Tourettes

Question 1

Criteria

Answer 1

The Tourette Syndrome Classification Study Group criteria for a definite diagnosis of TS are as follows [73]:

●Both multiple motor tics and one or more phonic tics must be present at some time during the illness, although not necessarily concurrently

●Tics must occur many times a day, nearly every day, or intermittently throughout a period of more than one year

●Anatomical location, number, frequency, type, complexity, or severity of tics must change over time

●Onset of tics before the age of 21 years (the DSM-5 criteria [74] require onset of tics before age 18 years)

●Involuntary movements and noises must not be explained by another medical condition (or by the physiological effects of substances as per the DSM-5 [74])

●Motor tics, phonic tics, or both must be witnessed by a reliable examiner at some point during the illness or be recorded by videotape or cinematography
DSM-5 diagnostic criteria[4]
Note: A tic is a sudden, rapid, recurrent, non-rhythmic motor movement or vocalisation.

Both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently.
The tics may wax and wane in frequency, but have persisted for more than 1 year since first tic onset.
Onset is before age 18 years.
The disturbance is not attributable to the physiological effect of a substance (e.g., cocaine) or another medical condition (e.g., Huntington’s disease, post-viral encephalitis).

Question 2

Pathophysiology

Answer 2

Largely unknown
Genetic component

DIsinhibition of synaptic transmission n the CSTC circuitry
Abnorally active striatal neurons leading to inhibition of the GPi or substantial nigra pars compact neurons-> disinhibition of thalamocortical circuits that cause hyperkinetic states

Question 3

Most common co-morbid conditions

Answer 3

OCD and ADHD 60-70%

Question 4

Important physical risks to consider

Answer 4

Self injurious behaviours

Cervical myelopathy- neck extending tics, retinal detachments and self laceration

Question 5

Approach to diagnosis

Answer 5

1. history
   Onset- usually in childhood, waxing and waning
   worsen late in childhood before puberty
   day-day exacerbating factors-> meds (stimulants, caffeine, OTC cold/flu), fatigue boredom, stressors.
   Define the tics- differentiate from other things such as allergies/cough variant asthma
   Simple vs complex tics
   premonitory sensations or “urges”
   feeling “compulsive” tic repeated until it feels “just right”-> relief after performing
   School performance and behaviour-> may indicate comorbid ADHD/OCD
   review of ritualistic behaviour or compulsive habits, mood symptoms, and non-OCD anxiety such as phobias or separation anxiety
   FHx tics, tourettes, OCD, affective, ADHD, substances
   *premontory urge for tics scale
2. Examination
   allow tics to emerge
   Asking the patient to suppress his or her tics for 30 to 60 seconds can induce premonitory sensations, which can aid in diagnosis.
   any compulsive behaviours- touching, tapping, drumming
   observe parents/siblings for any tics/compulsive behaviurs
   Generally normal neuro exam
   If neuro deficit- MRI
   Suggestion of seizure- EEG

Question 6

Differentials

Answer 6

Provisional tic disorder
Persistent/chronic motor or vocal tic disorder
Stereotypies
Akathisia
OCD
PANDAS-> Positive anti-streptolysin O antibodies (peak at 3 to 6 weeks) and antiDNAase B antibodies (peak at 6 to 8 weeks).
PANS-> A syndrome with primary diagnostic criteria of acute-onset OCD, with secondary features of anxiety, emotional lability, irritability, aggression, behavioural regression, deterioration in school performance, sensory or motor abnormalities, and somatic signs and symptoms such as loss of handwriting skills or frequent urination
Myoclonus
Wilsons->Increased serum copper levels and low serum ceruloplasmin levels.
MRI with symmetrical T2 hyper-intensities in the bilateral basal ganglia and mesencephalon with sparing of the red nuclei. Kaiser-Fleischer rings, parkinsonism, slow high-amplitude proximal tremor.
Huntingtons
Sydenhas chorea
Allergic rhinitis
COnjunctivitis
Cough-variant asthma

