Tourette's Syndrome

Question 1

Tourette’s syndrome (TS)

Answer 1

involves a number of clinical features including verbal and motor tics

Question 2

Tics

Answer 2

most obvious and distinguishable symptoms
brief actions (motor)
vocalisations (verbal)

Question 3

Tics range in complexity

Answer 3

Simple recurrent acts (eye blinking and coughing)
Multiple co-occuring tics or elaborate action sequences (complex tics)

Question 4

Most common misbelief about TS

Answer 4

As seen in TV shows and movies
Blurting out obscentities or curse words
Most people with TS do not excessively or uncontrollably use inappropriate languae

Question 5

Reality about coprolalia

Answer 5

Affects 1 in 10 people with TS
complex tic
difficult to control/suppress
embarassment

Question 6

Simple tics

Answer 6

one muscle group

Question 7

Complex tics

Answer 7

more than one muscle group (several)

Question 8

Tics explained (involuntary urge)

Answer 8

Involuntary
Unwilled
Unchosen
-
Tics follow the experience of an involuntary urge to move, to which the individual responds by voluntarily releasing the tic in order to relieve the urge.

Question 9

Prevalence, stress and comorbidity

Answer 9

Prevalence is about 1%
Affects about 1 in 100
Most find it gets better by late teens/early adulthood
Stress make tics worse, as with OCD
Comorbid with OCD and ADHD

Question 10

First line of treatment for TS

Answer 10

Habit reversal therapy
Two step process
Awareness training and competitive response training

Question 11

Example of HRT

Answer 11

The person says what tic is happening and the therapist gives them a counter response to perform e.g. if they have the urge to life their arm, the therapist tells them to sit on their hand

Question 12

Why is HRT first line?

Answer 12

Psychological therapy because physiological mechanisms of TS not fully understood

Question 13

Pharmacotherapy

Answer 13

Antiepileptics
Neuroleptics

Question 14

Dopamine blockers

Answer 14

Haloperidol
Risperidone
Help control tics
SE: weight gain, involuntary repetitive movements

Question 15

Tetrabenezine

Answer 15

Might be recommended but can cause severe depression

Question 16

Experimental procedures

Answer 16

Transcranial magnetic stimulation
Deep brain stimulation
Invasive
Not as common

Question 17

Mechanisms underpinning TS

Answer 17

Thought to primarily include the BG

Question 18

Prototypical motor control

Answer 18

Signals from the cortex encoding for motor plans enter CSTC circuits via the putamen

Question 19

TS motor control

Answer 19

Dysregulation within the basal ganglia pathways likely compromises inhibitory striatal output
Facilitating direct over indirect pathway activity

Question 20

Consequence of dysreg in TS

Answer 20

Disinhibition of thalamic output to motor cortex predisposes the production of actions that were not signalled via cortico-striatal inputs or had been weakly signalled

Question 21

Postmortem data

Answer 21

Revealed key insights into basis of striatal dysfunction in TS

Indicating 50% reduction in GABAergic interneurons

Similar to HD chorea

Question 22

Findings in TS brain consistent with

Answer 22

altered neural migration during neurodevelopment

Question 23

Animal models

Answer 23

Injection of GABAergic antagonist bucucilline into putamen leads to brief recurrent actions that closely resemble tics

Question 24

Mechanisms dysregulating synaptic communication and architecture have been suggested

Answer 24

Neuroligins
Neurexins
GABAergic
Glutamatergic AMPA receptor signalling

Question 25

As well as disturbances in mitochondrial function

Answer 25

Affecting energy balance within basal ganglia