Tourrette Syndrome Flashcards
What is the full name for Tourette Syndrome? What occurs in this medical disorder?
Tourette syndrome
-Known as Gilles de la Tourette Syndrome
- Chronic neurologic disorder characterized by tics which are involuntary, sudden, rapid, recurrent, non-rhythmic movements or vocalizations
What kind of tics are seen in Tourette syndrome?
Motor tics: Facial grimace, blinking, head jerking, shoulder shrugging, spitting
Vocal tics: grunting, sniffing, clearing throat, repetition of words.
What are complex vocal tics that people with Tourette syndrome also experience?
Some patients also experience Complex vocal tics:
Coprolalia: involuntary swearing (using inappropriate language)
Echoalia; repeating other people’s words
Palilalia: repeating your own words or phrases
What are the causes of Tourette Syndrome? What are possible risk factors? When does it usually occur? What do almost all patients also have with Tourette’s syndrome?
Tourette syndrome
Causes
-Unknown
-Environmental and genetic factors
-Possible risk factors include less than adequate weight gain and maternal use of alcohol or cannabis in the last 2 months pregnancy.
Usually develops in childhood (first year of birth), reaches peak severity in adolescent years, and improves in early Adulthood
Almost all patients have accompanying comorbid psychiatric conditions including OCD, ADHD, depression, anxiety, autism, spectrum disorder, impulse control disorders.
(abnormal Self-Activation or Self-Stimulation in brain result in these behaviors )
How is Tourette syndrome diagnosed? What is the Diagnostic Criteria? How can you distinguish Tourette syndrome from other movement disorders?
Clinical Diagnosis
Diagnostic Criteria:
-Presence of > or equal 2 motor tics and greater than or equal to 1 vocal tic for greater than or equal to 12 months.
-Onset before age 18
Distinguishing Tourette syndrome from other movement disorders
-presence of a premonitory urge, a feeling of inner mounting tension that is temporarily relieved by executing the tic (person knows and they symptoms are occurring)
-Ability to voluntarily suppress the tic partially or temporarily
(have some control over behavior)
Describe the complications of Tourette Syndrome
Complications of Tourette syndrome:
-Reduced quality of life
-Adverse effects on interpersonal and social interactions (hard to have conversations)
-Difficulties in school
-Tic-related physical injuries (banging head)
What kind of management and treatment is used for patients with Tourette Syndrome?
Management and Treatment
-Refer patients to a specialist clinic
-Educate
-Behavioral therapies
-Habit reversal training
-Exposure and response prevention
-CBT
Medications
-First line include alpha-2-adrenergic agonists including clonidine or guanfacine
-Typical antipsychotics (they will block dopamine receptors and calcium; Only considered when other drugs are ineffective)
-Atypical antipsychotics such as Risperidone or apriprazole (block dopamine receptors)
(these are used to reduce motor function, minimize tics)
Explain how Deep Brain stimulation can be sued a treatment for Tourette syndrome. Who can use this treatment? How does it work?
Deep Brain Stimulation (DBS) is an experimental treatment Only for SELECT ADULT patients with severe Refractory disease (not respond to treatment)
-It involves implanting a battery-operate medical device that delivers electrical stimulation to certain targeted areas that control movement
-Most frequently stimulated structures were Centromedian nucleus of Thalamus and GPi of striatum
(the electrodes placed in indirect pathways in brain to inhibit motor behavior)
How is DBS Placement (deep brain stimulation) done?
DBS Placement
-Usually done awake with IV pain medications, local anesthesia and intermittent sedation
(since you need patient to respond to command and slide the electrode in place)
-Placement of small recording microelectrodes occurs
What occruisn during the intraop testing in DBS placement ?
REVIEW
Neurophysiology and Intraop Testing:
Final lead position is determined from information obtained during micro electrode recording and test stimulation
(used to see if have placed electrode in right spot)
intraop testing (monitors all neural pathways in the brain during surgeries)
What is the purpose of 3d printed Stereotactic Frames?
3D printed Stereotactic Frames
-Allow fast, accurate placement of DBS Leads for awake Cases
-Improve patient comfort
What occurs during the initial Postoperative Care for Tourette syndrome?
Initial Postoperative Care
- Usually stay in the hospital one night following Cranial lead placement
-Battery is placed in the outpatient setting under general anesthesia
-Light activity for 2 weeks following the operation
-Activation of the DBS system 1 month after placement of Cranial leads.
Describe the Programming and Maintenance involved in the DBS placement
Programming and maintenance of the system is key
-Initially, the patient has frequent programming visits until they feel that the system is optimized
-Batteries are replaced every 3-5 years
-Rechargeable options are available
What are the Risks of DBS Surgery?
Risks of DBS Surgery
-Brain Hemorrhage: 3%, most are asymptomatic but some can cause weakness or deficits
-Infection: 5%. Usually treated with the removal of the battery and antibiotics
-Slurring of speech in Tremor patients (dysarthria)
-Cognitive difficulties (risk increases with age)
-Balance Problems