Question 7

Management approach

Answer 7

Multidisciplinary
Multimodal
Optimise patient’s QOL and support positive developmental trajectory
1. Educate family, clinicians, teachers, and peers regarding the symptoms and course of the disorder in order to reduce any associated stigma and distress.
2. establish whether psychiatric comorbid disorders are present, and to what degree they may be impacting the child’s functional capacity at home, at school, and with peers.
3. Referral to CAM is comorbid ADHD, OCD, mood or non-OCD anxiety disorder present. Treating comorbidities may reduce tics
4. Regular monitoring - ages 6 to 15, in which tics are most likely to be prominent
5. Treatment indicated if causing significant distress or functional impairment
6. comprehensive behavioural interventions for tics [CBIT], which includes habit-reversal therapy) are considered first-line intervention for children with mild to moderate tics. Medication should be recommended only when behavioural intervention has not been effective or is not available
7. If possible, avoid major life stressors or prepare for them in advance to prevent tic exacerbations.
Have a structured daily routine that includes vigorous physical activity and good sleep hygiene.
8. the patient and family should obtain progress reports from school to facilitate the assessment of academic progress and treatment.
9. Because the natural history of TS and other tic disorders is to wax and wane, it is recommended that medications be tapered during periods of time in which the patient is experiencing fewer symptoms. Often this can be performed regularly and at times of less stress (such as during summer break or on long holidays or vacations).
10. Family education through support and advocacy groups

Question 8

Habit reversal therapy

Answer 8

1. Awareness training
   - work to notice when perofmring behaviour
2. Competing response training- different behaviour to replace the unwanted one
3. Motivation and compliance- list of problems, praise and encouragement
4. Relaxation training
5. Generalisation training

Question 9

Pharmacotherapy

Answer 9

First-line pharmacotherapy for mild to moderate tics->alpha-2 agonist (e.g., clonidine and guanfacine).
guanfacine has a slightly more favourable side-effect profile, in that it may be less likely to cause drowsiness or sedation.

A benzodiazepine (e.g., clonazepam) may also be used in adolescents or young adults. Clonazepam can be helpful when there is a significant anxiety component to the patient’s symptoms.

Mod-severe-> neuroleptics: haloperidol, aripiprazole, risperidone, ziprasidone

If all fail- botox
Topiramate
Tetrabenazine

Question 10

Treatment with comorbid ADHD- ?worse tics

Answer 10

Cochrane review 2018
Methylphenidate, clonidine, guanfacine, desipramine, and atomoxetine appear to reduce ADHD symptoms in children with tics though the quality of the available evidence was low to very low. Although stimulants have not been shown to worsen tics in most people with tic disorders, they may, nonetheless, exacerbate tics in individual cases. In these instances, treatment with alpha agonists or atomoxetine may be an alternative. Although there is evidence that desipramine may improve tics and ADHD in children, safety concerns will likely continue to limit its use in this population.
One meta-analysis that examined pharmacotherapy for children with ADHD and tic disorders reported that methylphenidate, alpha-2 agonists, desipramine, and atomoxetine were effective in improving ADHD symptoms in children with comorbid tics. Alpha-2 agonists and atomoxetine significantly improved comorbid tic symptoms, and although supra-therapeutic doses of dexamfetamine reportedly worsened tics, therapeutic doses of dexamfetamine and methylphenidate did not
Bloch MH, Panza KE, Landeros-Weisenberger A, et al. Meta-analysis: treatment of attention-deficit/hyperactivity disorder in children with comorbid tic disorders. J Am Acad Child Adolesc Psychiatry. 2009

