Tics and Pediatric

Movement Disorders

PD Dr. Christos Ganos

Movement Disorders and Body Control Lab
Movement Disorders and Neuromodulation Unit
Charité, University Medicine Berlin

UNIVERSITÄTSMEDIZIN BERLIN
Introduc)on

• Caveats in the approach of pediatric cases

• Benign movement disorders of childhood

• Tic disorders and Tourette syndrome

• Difference between tics and stereotypies

• Less common pediatric Movement Disorders not to miss!

Caveats in the approach of pediatric cases
Special characteristics of pediatric cases
• Hyperkinesias >> hypokinesias

• Complex syndromes/combined MDS >> isolated MDS

(e.g. intellectual disability/neurodevelopmental syndrome +

movement disorder + seizures)

• Could it be epileptic?

• Inborn errors of metabolism are more often encountered/more

relevant than in adults

• Do not miss treatable conditions!

A series of benign disorders of movement in childhood
Tic disorders
Defini&on of &cs

Definition: Tics are brief movements or sounds that resemble

voluntary actions, but appear repetitive and inopportune to
discernable context

Tics are often exaggerations of voluntary actions

(Charcot: Caricatures of actions)
Typical features of tics

- Tics are typically preceded by premonitory urges

- Tics may sometimes be perceived as disturbing and they often are
socially disruptive
- Tics are suggestible and typically wax and wane over time
- Tics can be voluntarily suppressed *
- Tics may be sometimes associated with pain **
Typical features of tics

- Among the entire spectrum of movement disorders, tics have the

widest phenomenological variability

- Any possible movement can be a tic and even some rather

…impossible movements may also be tics

- Tics are classified based

- on duration: clonic vs tonic vs dystonic
- on complexity: simple vs complex
- on output: motor vs phonic
- rarely tics resemble negative motor phenomena: “blocking tics”
Tics as impossible movements
Differential diagnosis of Tics

Ganos, Muenchau and Bhatia, MDCP 2013

Tics: Differential diagnosis
Stereotypies: “Non–goal-directed movement pattern that is repeated continuously for
a period of time in the same form and on multiple occasions, and which is typically
distractible.” (Edwards, Lang and Bhatia, Mov. Dis. 2013)

Trisomy 21 BPAN L-Serine Deficiency

Tics vs Tic-like movements/functional tics

Martino et al. 2021

Tics vs Tic-like movements/functional tics

Martino et al. 2021

Other disorders commonly associated with 2cs

- Tics are most commonly encountered in primary tic disorders (e.g. Tourette
syndrome)

- Tics are common in children aged 6-12 years (ca. 4%)

- Tics as a result of other underlying conditions (secondary tic disorders) are

rather rare (NB: Adult-onset tics; Huntington’s disease, Chorea-acanthocytosis,
fragile X syndrome etc..)

Rohani et al. 2018

The PANS issue

Pediatric Acute Neuropsychiatric Syndroms (PANS)

- Pediatric infection triggered autoimmune neuropsychiatric disorders (PITANDS)

- Pediatric autoimmune neuropsychiatric disorders associated with streptococcal

infections (PANDAS)

- Pediatric acute-onset neuropsychiatric disorders (PANS)

The PANS issue
Manifesta)on of the movement disorder as emergency

Association of Group A Streptococcal infections and primary tics

• 715 children with CTD

• 16 months follow-up
• 405 exacerbations in 43% participants
• No association between GAS and
exacerbations

Martino et al. 2021

Tics in Tourette syndrome
- Definition of Gilles de la Tourette-Syndrome:
2 motor tics + 1 vocal/phonic tic
< 18 y age
> 1 y duration

- ca. 1% of school age children fulfil diagnostic criteria for the presence of TS
- > (ca. 4:1)
- Echo-, Paliphenomena typically present
- Coprophenomena (incl. Coprolalia in less than 1/3 of cases)
- Non-obscene inappropriate behaviours common
- Clinical investigations unrevealing
Tics in Toure7e syndrome

• Despite their high variability tics have a common somatotopic predilection

• Cranial (oculomotor, blinking, facial tics) and tics of the neck/shoulders most
common

