Transcript Tourette*s Disease - Rady Children`s Hospital

CBIT Challenges with Tic
Disorders are
Accompanied by
Psychiatric Diagnoses
Charles Farrow, Ph.D.
Clinical Psychologist
(858) 576-1700 x 3409; (858) 480-1484
E-mail: [email protected]; [email protected]
Tourette’s Demographics

Only 1-3% of all children meet the criteria for TS.

Transient Tic disorders are slightly more common (4-5%).

Boys are more likely to suffer TS than girls (1-8 versus 0.1-4 out of 1000).

Most cases of TS develop between 5-10 years of age.

The peak incidence is 11 years of age.

TS often worsens with puberty.

TS typically diminishes with adulthood.
Comorbidity #1: ADHD

Rates of motor and phonic tics among children with ADHD are around 10%

Rates of ADHD among children with TS are around 40-75%

This high percentage is considered to be secondary to shared pathways from
the frontal cortex to the basal ganglia.

When they co-occur, ADHD symptoms tend to appear first, followed by tics.

While methylphenidate was once considered to make tics worse, empirical
studies debunked this observationally based belief.

Both ADHD and TS are considered to have multifactorial genetic
determinants.

Both disorders probably involve genes that regulate and transport dopamine.
Comorbidity #2: OCD

This comorbidity has emerged over time and has surpassed ADHD in
prevalence and incidence.

Rates of motor and phonic tics among children with OCD are around 30%, and
45% have a family history of tics.

Estimated rates of OCD among children with TS start at 50%.

Some researchers believe the two disorders are caused by the same gene.
Comorbidity #2: OCD continued

When co-ocurring with TS, OCD is more severe behaviorally and cognitively.

OCD in a person with TS tends to be more violent, sexual, and aggressive and
to involve behaviors that require symmetry.

Compulsions and tics share a felt pressure to act until the pressure is
relieved.

The behavioral and descriptive differences between compulsions and tics are
often confusing.

Neurologists consider the two disorders to fit into a broad phenotype of habit
disorders.
Other Comorbidities:

Learning disabilities (e.g., dyslexia, arithmetic disorders, and disorders of
written expression).

Children with TS are more likely to have arithmetic disorders than children
without TS.

Children with TS are more likely to have anxiety and depressive disorders
than children without TS.

If a child with TS has an anxiety disorder, other than OCD, the most common
types are social anxiety and generalized anxiety disorder.
Neuropsychological Correlates

Impairments in fine motor coordination

Visuoperceptual / visuoconstructional deficits

Attention/Executive Function (especially behavioral disinhibition)

Memory issues

Verbal Dysfluency
Neuropsychological Correlates Cont’d.

However, this is all based on studies that included comorbid ADHD and OCD

When TS is unaccompanied by ADHD and OCD, memory issues and verbal
dysfluency disappeared and executive function issues were much less.

When there are neuropsychological correlates, psychiatric symptoms are
often present.
What about PANDAS???

Research in the late 90’s reported that a group of children developed both
tics and OCD symptoms after a streptococcal infection.

These children were described as suffering from Pediatric Autoimmune
Neuropsychiatric Disorders After Streptococcal infection (PANDAS for short).

They were reported to develop tic disorders, emotional regulation issues and
behavioral difficulties after their Strep infections.

There is intense controversy around the relationship of PANDAS to TS (and to
Sydenham’s Chorea).

Studies to show different levels of antibodies and enzyme linked
immunosorbent assay (ELISA) have been inconclusive.

Many researchers question a clear etiological relationship, and they
recommend “carefully controlled, prospective studies”.
Empirically Supported Interventions:
Behavioral Approaches

With roots in habit reversal therapy, CBIT has been shown to be very effective
in TS.

In this model, tics are viewed as operationally-conditioned responses to
premonitory urges.

That is, negative reinforcement occurs when the tic offers relief to the
premonitory urge.

