Review Article

Stereotypies: From Normal to Pathological

Teresa Temudo1,2 Cludia Melo3,4

1 Department of Pediatric Neurology, Centro Materno Infantil do Address for correspondence Teresa Temudo, MD, PhD, Hospital de
Norte, Porto, Portugal Santo Antnio, Largo Abel Salazar, 4000, Poro, Portugal
2 Pre-graduate Department, Institute of Biomedical Sciences, Abel (e-mail: ttemudo11@gmail.com).
Salazar, Porto, Portugal
3 Department of Pediatric, Centro Hospitalar do Mdio Ave, Vila Nova
de Famalico, Portugal
4 Center for Research in Health Technologies and Information Systems
CINTESIS, Universidade do Porto, Porto, Portugal

J Pediatr Neurol

Abstract Stereotypies are rhythmic, patterned, repetitive, purposeless, and continuous move-
ments which may be categorized into the following two ways: primary, if they appear in
healthy normally developing subjects; or secondary, when they are associated with
neurologic problems. They may also be divided into motor, vocal, or visual stereotypies.
Biological mechanisms of stereotypies are unknown but they seem to be related to the
basal ganglia or the cortical-basal ganglia-thalamo-cortical circuits. Stereotypies are
Keywords frequently found in patients with autistic disorders, intellectual disability, and sensorial
stereotypies decit disorders. Some neurodevelopmental disorders show specic motor stereoty-
movement disorders pies, which reinforce the notion of a genetic or neurobiological basis. When necessary,
autism treatment may include behavioral or drug-based approaches.

Introduction functional motor behaviors that interfere with normal activities

Stereotypies are hyperkinetic movement disorders character-
ized by involuntary, rhythmic, predictable, and repetitive
movements. They are more frequent in the pediatric popula-
tion, but may also be found in adults.1 Their presence in both
typically developing children and neurodevelopmental dis-
orders remains to be explained and formal prevalence is
unknown. A systematic classication and extensive clinical
characterization will guide the distinction between stereoty-
pies and other movement disorders.
Denition
Stereotypies are a common and heterogeneous movement
disorder typically characterized by involuntary, rhythmic, pat-
terned, coordinated, repetitive, and seemingly purposeless
movements that are usually continuous.2 The Diagnostic and
Statistical Manual of Mental Disorders, 5th edition, text revision
denes stereotypies as repetitive, seemingly driven, and non-
or result in injury.3 A new denition of stereotypies states that
stereotypies are non-goal-directed movement patterns that are
repeated continuously for a period of time in the same form and
on multiple occasions, and which are typically distractible.4
Harvey S. Singer5 in his denition reinforces the suppressibility
of stereotypies with distraction. Throughout the several de-
nitions, the authors agree on the involuntary, rhythmic, and
purposeless characteristics of the stereotypies, but a consensus
denition is still needed. Although easily suppressible in normal
children, they can be very difcult or impossible to suppress in
many children as is the case of stereotypies in patients with Rett
syndrome (RTT). Maybe those pleomorphic clinical manifesta-
tions could be explained by different pathophysiology of these
movements at the diverse diseases.
Current Etiologic Theories
The neuropathological basis of stereotypies remains un-
known, but several psychological and biological theories

