Movement

disorders in
children

BY: Zienab Mahmoud

Control of Movement
Cerebellum
Control of movement patterns + Extra pyramidal system
Motor learning Basal ganglia
Judge speed, force and direction Thalamus
Coordinator of information Subthalamic nuclei
Receives information from Substantia nigra
Muscle spindles
Red nucleus
Labyrinth, eyes, parietal cortex
Joints Brainstem reticular
Pressure receptors formation

Fluent movement
Start and stop of movement
Functional equilibrium in
EPS

Dopamine

Ach
 Differentiating between
types
SLOW Abn movement FAST

Stereotyped Non-stereotyped
(involuntary)
Dystonias
Athetosis
Not Chorea
Rhythmic Rhythmic

Tics
Tremor Myoclonus
 Involuntary movements

Abnormalities in structural and biochemical

function of the nuclear masses of the
basal ganglia
Not under voluntary control
Patient usually cant stop them
Without apparent purpose
Aggravated by physical, emotional and
mental stress
Many people engage in some degree of habit like
behavior in their lifetime.
Habit disorders now subsumed under the diagnostic term
stereotypic movement disorder, consist of repetitive
nonfunctional motor behaviors that interfere with normal
activities or that result in bodily injury
Fortunately, many childhood habits are benign, are
considered a normal part of development, and do not
meet the criteria for a disorder such habits typically remit
without treatment.
Stereotypies are repetitive, purposeless actions that are most commonly seen
in childhood. They do not have a clear definition, because of the wide range of
classify stereotypies into The following types:

Common stereotypies

These are by far the most frequent type and comprise habits such as nail
biting and bruxism

Complex motor stereotypies These consist of various repetitive limb

movements.

Head nodding This is a separate stereotypy that shares some

characteristics with complex motor stereotypies
Complex motor stereotypies are further subdivided
as follows:
Primary stereotypies These occur in otherwise
developmentally normal children and usually remain
stable or regress.
Secondary stereotypies These occur in conjunction
with a neurologic or behavior disorder, such as
autism, intellectual disability, Tourette syndrome,
and some rare neurodevelopmental syndromes in
older children they may
be associated with schizophrenia, obsessive
compulsive disorder, or early onset
neurodegenerative diseases.
Voluntary repetitive movements that mimic
stereotypies include attention deficit
hyperactivity disorder (ADHD), mannerisms, and
compulsions. ADHD tends to be
characterized by more generalized and restless
actions than stereotypies are.
Mannerisms are rarely continual and accompany a
normal activity. Compulsions comprise repeated
ritualistic movements to relieve anxiety or fear and
may occur with tics and stereotypies.
Some childhood habits remain unnoticed and can persist
if left untreated, even when they interfere with optimal
functioning. Childhood habits can result in negative
social interactions and avoidance by peers and family
members. Some repetitive behaviors can cause
damage. For example, bruxism (teeth grinding) can
result in tooth damage. Occasional hair pulling can result
in hair loss or evolve into a more severe disorder,
trichotillomania.
In most children who are otherwise developing normally,
however, few of these habits result in permanent
physical damage
Diagnostic criteria (DSM5)
The specific DSM5criteria for stereotypic movement disorder
are as follows[1] :
Repetitive, seemingly driven, and apparently purposeless
motor behavior
(eg, hand shaking or waving, body rocking, head banging,
self biting, or hitting ones own body)
The repetitive motor behavior interferes with social,
academic, or other activities and may result in self injury
Onset is in the early developmental period
The repetitive motor behavior is not attributable to the
physiologic effects of a substance or neurologic condition and
cannot be better explained by another neurodevelopmental
or mental disorder (eg, trichotillomania or obsessive
compulsive disorder)
Severity is specified as follows:
Mild Symptoms are easily suppressed by sensory
stimulus or distraction
Moderate Symptoms necessitate explicit protective
measures and behavioral modification
Severe Continuous monitoring and protective
measures are required to prevent serious injury
For common stereotypies.