By developing competing responses to the urges, the tics are avoided, the
urge dissipates, and the child gains mastery over weakening urges.
Negative Reinforcement Hypothesis of
Tic Maintenance
Premonitory
Urge
Tic
Relief
Negative Reinforcement
Tics can be maintained by elimination of
premonitory urge. Biological processes underlying
the urge and it’s reduction are not understood.
Case A: Assessment
• Developmental Questionnaire : This 11 year old European
American male client’s history was absent any significant
concerns other than Tics.
•YGTSS: TS was marked by a history of sudden onset when he
was 6 years old, and it has included a host of motor and phonic
tics, which never occur in synchronized bouts. His TS caused a
minimal level of impairment. YGTSS = 36 out of 100.
•PUTS: Medium intensity of his premonitory urges.
•CBCL: Absent any clinically significant or even borderline
clinically significant emotional behavioral concerns. Indeed, it
suggested that he was high functioning overall, and specifically
in terms of his social and academic life and level of activities. His
father's ratings on the scale appeared to be positively biased.
Case A: Tic Repertoire
• Eye blinking
• Squinting
• A quick Turning of the eyes
• Licking the lip
• Scrunching the face
• Tongue to teeth
• Throwing head back
• Shrugging shoulders
• Quickly extending/flexing
arms
• Nail Biting
• Popping knuckles
• Throat Clearing
• Sniffing
• Snorting (~every 15 seconds)
Case A: Severity
•Motor Tics: >5 Multiple discrete Tics that occurred
almost always (bouts of tics are common and not
limited to a single setting). Tics are more forceful than
voluntary actions, but within the range of comparable
actions. Some tics are purposive in appearance and
mimic automatic behaviors. The tics do not interrupt
the flow of behavior.
•Phonic Tics: 2-5 Multiple discrete Tics that occurred
almost always (bouts of tics are common and not
limited to a single setting). Tics are more forceful than
voluntary actions, but within the range of comparable
utterances. Some tics are purposive in appearance
and mimic meaningful utterances. The tics do not
interrupt the flow of speech.
Tic Symptom Hierarchy Tracker
Case A: Functional Progress
• Most annoying tic to client (nail biting) remitted by the 5th
session.
• Most annoying tic to client’s family (snorting) remitted by the
7th session.
• 5 urges a day for eye rolling and 5 urges a day for
sniffing/snorting. No nail biting urges. 1 urge every other day for
head movement (side to side). Client: “they’ve died down a lot,
they’ve gotten shorter, and they’re easier to resist”.
• Client: “Now, no one is going to be asking me why I’m doing
these tics or what I’m doing when I do them”.
• Client’s father: “Our biggest concern was the social
awkwardness, and I feel like that concern has been eliminated”.
• YGTSS = 3 out of 100.
Obsessive Compulsive Cycle
Obsessions
Distress
Repetitive negative
images or impulses
Anxiety, fear,
Disgust or shame
Relief
Compulsions
Distress subsides
temporarily
Repetitive thoughts,
Images or actions
Negative Reinforcement
Empirically Supported Interventions:
Behavioral Approaches

Given the similarity between tics and compulsions, a response prevention
paradigm has been tried.

In this approach, the child is encouraged to suppress the tic in the face of the
premonitory urge, while they simultaneously avoid the habitual response.

In Exposure Response Prevention (ERP), the therapist helps the child develop
a symptom hierarchy, and then the feared or triggering stimulus is gradually
introduced.

This is done in a controlled and continuous way, with success habituation.