received Issue Theme Movement Disorders; Copyright by Georg Thieme Verlag KG, DOI http://dx.doi.org/
March 27, 2015 Guest Editors: Nardo Nardocci, MD, Stuttgart New York 10.1055/s-0035-1559805.
accepted after revision and Giovanna Zorzi, MD ISSN 1304-2580.
March 28, 2015
Stereotypies: from Normal to Pathological Temudo, Melo
have been proposed. Psychologists theories claim that ste-
reotypies are learned behaviors that are sustained through
continuous reinforcement.6,7 Others argue for an arrested
neurologic maturation etiology,7 or that the behavior even
serves as a way to moderate levels of arousal.6,8
Stereotypies frequent association with neurodevelopmental
disorders such as autistic disorders, RTT, or fragile-X syndrome
supports a neurobiological basis. A familial history of stereoty-
pies reinforces their genetic basis.9,10 Reports have described the
implication of stereotypies on lesions at the basal ganglia11 and
frontotemporal lobe,11,12 but no clear correlation has been
demonstrated. Animal models have also shown that stereotypies
may appear after insults to the brain or from drugs.13,14 Never-
theless, the neuroanatomical localization for stereotypies is
unknown, although researchers have suggested abnormalities
within cortico-striatal-thalamo-cortical pathways. Furthermore,
interacting cortico-striatal-thalamo-cortical circuits provide the
framework for understanding goal-directed and habitual behav-
iors such as primary stereotypies and tics. Habitual behaviors
circuit seem to be associated to premotor putamen.5 This
evidence was supported by reductions in the size of frontal
and temporal white matter and the caudate nucleus that were
found in volumetric magnetic resonance imaging studies in a
small number of children with primary complex motor stereo-
typies.15 Stereotypies were also reported in patients following
strokes involving the right putamen,16 the right lenticular
nucleus,17 or the bilateral paramedian thalamic and midbrain
regions18 and in those with frontotemporal dementia.19 In
autistic children, stereotyped behaviors positively correlated
with frontal lobe volumes.20 Abnormalities of dopamine neuro-
transmission may also have been implicated.21 Support for
glutamate involvement in stereotypies is based on therapeutic
reports showing that glutamate-altering medications had a
benecial effect on ritualistic behaviors and other obsessive-
compulsive symptoms.22,23
Epidemiology
There have been few epidemiologic studies on stereotypies.
The prevalence in healthy children as well as in patients with
sensorial decits or neurodevelopmental disorders varies
extremely between studies. Confusion about stereotyped
behavior is revealed in several studies. The term stereotypy
is frequently applied to a repetitive behavior or interest (e.g.,
Table 1 Neurologic and developmental disorders and typical-related stereotypies
Disorders
Rett syndrome
Frontotemporal lobe degeneration
SmithMagenis syndrome
Autism
Angelman syndrome
Fragile X syndrome
Journal of Pediatric Neurology
lining up objects, walking in circles, undressing dolls). We
believe that those behaviors should be categorized indepen-
dently of stereotypies to allow a better understanding of their
nature and biological mechanisms.
Classication
Stereotypies may be classied into primary or secondary
stereotypies. Primary stereotypies occur in the absence of a
neurodevelopmental disorder, are more frequent in the
rst years of life, and tend to disappear during childhood,
although they can remain in healthy adults. Secondary
stereotypies are linked to children or adults with intellec-
tual disability, autism, and other neurodevelopmental dis-
orders and sensory impairments.24 Motor stereotypies are
the most common, but they may also be vocal25 or visual.26
Stereotypies can also be classied as simple (e.g., tapping,
mouthing, clapping) or complex (e.g., a sequence of differ-
ent movements always performed in the same way and
sometimes seeming to have a purpose)15,26 and according
to the predominant site involved (e.g., head, trunk, hands,
legs) (Tables 1 and 2).
Clinical Features
The diagnosis of stereotypies is based on clinical grounds. The
rst notion to retain is that stereotypies are hyperkinetic
movement disorders with jerky movements and they must be
distinguished from other hyperkinetic movement disorders
as myoclonus, chorea, and tics. Stereotypies tend to occur
when the child is stressed, excited, fatigued, or bored. Sup-
pression with distraction or with the beginning of other
activity has been described; nonetheless, we believe that
secondary stereotypies do not always adhere to these criteria.
It is also unknown whether children with developmental
delays are able to suppress their stereotypies. Second, stereo-
typies should be divided into motor or vocal and even simple
or complex categories. Stereotypies may be classied accord-
ing to the body segment involved (Table 2). Frequently
described stereotypies are body rocking, hand washing,
apping, tapping, and mouthing, but there are many others
(Fig. 1).
Stereotyped hand movements are a hallmark of RTT, and
one of its necessary diagnostic criteria.27 Usually, these
Typical stereotypies
Hand washing stereotypies
Vocal stereotypies
Spasmodic upper body squeeze or self-hug behavior
Atypical gazing at objects or ngers
Combination of hand apping, mouthing, and clapping
Self-rubbing
 Stereotypies: from Normal to Pathological Temudo, Melo

