Serotonin Syndrome: Preventing, Recognizing, and Treating It
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Spinal Segmental Myoclonus Masquerading As a Psychogenic Movement Disorder Laxmi Khanna1*, Anuradha Batra1 and Ankita Sharma2
Short Communication iMedPub Journals Journal of Neurology and Neuroscience 2019 www.imedpub.com Vol.10 No.1:282 ISSN 2171-6625 DOI: 10.21767/2171-6625.1000282 Spinal Segmental Myoclonus Masquerading as a Psychogenic Movement Disorder Laxmi Khanna1*, Anuradha Batra1 and Ankita Sharma2 1Department of Neurology and Neurophysiology, Sir Gangaram Hospital, New Delhi, India 2Department of Neurophysiology, Sir Gangaram Hospital, New Delhi, India *Corresponding author: Dr. Laxmi Khanna, Consultant Neurologist and Neurophysiologist, Department of Neurology and Neurophysiology, Sir Gangaram Hospital, New Delhi–110060, India, Tel: 9873558121; E-mail: [email protected] Rec Date: December 26, 2018; Acc Date: January 05, 2019; Pub Date: January 11, 2019 Citation: Khanna L, Batra A, Sharma A (2019) Spinal Segmental Myoclonus Masquerading as a Psychogenic Movement Disorder. J Neurol Neurosci Vol.10 No.1:282. his lower back followed by a painless involuntary rhythmic forward jerking of his legs which lasted for thirty seconds Abstract (Figures 1 and 2). These attacks occurred at rest and before falling asleep. The jerks increased in frequency and intensity Spinal generated movement disorders are uncommon. An curtailing his social life. There was no past history of any elderly gentleman presented with distressing jerks of both trauma, myelitis, demyelination or infections with human lower limbs which caused him much social immunodeficiency virus. embarrassment. He had received psychiatric treatment for these abnormal muscular spasms without relief. He had become depressed and withdrawn when he first presented to our outpatient department. A routine clinical examination followed by a long-term video EEG with simultaneous EMG clinched the diagnosis of a spinal segmental myoclonus. -
Clinical Controversies in Amyotrophic Lateral Sclerosis
Clinical Controversies in Amyotrophic Lateral Sclerosis Authors: Ruaridh Cameron Smail,1,2 Neil Simon2 1. Department of Neurology and Neurophysiology, Royal North Shore Hospital, Sydney, Australia 2. Northern Clinical School, The University of Sydney, Sydney, Australia *Correspondence to [email protected] Disclosure: The authors have declared no conflicts of interest. Received: 26.02.20 Accepted: 28.04.20 Keywords: Amyotrophic lateral sclerosis (ALS), biomarker, clinical phenotype, diagnosis, motor neurone disease (MND), pathology, ultrasound. Citation: EMJ Neurol. 2020;8[1]:80-92. Abstract Amyotrophic lateral sclerosis is a devastating neurodegenerative condition with few effective treatments. Current research is gathering momentum into the underlying pathology of this condition and how components of these pathological mechanisms affect individuals differently, leading to the broad manifestations encountered in clinical practice. We are moving away from considering this condition as merely an anterior horn cell disorder into a framework of a multisystem neurodegenerative condition in which early cortical hyperexcitability is key. The deposition of TAR DNA-binding protein 43 is also a relevant finding given the overlap with frontotemporal dysfunction. New techniques have been developed to provide a more accurate diagnosis, earlier in the disease course. This goes beyond the traditional nerve conduction studies and needle electromyography, to cortical excitability studies using transcranial magnetic stimulation, and the use of ultrasound. These ancillary tests are proposed for consideration of future diagnostic paradigms. As we learn more about this disease, future treatments need to ensure efficacy, safety, and a suitable target population to improve outcomes for these patients. In this time of active research into this condition, this paper highlights some of the areas of controversy to induce discussion surrounding these topics. -
Myoclonus Aspen Summer 2020
Hallett Myoclonus Aspen Summer 2020 Myoclonus (Chapter 20) Aspen 2020 1 Myoclonus: Definition Quick muscle jerks Either irregular or rhythmic, but always simple 2 1 Hallett Myoclonus Aspen Summer 2020 Myoclonus • Spontaneous • Action myoclonus: activated or accentuated by voluntary movement • Reflex myoclonus: activated or accentuated by sensory stimulation 3 Myoclonus • Focal: involving only few adjacent muscles • Generalized: involving most or many of the muscles of the body • Multifocal: involving many muscles, but in different jerks 4 2 Hallett Myoclonus Aspen Summer 2020 Differential diagnosis of myoclonus • Simple tics • Some components of chorea • Tremor • Peripheral disorders – Fasciculation – Myokymia – Hemifacial spasm 9 Classification of Myoclonus Site of Origin • Cortex – Cortical myoclonus, epilepsia partialis continua, cortical tremor • Brainstem – Reticular myoclonus, exaggerated startle, palatal myoclonus • Spinal cord – Segmental, propriospinal • Peripheral – Rare, likely due to secondary CNS changes 10 3 Hallett Myoclonus Aspen Summer 2020 Classification of myoclonus to guide therapy • First consideration: Etiological classification – Is there a metabolic encephalopathy to be treated? Is there a tumor to be removed? Is a drug responsible? • Second consideration: Physiological classification – Can the myoclonus be treated symptomatically even if the underlying condition remains unchanged? 12 Myoclonus: Physiological Classification • Epileptic • Non‐epileptic The basic question to ask is whether the myoclonus is a “fragment -
