Adult-onset stereotypical motor behaviors.

Stereotypies have been defined as non-goal-directed movement patterns repeated continuously for a period of time in the same form and on multiple occasions, and which are typically distractible. Stereotypical motor behaviors are a common clinical feature of a variety of neurological conditions that affect cortical and subcortical functions, including autism, tardive dyskinesia, excessive dopaminergic treatment of Parkinson's disease and frontotemporal dementia. The main differential diagnosis of stereotypies includes tic disorders, motor mannerisms, compulsion and habit. The pathophysiology of stereotypies may involve the corticostriatal pathways, especially the orbitofrontal and anterior cingulated cortices. Because antipsychotics have long been used to manage stereotypical behaviours in mental retardation, stereotypies that present in isolation tend not to warrant pharmacological intervention, as the benefit-to-risk ratio is not great enough.

Functional properties of behaviour problems depending on level of intellectual disability.

BACKGROUND: Behaviour problems are common among individuals with intellectual disabilities (ID) especially in those with more severe forms. The determination of the functional profile of a targeted behaviour has important implications for the design of customised behavioural interventions. METHOD: We investigated the relationship between the level of ID and the functional profile of aggression, stereotypy and self-injurious behaviour (SIB) using the Questions about Behavioural Function (QABF). Two staff members at two time points completed the QABF for each of 115 adults with varying levels of ID participating in a day training and habilitation programme. RESULTS AND CONCLUSIONS: Our results suggest that there is a differential relationship between the functions of behaviour problems and level of ID. While SIB is more often seen by raters to be maintained by escape of social demands and by attaining access to tangible items with the decline of the intellectual level, aggressive and stereotypic behaviours were identified more often as serving multiple functions equally across functioning level.

The role of task preference in the effectiveness of response interruption and redirection.

Response interruption and redirection (RIRD) is a common treatment for automatically reinforced vocal stereotypy; it involves the contingent presentation of task instructions. Tasks that are included in RIRD are typically selected based on caregiver report, which may affect the efficacy of RIRD. The purpose of the current study was to evaluate the role of task preference in the efficacy of RIRD for four participants who engaged in vocal stereotypy. We conducted task-preference assessments and selected tasks of varying preferences to include in RIRD. For three out of four participants, the results showed that RIRD with higher preference tasks was not effective at reducing vocal stereotypy, whereas RIRD with lower preference tasks was effective for all participants.

Development and Validation of an Assessment-Driven Behavioral Intervention for Primary Complex Motor Stereotypies in Young Children.

Complex motor stereotypies are rhythmic, repetitive, fixed, and non-goal directed movements (e.g., bilateral flapping/waving movements of the hands/arms). Movements typically begin in early childhood and can occur in otherwise normally developing ("primary") or autistic ("secondary") children. Stereotypies persist, occur multiple times a day, have prolonged durations, can be socially stigmatizing, and may lead to bullying and isolation. Prior behavioral treatment studies have focused on older children (ages 6-12) and report modest reductions in stereotypy (i.e., between 14% and 33%). The current study involves the functional assessment and treatment of five children with Primary Complex Motor Stereotypy using a modified awareness training procedure, differential reinforcement of other behavior, and schedule thinning in a nonconcurrent multiple baseline design. Results suggest a 99% reduction of motor stereotypy from baseline across all participants.

Examining Procedural Variations of Delivering Competing Stimuli in the Treatment of Stereotypy.

Competing stimulus assessments (CSA) are effective tools for identifying stimuli that compete with automatically reinforced behavior. However, Jennett et al. suggests there are cases for which non-contingent access to competing stimuli are insufficient at decreasing target responding and additional treatment components may be necessary. The purpose of the current study was to examine procedural variations (i.e., rotating competing items and prompted engagement) when presenting competing stimuli on increasing functional engagement and decreasing stereotypy. Following a functional analysis, a CSA was conducted to identify competing stimuli for four individuals with autism. Items identified were then used with two procedural variations. Levels of stereotypy, functional engagement, and item contact were measured. Results showed that for two participants both treatments were effective, while for the other two participants prompting functional engagement was more effective. Prompting functional engagement is likely a productive strategy for enhancing engagement with competing stimuli for automatically reinforced problem behavior as it may result in functional engagement becoming reinforcing in and of itself.