Table 2 Classication of stereotypies according to the body segment involved

Body segments Stereotypies

Body Body rocking; shifting weight from one leg to the other
Head Head nodding, retropulsion, bruxism, protrusion of the lips, eye rolling
Arms Arm apping; arm exion
Hands Wringing, clapping, mouthing, tapping, sevillana, apping, nger wiggling,
nger tapping, counting ngers, hair pulling, twisting ngers, hand behind the neck
Legs Intermittent leg elevation and tapping of the oor, toe walking, jumping, feet twirling
Visual Hand gaze; object gaze
Vocal Bruxism, repetitive sounds, repetitive words or phrases

movements are associated with or follow the disappearance
of purposeful hand movements, but can also be presented
before developmental regression begins.28,29 These almost
continuous, repetitive, compulsive automatisms disappear
during sleep. Other stereotyped movements and behaviors
can also be presented in RTT, but are much less well de-
scribed.30 It seems that hand gaze and stereotypies with
objects are more related to autism than with RTT.26
Differential Diagnosis
Stereotypies must be distinguished from other conditions
such as tics, dystonias, paroxysmal dyskinesias, myoclonic
jerks, chorea, hyperekplexia, seizures, drug-induced move-
ments, and psychogenic movement disorders.31,32 Probably,
the most challenging differential diagnosis is between ste-
reotypies and tics. Furthermore, tics and stereotypies may
coexist in the same patient. The taskforce on childhood
movement disorders dened tics as repeated, individually
recognizable, intermittent movements or movement frag-
ments that are almost always briey suppressible and are
usually associated with awareness of an urge to perform the
movement.31 Classically, tics are preceded by a premonitory
urge, can be suppressed by an effort of will, and have a waxing
and waning course.32 Clinical features that should make one
consider stereotypies rather than tics are listed herein
(Table 3).
Management of Stereotypies
Stereotypies may occur with no interference of normal
activity and treatment may not be required. Concern arises

Fig. 1 (A) Mouthing; (B) Counting ngers; (C) Flapping; (D) Knocking the hand; (E) Hand rubbing; (F) Hand to head; (G) Finger tapping; (H)
Rubbing; (I) Hand washing.

Journal of Pediatric Neurology

Stereotypies: from Normal to Pathological Temudo, Melo
Table 3 Clinical features of tics and stereotypies
Clinical features Stereotypies
Onset Before 3 y
Family history Sometimes positive
Premonitory urge No
Migration Not frequent
Duration More continuous
Topography Distal; arms and legs
Suppression with distraction Controversial
Characteristics Rhythmic, repetitive, and purposeless
Evolution Persistent, patterned
Drug treatment Poor responsive
Note: Modied classication from Mahone et al 2004.37
when they do interfere and mainly when they are self-
injurious. Severely restricting stereotypies are an indication
for treatment. Behavioral approaches include mechanical
restraints, response interruption and redirection methods,
noncontingent stimulation, and contingency manipula-
tions.33 Drug treatment of stereotypies is most appropriate
when the behavioral approach is not available or fails. Clo-
mipramine, risperidone, and uoxetine have been shown to
reduce stereotypies in autistic patients.3436 However, there
are no randomized and placebo-controlled clinical trials so
there is no clear, robust evidence.
In conclusion, stereotypies are a common condition in
healthy and developmentally delayed children. They must be
distinguished from tics. In autistic patients, an effort should be
made to separate stereotypies from repetitive behaviors and
restrictive interests. A better clinical characterization of stereo-
typies will probably help us to understand their biologic
mechanisms. Advances in neuroimaging, genetics, and neuro-
pathological studies may also help in understanding the nature
of stereotypies. Stereotypies may be disabling for patients and
their families, even though therapies targeting the pathophysi-
ology of the disorder are still missing. Behavioral approaches
should be the rst option when treatment is necessary. 15
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