TOURETTES SYNDROME

TOURETTES SYNDROME
Gilles de la Tourette Syndrome (TS) is one of a number of tic disorders No biological test Evolve in childhood Standard diagnostic criteria used Impairment defines the condition Diagnosis and Treatment take time

ANATOMY OF TOURETTE

ETIOLOGY
The cause of Tourette Syndrome is unknown. The basic defect is thought to be a biochemical abnormality in the basal ganglia of the brain.

TRANSMISSION
Genetically transmitted by autosomal dominant gene Patient has 50% chance of passing the gene to children. However, that genetic predisposition may express itself as TS, as a milder tic disorder or as obsessive compulsive symptom with no tics at all In some cases TS may not be inherited and are identified as Sporadic TS. The cause in these instances is unknown

DIAGNOSTIC CRITERIA

Onset before age 18 Multiple motor tics One or more vocal tics Tics evolve in a progressive pattern Symptoms wax and wane* Duration longer than one year Absence of precipitating illness Observation of tics by knowledgeable person

*Relapsing Remitting MS. RRMS is identified by distinct periods of disease activity (relapses) followed by longer periods of disease inactivity (remission)

OTHER TERMS

Chronic Motor or Vocal Tic Disorder Transient Tic Disorder Terms that may be used by doctors because the duration of the tics is less than one year

TOURETTES SYNDROME Affects BOYS 3 - 4:1 more than GIRLS Involuntary with limited capacity to suppress Mean age of onset for tics 6 7 years Affects 2% of the general population- a conservative estimate since it is an under diagnosed condition

SYMPTOMS

MOTOR TICS
Simple
Eye blinking Facial Grimacing Shoulder shrugging Head jerking Arm thrusting Nose twitching Mouth opening Eye rolling

Complex
Touching objects Touching or Hitting self/others Biting lips or arms Scratching persistently Twirling Foot tapping/dragging Jumping Hopping

SYMPTOMS

VOCAL TICS Simple

Throat clearing Sniffing Grunting Humming Whistling Spitting Squealing Clenching teeth

Complex
Stuttering Echolalia - Repeating of another s words Palallia - Repeating one s own words Copralalia - Speaking obscene word/phrases

LEARNING DISABILITIES

Writing Disorders Reading Comprehension Disorders Math Disorders Visual-Motor Integration is almost always a problem Processing Speed and Efficiency Difficulties

Common NANDA nursing care plans Diagnosis:

Risk for self-directed or other-directed violence Impaired social interaction Low self-esteem Goal: Minimize impairment Maximize adaptive skills Most important in planning: Encourage self-esteem Prevent depression

TREATMENT FOR TS Psychological Counseling Behavioral Therapy Medications Alternative Therapies Understanding and support from peers and adults

MEDICATIONS
Drugs such as pimozide (Orap) and clonidine (Catapres) are used to control tics.

NURSING INTERVENTIONS

Stress increases tics - Teach coping skills to handle stress; may need to avoid competition. Intensive involvement in enjoyable activities (sports, music) decreases tics and stress and calms. Waxing and waning of symptoms of comorbidities and tics Explain to parents, peers, teachers that student has very limited control and that expression of tics and other symptoms are involuntary as well as ever-changing and coming and going

NURSING INTERVENTIONS

Cognitive dulling, lethargy, seeming lack of interest, decrease in coordination - Could be due to medication and/or depression. If worsening or severe, Inform the Physician for reevaluation; Infrom Parents to allow extra time and attention for tutoring, studying, and testing. Short temper and argumentative - Provide opportunity for physical movement; encourage relaxation and body control techniques as well as movement education to increase body control. Provide explanations to parents and peers.

ADHD
(ATTENTION DEFICIT HYPERACTIVITY DISORDER)

ADHD
Arise in early childhood. Onset before age seven. Long lasting and evident for at least six months Affects 3-5% of all school aged children. 3:1 boys than girls 9:1 in clinical settings

ETIOLOGY

SYMPTOMS
Primarily Inattentive Type of Symptoms: Fails to give close attention to details or makes careless mistakes. Has difficulty sustaining attention. Does not appear to listen. Struggles to follow through on instructions. Has difficulty with organization. Avoids or dislikes tasks requiring sustained mental effort. Is easily distracted. Is forgetful in daily activities.

