TOURETTE SYNDROME Presented by: APS Healthcare Southwestern Health Care Quality Unit (HCQU) 11-17-03 Disclaimer Information or education provided by the HCQU is not intended to replace information by the consumer’s primary care physician or replace any existing facility policy. Certificates for training hours will only be awarded to those who attend a training in its entirety. Attendees are responsible for submitting paperwork to their respective agencies." Objectives The Participant will: Be able to define Syndrome Be able to define a Sign versus a Symptom Define Tourette Syndrome List & Define known Causes of Tourettes Identify Five known Signs of Tourettes Identify Treatment Strategies What is a Syndrome? A set of signs and symptoms which when studied together lead to the diagnosis of medical, behavioral or mental health disease. Not all Signs & Symptoms when studied together mean a certain disease is present. What is a Sign? An indication of the existence of something; any objective evidence of a disease; such as is observable by those charged for a Consumer’s care. Those observable changes which are measured through testing, and quantified through time. What is a Symptom? Any sensation of change within ones own body which is identified by a Consumer. These are not those items which are noted by outside observers but rather only those things which are identified by the Consumer. Tourette Syndrome Gilles de la Tourette A Syndrome Facial & Vocal Tics Onset in Childhood Progressing to generalized jerking movements in any part of the body, with echolalia, coprolalia. Once thought to be terminal now responds well to prescribed medications. DSM IV-TR Criteria – 307.23 This neuropsychiatric syndrome of uncertain cause, develops in latency or early adolescence with the onset of one or more poorly controlled symptoms, including head or extremity tics, eye blinks, and the spasmodic production of coughs or grunts which occasionally can include verbal obscenities (coprolalia). Often it is severe and life long. DSMIV-TR Criteria - II This syndrome occurs with increased incidence in families, indicating a possible genetic component, and is associated with obsessive compulsive disorder and with hyperactivity and learning disorders in family members. Symptoms are worsened by stress. Diagnostic criteria Medical Study Complete Medical & Behavioral History Age at which first identified Observation of Tic’s Scales are available to rate Tic severity and frequency Possible Etiology Genetic Disorder Environment Exposure to Drugs or other Toxins in early development, often prior to birth Dopamine, Nor-Epinephrine, and Serotonin in dysregulation Definition of a “Tic” Involuntary movement of one or more muscle groups. Most of the time they are meaningless. Some think that the complex tics are purposeful. Categories of Tics Simple Motor Vocal Complex Motor Vocal What are Motor Tics? Eye rolling Eye blinking Facial grimacing Licking Lip smacking Nose twitching Clapping Foot tapping Shrugging Hair tossing Stomping Shaking the head Foot dragging Facial grimacing Squinting Arm squeezing What are Vocal Tics? Clearing the throat Sniffing Shouting Belching Snorting Sucking Honking Gasping Tongue clicking Yelping Moaning Unusual noises Hissing Screaming Gurgling Hiccupping Examples of Complex,Motor Tics Hopping Banging Jumping Kicking Punching Pinching Kissing Copropraxia Echopraxia Skipping Scratching Toe walking Throwing things Tearing things Smelling things Shivering Examples of Complex,Vocal Tics Animal sounds Laughing Palilalia Echolalia Coprolalia Stuttering Repeating Words Phrases Parts of words Spitting Talking to self Tremors & Tourettes Unrelated movements that share one major characteristic, oscillation of a limb or body parts. Physiologic Tremor is normally not observable. Only with increased stress, anxiety or stimulants. Treatment involves removal of medications which over stimulate, and if needed the addition of a Beta-Blocker like Propranalol. Treatment Strategies Offer testing and other activities in separate location with no time limits. Educate other staff and Consumers. Provide a “Safe Area” where a Consumer may go and calm down, release tics or obsessions. Give the Consumer frequent breaks to release tics in a less embarrassing environment. If tics are inappropriate, such as spitting, swearing touching people, it may be necessary to brainstorm solutions. E.g. spitting into a tissue regularly. Motor/Visual Impairment Use of a word processor is helpful Occupational Therapy Sensory Integration Shorten required tasks required Never penalize the Consumer for tic which impairs ability to complete assigned tasks. Obsessive/Compulsive Symptoms Obsessions & Compulsions can take so many forms that it is difficult to give a few pat answers to the problem. First assess the nature of the obsessions and then brainstorm with the treatment team effective interventions. Irritability & Poor Coping Many Consumers with TS become easily frustrated, become over stimulated, and feel over whelming anxiety. Crowds, Cafeteria, Workshops, Living Quarters tend to cause increased anxiety. Lack of Structure leads to increased tics Poor transitioning ability. Sensory defensiveness, which leads to quick over loading and then being easily “set-off” How to Help ? Educate the Staff and Provide Structure for the Consumer. Educate the Consumer in ways to remove her/himself from an escalating and frustrating situation. Some Examples: Permit the Consumer to leave the area ahead of the group to avoid crowds. Permit the Consumer to eat alone and at a different time than the rest of the group. ADHD & Tourettes Preferential Seating at the Workshop Provide a quiet place to complete tasks. Consider a head set with music to block out distractions. Permit freedom of movement Establish communication signs that a Consumer may utilize and recognize as a sign of needing to stay on task. Use color coded cards that identify tasks for the Consumer. Lets tie this together Tourette Syndrome is in fact a group of neuropsychiatric disorders which resemble other diseases. Treatment should encompass all areas identified by the treatment team. The Consumer with Tourettes does not want to be out of control. This is not bad behavior, it is a disease. Medications The treatment of TS is complex. Pediatricians, Internists, and Neurologists with Psychiatrist may be required. Medications have side effects and staff and/or care givers require formal education regarding their use. Medications - II Prolixin Haloperidol Pimozide Olanzapine Some Consumers may be intolerant to the side effects of these drugs. Acute Dystonia, Akathisia, daytime sedation and Task Phobias may occur. Recent Research Risperidone may be effective for tics and has a lower side effect profile. Clonidine Clonazepam Nicotine – the patch These so called a-typical drugs have a much more tolerable side effect profile. What to Do? Observe Analyze Medical Analysis & Evaluation Psychiatric Analysis & Evaluation Neurologic Analysis & Evaluation Environmental Accommodations Comprehensive Treatment Plan - Must include Medical & Behavioral Facets REFERENCES http://samed.com http://apa.org Olney RK, Aminoff MJ: Weakness, Abnormal Movements, and Imbalance, Chap. 21 p. 107 http://www.nimh.gov http://www.who.org Harrisons Companion Textbook of Internal Medicine, Fauci A.S., (et al.). Chap. 1 p. 57 THANK YOU For further information regarding this or any other behavioral or physical health topic please visit our website @ http://hcqu.apshealthcare.com