Thumb sucking is an oral habit that involves mouthing of the thumb. Other fingers
or the hand may also be involved. Nail biting consists of biting on or chewing the
nails of the hand.

Nose picking is the insertion of a finger into a nostril and may involve the removal of
nasal discharge ,Older children and adults are most likely to pick their nose in
private, whereas young children may commonly do this in public view.
Bruxism is the forcible, grinding, clicking, or clenching of teeth.
Nocturnal bruxism occurs during sleep, and the child is usually unaware
of the problem.
Episodes are typically brief, lasting 89 seconds, with audible grinding
noises.
Diurnal (daytime) bruxism is primarily associated with clenching of the
teeth and generally does not produce audible noises. Diurnal bruxism is
related to other oral
habits, such as nail biting or lip chewing.
Complex motor stereotypies include the following:
Flapping
Waving
Opening and closing of a fist
Finger wiggling
Wrist flexion and extension
Atypical gazing at objects or fingers (secondary
stereotypies described in
children with autism)

The characteristics of head nodding are slightly

different from those of complex
motor stereotypies of the limbs.
A breathholding spell is a paroxysmal event in which a child
stops breathing at end expiration after crying, typically
because of pain or anger.
Breathholding spells may be divided into the following 3
categories:
Simple breathholding spells These result when the child
becomes apneic
(cyanotic or pale) but then takes a deep breath
Cyanotic breathholding spells In these spells, which typically
have an
emotional precipitant (eg, anger or frustration) and typically
last less than 1 minute, the child progresses from cyanotic to
apneic and may then become limp and lose consciousness if
a seizure occurs, the results from electroencephalography
(EEG) performed during rest or sleep are normal.
Pallid breathholding spells In these spells, which are
generally observed in response to pain, the child quickly
becomes apneic and pale an enhanced vagal response.
Head banging is the rhythmic hitting of the head (usually the frontal or
parietal region) against a solid surface. In children who are
developmentally normal, this behavior usually lasts less than 15
minutes, but it can last hours in some cases.
. Head banging can be associated with temper tantrums, tension, or
stress. It can also develop as a sleep ritual if it occurs as the child falls
asleep.
Complex stereotypies
Research on the mechanisms of complex motor stereotypies has
followed 2
different approaches. Psychogenic hypotheses aim to explain the
cause of
stereotypies from a behavioral standpoint. Neurobiologic hypotheses
look at the structural and molecular basis for stereotypies.
Psychogenichypotheses
stereotypies are more common in children with sensory deprivation
due to blindness or deafness and in conditions where there is less
interaction with the external environment (eg, autism).[4]
Another hypothesis is that stereotyped movements are a method of
expending excess energy or attention. Attending to the movements
may help to diminish other unwanted or unpleasant stimuli, as a form
of negative reinforcement..
For example, vestibular stimulation may reinforce head banging. It is
more common in infants with otitis media who have an unwanted
stimulus (eg, ear pain).
Neurobiologichypotheses