Motor Neuron Disease (Amyotrophic Lateral Sclerosis) Arising from Longstanding Primary Lateral Sclerosis
74274ournal ofNeurology, Neurosurgery, and Psychiatry 1995;58:742-744 SHORT REPORT J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.58.6.742 on 1 June 1995. Downloaded from Motor neuron disease (amyotrophic lateral sclerosis) arising from longstanding primary lateral sclerosis R P M Bruyn, J H T M Koelman, D Troost,J M B V de Jong Abstract family history was negative and consanguinity Three men were initially diagnosed as was excluded. Examination disclosed slight having primary lateral sclerosis (PLS), weakness of the left thigh muscles, mild spas- but eventually developed amyotrophic ticity of the legs, knee and ankle cloni, and lateral sclerosis (ALS) after 7-5, 9, and at extensor plantars. Blood chemistry, CSF, and least 27 years. Non-familial ALS and EMG were unremarkable. Magnetic reso- PLS might be different manifestations of nance imaging of the cervical spine was nor- a single disease or constitute completely mal and PLS was diagnosed. Micturition distinct entities. The clinical diagnosis of urgency began in 1987. Examination in 1988 PLS predicts a median survival that is showed definite spastic paraparesis and mild four to five times longer than in ALS. proximal weakness of the legs. Sural and tib- ial nerve somatosensory evoked potentials (J Neurol Neurosurg Psychiatry 1995;58:742-744) (SSEPs) were bilaterally absent and delayed respectively. Visual and brainstem auditory evoked potentials (VEPs, BAEPs), and brain Keywords: amyotrophic lateral sclerosis; primary MRI were normal. By 1990, walking had lateral sclerosis become troublesome and dysarthria was pre- sent; the calves showed fasciculation and Primary lateral sclerosis (PLS), defined as some atrophy. -
ASAH1 Variant Causing a Mild SMA Phenotype with No Myoclonic Epilepsy: a Clinical, Biochemical and Molecular Study
European Journal of Human Genetics (2016) 24, 1578–1583 & 2016 Macmillan Publishers Limited, part of Springer Nature. All rights reserved 1018-4813/16 www.nature.com/ejhg ARTICLE ASAH1 variant causing a mild SMA phenotype with no myoclonic epilepsy: a clinical, biochemical and molecular study Massimiliano Filosto*,1, Massimo Aureli2, Barbara Castellotti3, Fabrizio Rinaldi1, Domitilla Schiumarini2, Manuela Valsecchi2, Susanna Lualdi4, Raffaella Mazzotti4, Viviana Pensato3, Silvia Rota1, Cinzia Gellera3, Mirella Filocamo4 and Alessandro Padovani1 ASAH1 gene encodes for acid ceramidase that is involved in the degradation of ceramide into sphingosine and free fatty acids within lysosomes. ASAH1 variants cause both the severe and early-onset Farber disease and rare cases of spinal muscular atrophy (SMA) with progressive myoclonic epilepsy (SMA-PME), phenotypically characterized by childhood onset of proximal muscle weakness and atrophy due to spinal motor neuron degeneration followed by occurrence of severe and intractable myoclonic seizures and death in the teenage years. We studied two subjects, a 30-year-old pregnant woman and her 17-year-old sister, affected with a very slowly progressive non-5q SMA since childhood. No history of seizures or myoclonus has been reported and EEG was unremarkable. The molecular study of ASAH1 gene showed the presence of the homozygote nucleotide variation c.124A4G (r.124a4g) that causes the amino acid substitution p.Thr42Ala. Biochemical evaluation of cultured fibroblasts showed both reduction in ceramidase activity and accumulation of ceramide compared with the normal control. This study describes for the first time the association between ASAH1 variants and an adult SMA phenotype with no myoclonic epilepsy nor death in early age, thus expanding the phenotypic spectrum of ASAH1-related SMA. -
Restless Legs Syndrome and Periodic Limb Movements of Sleep
