Author: Harvey S. Singer, MD, Professor Emeritus Johns Hopkins Medicine, Clinical Faculty Kennedy Krieger Institute
Reviewed: Janaury 2022
SUMMARY
The term “motor stereotypies” is used to define a large group of repetitive movements that are:
- Involuntary
- Rhythmic
- Fixed
- Purposeless
- Prolonged
These movements occur in a variety of different types and forms. They increase when a child is engrossed in an activity. They stop with distraction. Stereotypies are different from other kinds of movement disorders.
Primary complex motor stereotypies (PCMS) is a common type of motor stereotypy.
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Disorder Overview
DESCRIPTION
The exact percentage of people with motor stereotypy is not known. However, it is estimated that it affects:
- 90% of children with autism spectrum disorder
- 20% to 70% of children (simple type)
- 2 to 4% of children (complex type)
Defining Characteristics
There are several characteristics that define the disorder. A motor stereotypy:
- Is involuntary. A child does it without conscious choice.
- Is rhythmic. It occurs at regular intervals
- Is fixed. It occurs in the same pattern every time.
- Is purposeless. It is not done to achieve a goal.
- Is prolonged. It can continue for multiple seconds, minutes, or hours.
- Stops with distraction. Calling or touching the individual stops the movement.
Primary and Secondary Forms
There are two distinct stereotypy forms:
- Primary form. This form occurs in an otherwise normally developing child. In this form, the movements themselves are a primary symptom. In other words, they are not caused by another disorder.
- Secondary form. This form usually appears in children or adults with other developmental issues. Most stereotypies that begin in adulthood are secondary. The secondary form is:
- Common in children with autism spectrum disorder (ASD)
- Sometimes seen in those who have:
- Genetic syndromes
- Encephalitis
- Metabolic disorders
- Degenerative conditions
- Sensory deficits, such as deafness or blindness
- Sometimes induced by drug or medication use
Age of Onset
SIGNS AND SYMPTOMS
There are three main types of stereotypic movements.
1. Simple.
2. Head nodding.
3. Complex.
Other activities may accompany the movements above. These can include:
- Opening the mouth
- Grimacing
- Rocking
- Pacing
- Making a sound or noise
Triggers
Duration and Timing
Movements can last for seconds or minutes. In some people they can last hours. They usually occur multiple times per day.
Motor stereotypies can usually be quickly stopped by:
- Stimulating the senses in another way
- Distracting the child by touching them
- Distracting the child by calling their name
CAUSES
The exact cause of the primary form is unknown. The secondary form is caused by another underlying disorder, such as autism spectrum disorder.
DIFFERENTIATING STEREOTYPIES FROM OTHER MOVEMENT DISORDERS
Other movement disorders are sometimes confused with motor stereotypies. It is important to correctly diagnose movement disorders since a correct diagnosis can lead to proper treatment. Some of these other movement disorders include:
1. Tics.
Motor tics are quick, rapid movements. They involve either a cluster of simple movements or a more complex sequence of movements. Stereotypies are different from tics:
- Stereotypies begin at an earlier age than tics
- Stereotypies are fixed, rhythmic, consistent, and prolonged in duration
- Tics involve an urge or desire to perform
- Tics do not abruptly stop with distraction
2. Self-injurious behaviors (SIBs).
3. Compulsions.
Compulsions are performed over and over in a fixed fashion. They must be done a certain number of times. They must be done equally on both sides of the body. They are driven by unconscious ideas or impulses. Examples of common compulsions include:
- Handwashing
- Touching
- Counting
- Checking
COMMON SUBTYPE: PRIMARY COMPLEX MOTOR STEREOTYPIES
Primary Complex Motor Stereotypies (PCMS) is a common subtype of the disorder. Here are some of its defining characteristics:
- It is a primary form of the disorder. It is present in children who are otherwise developing normally.
- It involves complex motor stereotypy movements. The movements must also occur on both sides of the body. They may evolve over time.
- It lasts for longer than a few moments. It lasts for seconds to minutes, or even up to about an hour.
- It has notable triggers. It is associated with periods of engrossment, excitement, stress, fatigue, or boredom.
- It can occur multiple times per day.
- It has an early age of onset. It typically begins before a child reaches age 3.
- It can be stopped with distraction.
Children often report at least one of the following:
- They are unaware of the movements
- They enjoy doing the movements
- They are thinking about a TV program or activity (they are mentally visualizing something)
Children with PCMS may also have:
- Attention-deficit/hyperactivity disorder (ADHD)
- Tics
- Anxiety
- Motor coordination problems
- Obsessive-compulsive behaviors
Children with PCMS tend not to be bothered by the movements when they are young. However, parents often worry about the disruptions and social stigma they can cause. The movements may affect the quality of life of older children.