Ganos et al. Mov Dis 2015

Comorbidi(es in Toure/e syndrome

- ca. 90% of people with Tourette syndrome also have comorbid

conditions

Hirschtritt et al. JAMA Psy 2015

Self-injurious behavior in Movement Disorders

„Non-accidental behavior resulting in demonstrable, self-inflicted physical injury, without

intent of suicide or sexual arousal. Typically the behavior is repetitive* and persistent.”
(Huisman, S. et al; Neuroscience and Biobehavioral Reviews 2018)

Fischer et al. 2020 JNNP

Self-injurious behavior in Toure3e syndrome

• SIB occurs in more than 1/3 of patients (18-60%)

• Compared to other movement disorders with SIB (e.g. Lesch-Nyhan

syndrome) SIB in TS is more diverse and polytopic

• SIB in TS includes skin picking, scratching, biting (lips, hands), hair pulling,
nail/skin biting, pressure on eyeballs/eye poking, head banging…

• One study (n=98/297) found an association between mild and moderate SIB
with OCD, and severe SIB with impulsivity and rage attacks

Fischer et al. 2020 JNNP, Matthews et al. JNNP 2003, Zhang et al. MDCP 2019
Suicidality in primary tic disorders

• 7736 TD/CTD cases (76% male) in the Swedish National Patient Register
during a 44-year period (1969–2013)

• Matched case-cohort design (1:10)

Clinical course and prognosis

Bloch and Leckmann. J Psychosom Res 2009, Rizzo et al. Brain Dev. 2012
Treatment: Psychoeduca<on

Ganos et al. MDCP 2017

Tourette Syndrome: Treatments

PHARMACOLOGICAL BEHAVIOURAL SURGICAL

TREATMENTS TREATMENTS INTERVENTIONS

BOTULINUM TOXIN HABIT REVERSAL DEEP BRAIN

THERAPY/CBIT STIMULATION

ORAL MEDICATION
EXPOSURE RESPONSE
PREVENTION
CANNABINOIDS
Tourette Syndrome: Treatments

PHARMACOLOGICAL BEHAVIOURAL SURGICAL

TREATMENTS TREATMENTS INTERVENTIONS

BOTULINUM TOXIN HABIT REVERSAL DEEP BRAIN

THERAPY/CBIT STIMULATION

ORAL MEDICATION
EXPOSURE RESPONSE
PREVENTION
CANNABINOIDS
Tourette Syndrome: Treatments
Tourette Syndrome: Treatments

PHARMACOLOGICAL BEHAVIOURAL SURGICAL

TREATMENTS TREATMENTS INTERVENTIONS

BOTULINUM TOXIN HABIT REVERSAL DEEP BRAIN

THERAPY/CBIT STIMULATION

ORAL MEDICATION
EXPOSURE RESPONSE
PREVENTION
CANNABINOIDS
Toure&e Syndrome: Treatments

PHARMACOLOGICAL BEHAVIOURAL SURGICAL

TREATMENTS TREATMENTS INTERVENTIONS

BOTULINUM TOXIN HABIT REVERSAL DEEP BRAIN

THERAPY/CBIT STIMULATION

ORAL MEDICATION
EXPOSURE RESPONSE
PREVENTION
CANNABINOIDS
Tourette-Syndrom: Deep Brain Stimulation

• Points that remain unclear: patient selection, optimal target area, infections, long-
term efficacy?

Mar$nez-Ramirez et al. JAMA Neurology 2018

Switching gears…

IES
L OG
ET IO
D ER
S OR
T D I
M EN ! !
V E I SS !
M O O M
T T
NO

Mar$nez-Ramirez et al. JAMA Neurology 2018

Movement Disorder E/ologies not to miss!
Late Treated Phenylketonuria

• Common metabolic disorder

• AR, mutation in PAH gene (phenylalanin hydroxylase, chr.12q)

• ≈ 1:5500 newborns (Germany)

• Screening in Germany (also DDR) since 1970

Ph.-pyruvate
Ph.-acetate TOXIC Ph.-ethylamine

Excessive Phenylalanine-levels block

transporter for neutral amino acids over BBB
Not to miss: Dopamine responsive dystonia

• Lower Limb dystonia with diurnal fluctuations

• Excellent response to Levodopa
Not to miss: …and others…
Not to miss: …and others…
Not to miss: Niemann Pick Type C
Not to miss: Wilson’s Disease
Not to miss…many more
Thank you