Volume I, Issue 2 Restless Legs Syndrome and Periodic Limb Movements of Sleep Overview Periodic limb movement disorder (PLMD) ; May cause involuntary and restless leg syndrome (RLS) are jerking of the limbs distinct disorders, but often occur during sleep and simultaneously. Both PLMD and RLS are sometimes during also called (nocturnal) myoclonus, which wakefulness describes frequent or involuntary muscle spasms. Periodic limb movement was If you do have restless legs formally described first in the 1950s, and, syndrome (RLS), you are not alone. Up to Occupy your mind. Keeping your by the 1970s, it was listed as a potential 8% of the US population may have this mind actively engaged may lessen cause of insomnia. In addition to producing neurologic condition. Many people have a your symptoms of RLS. Find an similar symptoms, PLMD and RLS are mild form of the disorder, but RLS severely activity that you enjoy to help you treated similarly. affects the lives of millions of individuals. through those times when your symptoms are particularly What is Restless Legs Syndrome (RLS)? Living with RLS involves developing troublesome. Restless Legs Syndrome is an coping strategies that work for you. Here overwhelming urge to move the legs are some of our favorites. Rise to new levels. You may be more usually caused by uncomfortable or comfortable if you elevate your unpleasant sensations in the legs. It may Talk about RLS. Sharing information desktop or bookstand to a height that appear as a creepy crawly type of sensation about RLS will help your family will allow you to stand while you work or a tingling sensation. -
Part Ii – Neurological Disorders
Part ii – Neurological Disorders CHAPTER 14 MOVEMENT DISORDERS AND MOTOR NEURONE DISEASE Dr William P. Howlett 2012 Kilimanjaro Christian Medical Centre, Moshi, Kilimanjaro, Tanzania BRIC 2012 University of Bergen PO Box 7800 NO-5020 Bergen Norway NEUROLOGY IN AFRICA William Howlett Illustrations: Ellinor Moldeklev Hoff, Department of Photos and Drawings, UiB Cover: Tor Vegard Tobiassen Layout: Christian Bakke, Division of Communication, University of Bergen E JØM RKE IL T M 2 Printed by Bodoni, Bergen, Norway 4 9 1 9 6 Trykksak Copyright © 2012 William Howlett NEUROLOGY IN AFRICA is freely available to download at Bergen Open Research Archive (https://bora.uib.no) www.uib.no/cih/en/resources/neurology-in-africa ISBN 978-82-7453-085-0 Notice/Disclaimer This publication is intended to give accurate information with regard to the subject matter covered. However medical knowledge is constantly changing and information may alter. It is the responsibility of the practitioner to determine the best treatment for the patient and readers are therefore obliged to check and verify information contained within the book. This recommendation is most important with regard to drugs used, their dose, route and duration of administration, indications and contraindications and side effects. The author and the publisher waive any and all liability for damages, injury or death to persons or property incurred, directly or indirectly by this publication. CONTENTS MOVEMENT DISORDERS AND MOTOR NEURONE DISEASE 329 PARKINSON’S DISEASE (PD) � � � � � � � � � � � -
The Serotonin Syndrome
The new england journal of medicine review article current concepts The Serotonin Syndrome Edward W. Boyer, M.D., Ph.D., and Michael Shannon, M.D., M.P.H. From the Division of Medical Toxicology, he serotonin syndrome is a potentially life-threatening ad- Department of Emergency Medicine, verse drug reaction that results from therapeutic drug use, intentional self-poi- University of Massachusetts, Worcester t (E.W.B.); and the Program in Medical Tox- soning, or inadvertent interactions between drugs. Three features of the sero- icology, Division of Emergency Medicine, tonin syndrome are critical to an understanding of the disorder. First, the serotonin Children’s Hospital, Boston (E.W.B., M.S.). syndrome is not an idiopathic drug reaction; it is a predictable consequence of excess Address reprint requests to Dr. Boyer at IC Smith Bldg., Children’s Hospital, 300 serotonergic agonism of central nervous system (CNS) receptors and peripheral sero- 1,2 Longwood Ave., Boston, MA 02115, or at tonergic receptors. Second, excess serotonin produces a spectrum of clinical find- [email protected]. edu. ings.3 Third, clinical manifestations of the serotonin syndrome range from barely per- This article (10.1056/NEJMra041867) was ceptible to lethal. The death of an 18-year-old patient named Libby Zion in New York updated on October 21, 2009 at NEJM.org. City more than 20 years ago, which resulted from coadminstration of meperidine and phenelzine, remains the most widely recognized and dramatic example of this prevent- N Engl J Med 2005;352:1112-20. 4 Copyright © 2005 Massachusetts Medical Society. able condition. -
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. -
The Effects of Reflex Path Length on Clonus Frequency in Spastic Muscles