What Causes PCMS?
Researchers have been working to understand what causes PCMS. Some early research suggests a genetic cause. This is based on the finding that an affected child has about a 25% to 40% chance of having a close or distant family member with a similar problem.
Researchers have also tried to understand exactly how motor stereotypies are controlled by the brain. One study looked at pathways in the brain that are necessary for normal movements. It found that there was an imbalance in connections involving two essential pathways in children with PCMS.
Chemical messengers transmit signals in the brain. They may also play a role in causing PCMS.
LABORATORY TESTS
Primary Form
Secondary Form
Lab tests are used to evaluate individuals with secondary forms of motor stereotypies. Various tests may help doctors identify the underlying problem. Some of the tests used are:
- Blood tests
- Urine tests
- Genetic tests
- Magnetic resonance imaging (MRI), to see pictures of the brain
- Electroencephalogram (EEG), to see electric patterns in the brain
TREATMENT AND THERAPIES
Education
Behavioral Therapy and Medication
It may be important to consider treatment. This is usually considered if a child’s movements are causing:
- Frequent comments from the child’s peers or family
- Physical problems
- Disruptions at school
Behavioral Therapy
Finding an effective way to treat motor stereotypies with behavioral therapy can be difficult. A variety of approaches have been used, especially for children with autism spectrum disorder (ASD). Two of the more popular approaches for children with ASD include:
- Response blocking
- Response redirection
Effective approaches in children with PCMS have included a combination of:
- Awareness training
- Differential reinforcement of other behaviors
In children with PCMS, behavioral therapy may be offered at home by a parent. This approach works best with help from a professional therapist.
Medications
Some early research has suggested that certain types of drugs might be able to reduce stereotypies in children with autism spectrum disorder. However, more research is needed to learn whether they can really help. Further, the use of the suggested medications are associated with significant side effects.
There is no formal research on the effect of medication in children with PCMS. However, based on parent reports, a wide range of medications have not been helpful with PCMS.
OUTCOMES
Primary Form
Simple Type
Movements in children with primary simple motor stereotypies often resolve over several years.
Complex Type
In contrast, children with primary complex motor stereotypies continue to experience movements during their teenage years. In some, the movements can last into adulthood. The number of daily episodes and how long they last, however, typically go down during the teenage years.
Secondary Form
The outcome of secondary motor stereotypies can vary widely. They differ based on the type, cause, and severity of the underlying cause.
Resources
Johns Hopkins Motor Stereotypies Behavioral Therapy Program
Johns Hopkins researchers have developed a behavioral therapy program–The Johns Hopkins Motor Stereotypies Behavioral Therapy Program–for parents to use with their children between the ages of 7 and 17. The program has been demonstrated to be helpful in reducing the severity of motor stereotypies. A two-minute YouTube video gives an overview of the program and highlights examples of this condition. The video program (which will be sent only as a downloadable .mp4 file) provides information about the home-based behavioral therapy for children age 7 and older who have been diagnosed with Primary Complex Motor Stereotypies. The cost is $95.99 and can be purchased online through an order form.
Research
Currently, there are no listings for Motor Stereotypies in ClinicalTrials.gov. Check back often and talk with your healthcare provider to identify upcoming trials.
If you have an interest in searching for any future trials that may be starting:
- Go to the Home page of ClinicalTrials.gov
- Under “Status”, click on “Recruiting and not yet recruiting studies”
- Under “Condition or disease”, type Motor Stereotypies in the field. A drop-down list will appear if this disorder is included.
- You can narrow the search by entering a Country name
- Once on the disorder page of trials, narrow your search under “Eligibility Criteria” and click “Age/Age Group/Child (birth to 17)”
ClinicalTrials.gov is a database of privately and publicly funded clinical studies conducted around the world. This is a resource provided by the U.S. National Library of Medicine (NLM), which is an institute within the National Institutes of Health (NIH). Listing a study does not mean it has been evaluated by the U.S. Federal Government. Please read the NLM disclaimer for details.
Before participating in a study, you are encouraged to talk to your health care provider and learn about the risks and potential benefits.
The information in the CNF Child Neurology Disorder Directory is not intended to provide diagnosis, treatment, or medical advice and should not be considered a substitute for advice from a healthcare professional. Content provided is for informational purposes only. CNF is not responsible for actions taken based on the information included on this webpage. Please consult with a physician or other healthcare professional regarding any medical or health related diagnosis or treatment options.
References
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