myeli- tis; notwithstanding, other etiologies should be considered, such as acute disseminated encephalomyelitis, infectious myelitis, myasthenia gravis, stroke, alternating hemiplegia of childhood, periodic paralyses, brainstem encephalitis, and functional muscle weakness. -
Case Report Hirayama Disease: a Rare Disease with Unusual Features
Hindawi Publishing Corporation Case Reports in Neurological Medicine Volume 2016, Article ID 5839761, 4 pages http://dx.doi.org/10.1155/2016/5839761 Case Report Hirayama Disease: A Rare Disease with Unusual Features S. Anuradha and Vanlalmalsawmdawngliana Fanai Department of Medicine, Maulana Azad Medical College and Associated Hospitals, New Delhi 110002, India Correspondence should be addressed to S. Anuradha; [email protected] Received 28 August 2016; Accepted 4 December 2016 Academic Editor: Dominic B. Fee Copyright © 2016 S. Anuradha and V. Fanai. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Hirayama disease, also known as monomelic amyotrophy (MMA), is a rare cervical myelopathy that manifests itself as a self- limited, asymmetrical, slowly progressive atrophic weakness of the forearms and hands predominantly in young males. The forward displacement of the posterior dura of the lower cervical dural canal during neck flexion has been postulated to lead to lower cervical cord atrophy with asymmetric flattening. We report a case of Hirayama disease in a 25-year-old Indian man presenting with gradually progressive asymmetrical weakness and wasting of both hands and forearms along with unusual features of autonomic dysfunction and upper motor neuron lesion. 1. Introduction both lower limbs within the next 6 months. The tremors resulted in severe disability in performing activities involving Hirayama disease (HD), a rare neurological condition, is his hands like writing. He also developed excessive sweating a sporadic juvenile muscular atrophy of the distal upper of both palms.