J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.47.10.1122 on 1 October 1984. Downloaded from Journal ofNeurology, Neurosurgery, and Psychiatry 1984;47:1122-1124 Short report The effects of reflex path length on clonus frequency in spastic muscles ROBERT IANSEK From the Department ofNeurology, Prince Henry's Hospital, Melbourne, Australia SUMMARY Clinical evaluation of clonus in spastic muscles was performed by comparing reflex path length with clonus frequency for different muscles in the same patient. It was found that clonus frequency varied inversely with reflex path length (r = 0-84, p < 0-001). The findings confirm that clonus is generated by a peripheral mechanism of self re-excitation rather than a central spinal pacemaker. Clonus is a well recognised clinical sign, usually Methods guest. Protected by copyright. occurring in spasticity. A normal, nonspastic limb may manifest clonus, but only under certain condi- Twenty-one unselected patients who were seen on the tions.' The repetitive self perpetuating movement is neurology service were used as subjects. Attempts were induced by brisk stretch of the involved muscle made to study patients with clonus in more than one mus- group, although cutaneous sensory inputs can initi- cle. No restriction was placed on the underlying pathologi- cal basis for the spasticity. Neurological examination and ate clonic movements in a susceptible limb.2 The routine nerve conduction studies were performed to underlying mechanism of clonus is poorly under- exclude an underlying neuropathy. Clonus frequency was stood and controversial. Some investigators34 have measured by use of surface electrodes and the raw EMG evidence to support the theory of a self re-excitation was recorded on photosensitive paper. -
Spasticity in the Podiatric Patient
CHAPTER II SPASTICITY IN TI{E PODIATRIC PATIENT Lwke D. Cicchinelli, DPM The management of the podiatric patient presenting with diseases at this level such as polio effect the classic lower spasticity can be both vexing and rewarding. Content with a motor neuron syndrome. seemingly good outcome at 1 year postoperarively must be This lower motor neuron condition exhibits in tempered wit-h an appreciation for the potential conse- general that individual muscles may be affected, atrophy quences of a growing limb and the complex interplay is pronounced as the muscles do not receive normal between muscles, tendons and osseous factors. The 10 year innervation, flaccidiry and hypotonia of affected muscles follow up outcome might be very different and the patient with loss of tendon reflexes, plantar or babinski reflex if may have other as yet unrecognized functional aberrations. present is of the normal or flexor type, fasciculations may So how does one treat rhese patients? Many be present representing the sporadic discharge of questions come to mind. Do you transfer a spastic muscle fibers due to diseased and irritable axons and muscle? Is the neuromuscular lesion static or progressive? electromyograms reveal reduced numbers of motor units You are a podiatrist, what about the suprastructural and fibrillations representing isolated activity of individ- deformity such as spastic hip adductors, whar is their affect ual muscle fibers. Sensorium is intact. No clonus is on the lower limb function? Is the foot reducible or rigid? Present. Diseases of lower motor neurons or peripheral \fill a muscle tendon transfer shift the muscle power rhe nerves show distal limb weaknesses such as foot drop or a wrong way causing a resultant different deformiry? Are the steppage gait. -
Abnormal Movements in Sleep As a Post-Polio Sequelae
Abnormal Movements in Sleep as a Post-Polio Sequelae AMERICAN JOURNAL OF PHYSICAL MEDICINE AND REHABILITATION, 1998; 77: 1-6. Dr. Richard L. Bruno The Post-Polio Institute International Centre for Polio Education ABSTRACT Nearly two-thirds of polio survivors report abnormal movements in sleep (AMS), with 52% reporting that their sleep is disturbed by AMS. Sleep studies were performed in seven polio survivors to objectively document AMS. Two patients demonstrated Generalized Random Myoclonus (GRM), brief contractions and even ballistic movements of the arms and legs, slow repeated grasping movements of the hands, slow flexion of the arms and contraction of the shoulder and pectoral muscles. Two other patients demonstrated Periodic Movements in Sleep (PMS) with muscle contractions and ballistic movements of the legs, two had PMS plus Restless Leg Syndrome (RLS) and one had sleep starts involving only contraction of the arm muscles. AMS occurred in Stage II sleep in all patients, in Stage I in some, and could significantly disturb sleep architecture even though patients were totally unaware of muscle contractions. Poliovirus-induced damage to the spinal cord and brain is presented as a possible cause of AMS. The diagnosis of post-polio fatigue, evaluation AMS and management of AMS using benzodiazepines or dopamimetic agents is described. Abnormal Movements in Sleep as a Post-Polio Sequelae Despite numerous late-onset symptoms reported by polio survivors -- fatigue, muscle weakness, pain, cold intolerance, swallowing and breathing difficulties -- one symptom was totally unexpected: abnormal movements in sleep (AMS). As early as 1984 our post-polio patients were reporting muscle contractions as they fell asleep.