Question 11

Treatment with comorbid OCD

Answer 11

CBT, exposure and response prevention

SSRI or clomipramine if unsuccessful

Question 12

Patient information about TS

Answer 12

When someone has Tourette’s syndrome they have what are called tics. Tics are repeated
movements or sounds that they can’t help making. For example, they might squint or make
squeaking noises.
Not every child with tics has Tourette’s syndrome. Plenty of children have mild tics and
repetitive habits that come and go and don’t cause them any problems. Tourette’s is more
severe and longer term.
The tics with Tourette’s follow a pattern:
• They start with a strong urge to perform the tic.
• The person then performs the tic.
• The person might also need to repeat the tic until it feels ‘just right’.
• There is then a brief sense of relief before the person feels the urge to repeat the tic.
Performing these tics can be distressing and exhausting for the person with Tourette’s and
for people close to them.
Tourette’s affects the body’s nervous system. We don’t know everything about what causes
it. But it seems to be largely inherited. Other things we know about Tourette’s include:
• Tourette’s is much more common in boys than in girls.
• Tourette’s starts in early childhood and usually peaks at about 10 years old.
• The symptoms usually become gradually less severe as young people reach adulthood.
• By early adulthood, only about 20 in 100 people with Tourette’s will still have tics that
cause them any problems.
Tourette’s often happens alongside other mental-health conditions. The most common ones
are obsessive-compulsive disorder (OCD) and attention-deficit hyperactivity disorder (ADHD).
There are medical treatments that can help with Tourette’s. But some of the best treatments
are simple things you can do to help your child and yourself.
The first, and possibly the most useful thing you can do, is for you and your child to learn as
much as you can about Tourette’s. Understanding the condition can help you and your child
feel more relaxed about it. And learning about it together can help your child feel that you are
dealing with it as a team.
Other things that help children with Tourette’s include:
• having a structured daily routine that includes lots of physical activity
• good sleep hygiene. This means having a regular bedtime, and a regular bedtime routine
that is as calm as possible, and making sure your child is not over-stimulated at bedtime.
For example, using devices with bright screens, such as mobile phones and tablets,
close to bedtime doesn’t help children to relax and sleep. You could try reading with your
child at bedtime instead
• avoiding stressful situations as much as possible. Stress can make tics worse.
Of course, all these things are hard to do all the time - things will happen to disrupt even your
best plans. You can only do your best. But getting into good habits can be a big help.
If your child is diagnosed with Tourette’s you should make sure that his or her family
members, teachers, and friends know about it. The more you can do to help the important
people in your child’s life learn about Tourette’s, the better
Children with Tourette’s usually only need any medical treatment if their tics are causing them
distress.
For children who do need help, the first treatment that is usually recommended is called CBIT
(pronounced ‘see bit’). This stands for Comprehensive Behavioural Interventions for Tics.
It is a form of therapy that, over time, helps people to manage their tics.
There are some medicines that can help some children with Tourette’s. But they are not
usually recommended unless CBIT has been tried without success, or unless it is not
available.
The choice of medication usually depends on whether your child also has ADHD or OCD.
This is because different medicines help with the different symptoms of these conditions.
Children without OCD or ADHD sometimes benefit from medicines that reduce anxiety,
or from medicines called neuroleptics. Neuroleptics are usually used to help prevent
hallucinations in people with a variety of psychiatric conditions. But they can help reduce
Tourette’s symptoms in some people.
• Children with ADHD are sometimes prescribed medicines that stimulate the body’s
nervous system, which helps them to focus and concentrate.
• Children with OCD can sometimes benefit from antidepressants. These drugs are
usually used to treat depression, but they can help reduce the symptoms of OCD and the
anxiety it causes.
All these medications can cause side effects in some children. Your doctor should discuss
these with you carefully. Tell your doctor straight away if your child has any side effects from
his or her medications.
Some parents try alternative medicines for children with Tourette’s. But there is no good
evidence that any of them work.
Tourette’s symptoms tend to wax and wane (come and go, or at least get more or less
severe) over time. If your child has a spell with mild symptoms, your doctor might suggest
reducing his or her medication, and using it more when symptoms are worse.
You should regularly check with your child’s school about how he or she is doing - but you
probably know this anyway.
Teenagers with Tourette’s are more likely than other people their age to suffer from
depression. So this is something to look out for and to talk to your doctor about if it affects
your child.
Adults with Tourette’s can face problems in daily life, such as with further education and
employment. And Tourette’s often goes alongside other mental-health issues, such as
depression, obsessive compulsive disorder (OCD), and attention-deficit hyperactivity disorder
(ADHD).
So if you are an adult with Tourette’s, it’s important to try to get the treatment and help you
need.
There are plenty of support groups for people with Tourette’s syndrome. Your doctor might be
able to direct you to one in your area, or you could look online.