SYMPTOMS
Primarily Hyperactive/Impulsive Type of Symptoms: Fidgets with hands or feet or squirms in chair. Has difficulty remaining seated. Runs around or climbs excessively. Has difficulty engaging in activities quietly. Acts as if driven by a motor. Talks excessively. Blurts out answers before questions have been completed. Has difficulty waiting or taking turns. Interrupts or intrudes upon others.

TREATMENT
There is no "cure" for ADHD, however, many treatment approaches may alleviate or significantly decrease ADHD symptoms. As a result, improvements are evident in school/work performance, relationships with others improve, and self esteem increases.

PSYCHOPHARMACOLOGY Stimulants
Methylphenidate (Ritalin) Sustained release (RitalinSR Concerta Metadate-CD Dosage (mg/day) 10-60 in 3-4 divided doses 20-60 in the morning Nursing Considerations Monitor for appetite suppression or growth delays. Give regular tablets after meals. Alert client that full drug effect takes 2 days. Wear patch for 9 hours effect lasts 3 hours after removal. Monitor for insomia.

Transdermal patch (Daytrana) Dextroamphetamine (Dexedrine) Sustained release (Dexerine-SR)

15

5-40 in 2-3 divided doses

10-30 in the morning

Give last dose early afternoon. Monitor for appetite suppresion. Alert client that full drug effect takes 2 days.

PSYCHOPHARMACOLOGY Stimulants
Amphetamine (Adderall) Sustained Release (Adderall-XR) Pemoline (Cylert) Dosage (mg/day) 5-40 in 2-3 divided doses 10-30 in the morning Nursing Considerations Monitor for insomia.

37.5-112.5 in the morning

Monitor for elevated liver function tests and appetite suppression Alert client that full drug effect takes 2 days. Nursing Considerations Give with food. Monitor appetite for suppression. Use calorie free beverages to relieve dry mouth Monitor for elevated liver function tests

Antidepressant (SNRI)
Antomoxetine (Strattera)

Dosage (mg/day) 1.2 mg/kg/day in 1 or 2 divided doses (children <70 kg) 40-80 in 1 or 2 divided doses (children >70 kg and adults)

COMMON NURSING DIAGNOSIS

Risk for self directed or other directed violence Defensive coping Impaired social interaction Ineffective coping Low self esteem Noncompliance Anxiety (moderate to severe) Compromised family coping Imbalanced nutrition: Less than body requirements Ineffective family therapeutic regimen management Interrupted family processes Risk for impaired parenting

NURSING INTERVENTIONS FOR ADHD

Set realistic expectations and limits because the patient with attention deficit hyperactivity disorder is easily frustrated Always remain calm and consistent with the child. Keep all your instructions to the child short and simple. Provide praise and rewards whenever possible. Provide the patient with diversional activities suited to his short attention span. Help the parents and other family members develop planning and organizing systems to help them cope more effectively with the child's short attention span.

NURSING INTERVENTIONS

Risk for self directed or other-directed violence

Observe clients behavior frequently. Observe for suicidal behaviors: Verbal statements, such as statement going to kill myself Determine suicidal intent and available means. Ask how where and when you plan to kill yourself Obtain contract from client not to harm self and agreeing to seek out staff when ideation occurs. Help client to recognize when anger occurs and to accept those feelings Act as a role model for appropriate expression of angry feelings. Give positive reinforcement.

NURSING INTERVENTIONS

Defensive coping
Encourage client to recognize and verbalize feelings of inadequacy and need for acceptance from others and to recognize how these feelings provoke defensive behaviors Provide immediate, fact, nonthreatening feedback for unacceptable behaviors Help client identify situations that provoke defensiveness Practice with role play for appropriate responses Give positive feedback for acceptable behaviors Evaluate and discuss with client the effectiveness of the new behaviors and any modifications for improvement

NURSING INTERVENTIONS

Impaired social interaction

Develop trust relationship Give to the clients constructive criticism and positive reinforcement for clients efforts Give Positive feedback to client Provide group situations for client

NURSING INTERVENTIONS

Ineffective coping Provide safe environment for continuous large muscle movement, If client is hyperactive Provide large motoric activities Do not debate, argue, rationalize, or bargain with the client. Explore with client and discus alternative ways of handling frustration that would be most suited for client

NURSING INTERVENTIONS

Anxiety
Establish a trusting relationship Maintain an atmosphere of calmness Offer support during times of elevated anxiety, Use of touch is comforting for some clients When anxiety diminishes, help client to recognize specific events that preceded onset of anxiety. Provide help to client to recognize signs of escalating anxiety On escalating anxiety provide tranquilizing medication, as ordered