In humans, case reports of stereotyped movements have

spontaneously emerged after lesions of the putamen,
orbitofrontal cortex, or thalamus. A volumetric magnetic
resonance imaging (MRI) study in children with complex
motor stereotypies demonstrated a reduction in the size of
the caudate nuclei and also in
frontal white matter Dopaminergic pathways appear to
mediate complex motor stereotypiesFrom studies
investigating
the effect of selective dopamine agonists, dopamine D2
receptors are known to be important in enhancing
stereotypies.[7]
Genetic factors
In a study of developmentally normal children, 25%
had a family history of stereotypies, and a first
degree relative was affected in 17% of
cases.[11] Nearly half of the children had a
coexisting behavioral disorder.
Epidemiology
United States and international statistics Accurate prevalence figures for
childhood habits are extremely difficult to estimate,because of the
various classes of habits and the differing topographies of a childs
presenting habit.
many as two thirds of infants and in 20-50% of children overall.[13]
Thumb and hand sucking are seen in 17-59% of children younger than
15 years.
Thumb sucking is common in infancy and in as many as 25-50% of 2
yearold children however, it is observed in only 15-20% of 5 to 6yearold
children.
Nail biting is mainly observed from preschool age to adolescence and is
the most common stereotypy in school age children and college
students. it was found to exceed 60% in a group of 8yearolds.
Few studies of nose picking have been performed. However, in one
survey, as many as 91% of adults reported nose picking.
Breathholding spells are common in as many as 4-5% of children
younger than 8 years.
Bruxism is observed in 5-30% of children. The
worldwide prevalence is 8%.[14]
Head banging can occur in approximately 10% of
developmentally normal infants
and in 3-19% of developmentally normal children
younger than 3 years. It is more frequently observed
in children with autism or developmental delay
Age related demographics
The age at which specific habits originate, peak, and remit is related to the
individual habit behavior. The age of onset and resolution of habit behaviors
may be
delayed in children with developmental disabilities.
Thumb sucking may be observed in utero as early as 29 weeks gestation. It
declines with increasing age most children spontaneously stop at about age 4
years.
Nail biting rarely begins in children younger than 4 years. Its peak prevalence is
between the ages of 8 and 11 years,.
Nose picking is common in adults and children.
Bruxism is observed in 5-20% of children. The frequency increases during
childhood, peaking at age 7-10 years and decreasing after that. It is common in
children and adults.
Trichotillomania begins in later childhood.
Breathholding spells are reported to occur in 45% of the pediatric population.
They
may begin as early as infancy, and their peak incidence is at age 2-3 years.
Approximately 80-90% of preschoolers with breathholding spells stop by age 6
Sex and race related demographics

Overall, complex motor stereotypies are nearly

twice as likely to occur in males as in females. The
increased prevalence of conditions such as autism
or intellectual disability in males is a confounding
factor, but even in primary stereotypies, males
are more likely to be affected.There are no known
racial differences in the frequency of stereotypies.
Treatment
As of yet, there is no established pharmacological treatment for primary
motor stereotypies, however behavioral
therapy (habit reversal) has been shown to be helpful. There has been little
research in normally developing
(nonautistic) children.,.
Behavior therapy
Behavior therapy, administered by a psychologist, consisting of a
combination of awareness training and
competing response training has been an effective treatment for primary
motor stereotypies. Children are taught to
recognize the presence of their repetitive behaviors.
Drug therapy
To date, drugs have not proven to be an effective treatment for primary
motor stereotypies and are rarely
prescribed
 Tics (complex stereotyped
Most common movement)
movement disorder in children
Motor: eye blinking, shoulder shrug..
Vocal: squeaking, cough, sniffing
Sensory: sensation clothes not right..
Can be supressed; relief when expressed
again
Lessens in sleep
Aggravated by stress / anxiety
 Transient Chronic Tourettes

Single/multiple Single/multiple Multiple motor

motor AND/OR motor OR vocal AND
vocal single/multiple
vocal

=/> 4 weeks > 1 year > 1 year

< 1 year Not tic free > Not tic free >
3 months 3 months
 Tourettes Syndrome:
- combination motor and vocal tics
- present > 1 year
- onset before 18 years
- not only present during use of psychotropic drugs
Vocal tics can include echolalia, palilalia and
coprolalia but rarer than led to believe in lay
press (present in about 20%)
One third of cases asymptomatic by 17 years
 Clinical Diagnosis
Clues:
Previous normal phenomena (throat clearing,
eye blinking)
Family history tics / OCD
Features ADHD
Urge
Awareness occurrence
ABILITY TO SUPPRESS
No functional disability
Can persist in sleep
 Diff dx:
Chorea
Myoclonus
Stereotypies
Compulsions
Pseudotics
Secondary ass Strep infection PANDAS
 Associated disorders
Obsessions and compulsions - OCD
ADHD
50 60% of TS
precedes tics by 2-3 years
Sleep disorders
Learning problems - 5X more special ed
Behavioural problems
Mood disorders
Tics
OCD

TS

ADHD
THANK YOU