Stereotypic Movement Disorders
Introduction
Stereotypies are described as repetitive and purposeless movements that occur in a specific pattern and are distractible.1 These movements tend to occur more at times of increased stress, anxiety, excitement, focused concentration, or boredom.1, 2 Simple motor stereotypies (aka physiological stereotypies or common behaviors) occur commonly in both children and adults. These include leg shaking, hair twirling, and nail biting. Complex motor stereotypies are more complex movements such as hand flapping, finger or arm wiggling, mouth opening, orofacial movements, and body rocking.1 Alterations in breathing patterns or vocalizations may also accompany the movements. Both types of stereotypies, simple and complex, are also divided into 2 categories as either primary or secondary, depending on the presence of an additional neurologic or psychiatric diagnosis.3 Table 1 outlines common examples of stereotypies.
Section snippets
Description and Prevalence
Children with stereotypies often report that these movements are a pleasant experience, and may feel frustrated when parents and teachers interrupt them.4 One child described her movements as follows, “it just feels right…sometimes I hold my hands by my side to stop the movements but it doesn’t feel nice.”5 One theory is that the movements are a way of physically expressing or dealing with excitement, or can be a way of coping with boredom (ie, in both understimulating and overstimulating
Assessment of Children With Stereotypies
There are 3 main care-giver based rating scale questionnaires that can be helpful in evaluating stereotypies in children: (1) Motor Stereotypy Severity Scale (see Table 2 for description20) broken down into 3 components: Stereotypy Severity Scale motor (number, frequency, intensity, and interference), SS impairment to characterize global impairment, and a Linear Analog Scale 21, 22 (2) Repetitive Behavior Scale (see Table 3 for description23, 24) with 6 subscales to assess for stereotyped
Differential Diagnosis
A frequent question from referring physicians to pediatric neurology clinics is whether these movements could represent a seizure or a motor tic. Through a careful history, review of home videos, and observation in the clinical setting, these diagnoses can generally be distinguished from one another as described.
Parents and teachers often raise concerns that stereotypies could be seizures. The semiology of the movements typically points toward the appropriate diagnosis. Distinguishing features
Pathophysiology
Stereotypies are associated with dysfunction of the prefronto-corticobasal ganglia circuits, or cortico-striatal-thalamo-cortical pathways.15, 30 They are clearly linked to dopaminergic overstimulation given that they are a side effect from dopaminergic drugs such as amphetamine, cocaine, and levodopa.31 Similarly, lesioning dopaminergic input to striatal neurons prevents stimulant-induced stereotopy.32 Inhibitory cholinergic interneurons in these regions also play a role in modulating and
Management
The majority of stereotypies does not cause significant physical or emotional distress, and often do not require intervention. For stereotypies that are bothersome, habit reversal therapy can be successful in reducing their severity and frequency.42 In the classroom or home setting, response interruption and redirection when performed regularly and consistently can also be effective.43 A recent program aimed at DVD-based parent-delivered behavioral therapy showed reduction in stereotypies by
Conclusion
Motor stereotypies are relatively common in childhood and can occur with or without additional comorbidities, or in the setting of additional neurodevelopmental disorders. Complex motor stereotypies tend to present in early childhood and often do not change over a person’s lifetime. Current understanding of pathophysiology suggests involvement in dopaminergic pathways, and to some extent GABAergic and cholinergic pathways, in the prefronto-corticobasal ganglia circuits and
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Cited by (32)
Differential Diagnosis of Autism and Other Neurodevelopmental Disorders
2024, Pediatric Clinics of North AmericaSunflower Syndrome: A Survey of Provider Awareness and Management Preferences
2024, Pediatric NeurologyExamining the role of attention problems in motor stereotypy in children with autism spectrum disorder
2023, Research in Autism Spectrum DisordersCitation Excerpt :There seems to be a relationship between these factors as an overwhelming environment can lead to sensory overload and overwhelming thoughts which in turn lead to the excess of emotion that triggers stereotypy (Kapp et al., 2019). Indeed, both primary and secondary motor stereotypy have been associated with or enhanced by emotional and internal states such as excitement, daydreaming, anxiety, or boredom (Goldman et al., 2008; Harris et al., 2008, 2016; Machenzie, 2018; Mahone et al., 2004; Marino & Hedderly, 2019; Muthugovindan & Singer, 2009; Singer, 2009), and researchers have shown that certain types of motor stereotypy are associated with cardiovascular patterns of acceleration and deceleration that were unrelated to the physical demands of the movements (Heathers et al., 2019), which could suggest that the emotional state connected to the physical movements contributes to the increase in cardiovascular activity seen in motor stereotypy. Previous research has shown that children with ASD score significantly higher than children with ADHD and typically developing children on the Anxious/Depressed and Withdrawn subscales of the CBCL (Ooi et al., 2011) and that youth with autism’s Anxious/Depressed and Withdrawn subscale scores are significantly correlated with measures of anxiety and depression, but not autism related symptoms (Pandolfi et al., 2014), suggesting that these CBCL subscales are good at measuring anxiety and depression that is independent of autism symptoms but still significantly associated with autism over other conditions.
Mechanisms and Genetics of Neurodevelopmental Cognitive Disorders
2021, Mechanisms and Genetics of Neurodevelopmental Cognitive DisordersAbnormal Repetitive Behaviors and Self-Mutilations in Small Mammals
2021, Veterinary Clinics of North America - Exotic Animal PracticeCitation Excerpt :Cerebral plasticity is a process by which cerebral connections established in early life can change based on the environment. Although no specific gene had been linked to stereotypies in humans, it was found that up to 40% of affected children have a family member with stereotypies.10 Similarly, a genetic transmission of the fur-chewing behavior has been documented in chinchillas.17
Seizure mimics in children: An age-based approach
2020, Current Problems in Pediatric and Adolescent Health CareCitation Excerpt :Many children continue to have primary complex motor stereotypies into adulthood, though most reported an overall improvement in frequency and severity.29 Medications for secondary stereotypies such as fluoxetine, risperidone and clomipramine have been effective.26 Semiology: Dystonic movements are rhythmic movements from sustained or intermittent muscle contractions.30