Like CBIT, this approach is considered viable, because it interferes with the
stimulus-response sequence.
Case B: Assessment
• Neurodevelopmental Questionnaire : This 15 year old
European American male client’s history was replete with
significant concerns other than Severe Tics. This included
multiple psychiatric hospitalizations for severe OCD, and a
question of a thought disorder (paranoia). Three other therapists
had worked with him, attempting CBIT.
•YGTSS: Recently administered with a maximum score of 100,
and the impairment rating was repeated and this was indeed
severe. TS started abruptly at age 8, and anxiety followed.
YGTSS = 100 out of 100.
•PUTS: Extremely high intensity of his premonitory urges.
•Y-BOCS: Current: 6 obsessions and 7 compulsions.
•BASC-2: Parental questionnaire: His mother’s ratings hyperactivity, (borderline clinical significance) and activities of
daily living (extremely low). His father's ratings - in agreement,
and social withdrawal and somatization (borderline clinical
significance). Self-rating – hyperactivity (agreement), and
atypicality, anxiety and somatization (borderline clinical
significance) and self-reliance (below average).
Case B: Tic Repertoire
• Slamming of head to right
shoulder so forcefully that it
caused bleeding on his
shoulder and a welt on his
head.
•Stomping/Kicking
•Downward elbow slam
•Hitting sideways/downwards.
•“Crane’s Beak”
• Hi Pitched Yelp/Vocalization
Case B: Severity
•Motor Tics: Multiple discrete Tics plus several
orchestrated Tics that occurred always (Tic free
intervals are difficult to identify). Tics are extremely
forceful and call attention/result in injury. Tics are
impossible to camouflage. The tics frequently
interrupt intended behavior.
•Phonic Tics: 2-5 Multiple discrete Tics that occurred
always (Tic free intervals are difficult to identify). Tics
are extremely forceful and call attention to client.
Tics are impossible to camouflage. The tics
interrupt the flow of speech.
Case B: Functional Progress
• October 2015: Tics overpowering, missing school, no longer
going to school by Mid-October
• November 2015: Painful Head/shoulder Slam Tics. No School.
Laying Down.
• December 2015: ER, due to Pain from head/shoulder tic. Still
spotty with medication compliance. Lost hope. No School.
• January 2016: Laying down all day to less tics. Hair loss. No
School. Attended TS Symposium at Rady’s. Met with Dr.
Friedman at Behavior Unit at Rady’s. Met Greg Nunn, Gail
Reiner. Medication adjustment, slowly off abilify to Risperidone
and Sertraline.
• February 2016: Hygiene - Having difficulty bathing, brushing
teeth, washing hair. Began with Psychiatrist Dr. Saxena of UCSD
OCD clinic. Referred to CBIT at DEC.
Tic Symptom Hierarchy Tracker
Tic Symptom Hierarchy Tracker
Case B: Functional Progress
• March 2016: First CBIT appointments at DEC.1st session:
Able to hold tic at bay for 5-7 seconds. By 4th session, not hitting
shoulder so less pain.
• April 2016: Back to School.
• May 2016: A’s
• June 2016: Honor Roll Award, and 10th Grade Graduation.
• Much brighter affect.
Empirically Supported Interventions:
Cognitive Approaches

With children with impulsivity (think ADHD), an efficacious technique is
“Stop, Think and Act”.

In the first step, the child’s quick cognitive tempo is slowed down by
encouraging them to stop, listen and look.

In the next step, they are asked to consider options before proceeding.

Lastly, once a decision is made they are asked to act.

Additionally, children with TS often have reduced self-esteem and lower sense
of self-efficacy, so that having the child reflect on how their negative thinking
about themselves may be erroneous can be quite liberating.

This needs to be done in sync with the success habituation of either CBIT or
ERP, as well as supportive accommodations at home or school.
Empirically Supported Interventions:
Dopamine Antagonists

Pharmacologically, the first-line of defense for TS has been dopamine
antagonists (think Haldol and Pimozide), but these led to serious sedation,
cognitive slowing and extrapyramidal symptoms (think dystonia, akathisia,
parkinsonism, bradykinesia, and tremor, and tardive dyskinesia).

This led to the development of atypical dopamine antagonists (think Risperdal
and Zyprexa), which still run the risk of sedation and weight gain.

Newer drugs (think metoclopramide) are promising (i.e., significantly better
than placebo), with minimal side-effects, largely due to basal ganglia
selectivity.
Selective Serotonin Reuptake Inhibitors
(SSRIs)

These medications (think Celexa, Lexapro, Paxil, Zoloft, etcetera) have been
known to help with OCD.

However, they have been known to exacerbate tics.

So… they are sometimes carefully considered in the case of comorbid TS and
OCD (but not TS alone).
Noradrenergic Agonists

These medications (think Clonidine) have been known to help with tic
reduction.

Moreover, they have been known to help with impulsivity (think ADHD).

From this class of medications has emerged a useful drug, Guanfacine, which
helps with tics and improves the metacognitive index on the Behavior Rating
Inventory of Executive Function (BRIEF).
Stimulants

While this class of medication has helped with ADHD (think Ritalin), their use
with comorbid ADHD and TS is controversial.

Stimulants are generally considered likely to increase or intensify tics.

Moreover, in simple ADHD, there have been concerns that this may bring on
tics.
Selective Norepinephrine Reuptake
Inhibitors.

This type of medication (think Atomoxetine) was investigated for ADHD cooccurrence with TS.

It is considered a possible adjunct to stimulants when ADHD and TS are
present.
CBIT Challenges with Tic
Disorders are
Accompanied by
Psychiatric Diagnoses
Charles Farrow, Ph.D.
Clinical Psychologist
(858) 576-1700 x 3409; (858) 480-1484
E-mail: [email protected]; [email protected]