Raclopride or high-frequency stimulation of the subthalamic nucleus stops cocaine-induced motor stereotypy and restores related alterations in prefrontal basal ganglia circuits.

Motor stereotypy is a key symptom of various neurological or neuropsychiatric disorders. Neuroleptics or the promising treatment using deep brain stimulation stops stereotypies but the mechanisms underlying their actions are unclear. In rat, motor stereotypies are linked to an imbalance between prefrontal and sensorimotor cortico-basal ganglia circuits. Indeed, cortico-nigral transmission was reduced in the prefrontal but not sensorimotor basal ganglia circuits and dopamine and acetylcholine release was altered in the prefrontal but not sensorimotor territory of the dorsal striatum. Furthermore, cholinergic transmission in the prefrontal territory of the dorsal striatum plays a crucial role in the arrest of motor stereotypy. Here we found that, as previously observed for raclopride, high-frequency stimulation of the subthalamic nucleus (HFS STN) rapidly stopped cocaine-induced motor stereotypies in rat. Importantly, raclopride and HFS STN exerted a strong effect on cocaine-induced alterations in prefrontal basal ganglia circuits. Raclopride restored the cholinergic transmission in the prefrontal territory of the dorsal striatum and the cortico-nigral information transmissions in the prefrontal basal ganglia circuits. HFS STN also restored the N-methyl-d-aspartic-acid-evoked release of acetylcholine and dopamine in the prefrontal territory of the dorsal striatum. However, in contrast to raclopride, HFS STN did not restore the cortico-substantia nigra pars reticulata transmissions but exerted strong inhibitory and excitatory effects on neuronal activity in the prefrontal subdivision of the substantia nigra pars reticulata. Thus, both raclopride and HFS STN stop cocaine-induced motor stereotypy, but exert different effects on the related alterations in the prefrontal basal ganglia circuits.

A retrospective analysis of stereotypy: Applicability of the behavioral subtyping model.

Recent research on automatically reinforced self-injurious behavior (ASIB) has identified specific patterns of responding in functional analyses that correlate with intervention efficacy (Hagopian et al., 2015; Hagopian et al., 2017). Whereas research by Hagopian et al. (2015, 2017) points to an important development in the assessment and treatment of ASIB, it is unclear if the applicability extends to automatically reinforced noninjurious behaviors, including stereotypy. Therefore, the current study replicated the methods of Hagopian et al. (2017), extending this research to published cases of stereotypy and related behavior. The behavioral subtype for each case was identified, and where applicable, the subtype was compared to intervention outcome data. The categorization of data sets as either Subtype 1 or Subtype 2 did not correspond with specific treatment outcomes. Unlike the results of Hagopian et al. (2015, 2017), reinforcement-based interventions were not more likely to be effective for Subtype 1 stereotypy than for Subtype 2 stereotypy.

Insights into pathophysiology of punding reveal possible treatment strategies.

Punding is a stereotyped behavior characterized by an intense fascination with a complex, excessive, nongoal oriented, repetitive activity. Men tend to repetitively tinker with technical equipment such as radio sets, clocks, watches and car engines, the parts of which may be analyzed, arranged, sorted and cataloged but rarely put back together. Women, in contrast, incessantly sort through their handbags, tidy continuously, brush their hair or polish their nails. Punders are normally aware of the inapposite and obtuse nature of the behavior; however, despite the consequent self-injury, they do not stop such behavior. The most common causes of punding are dopaminergic replacement therapy in patients affected by Parkinson's disease (PD) and cocaine and amphetamine use in addicts. The vast majority of information about punding comes from PD cases. A critical review of these cases shows that almost all afflicted patients (90%) were on treatment with drugs acting mainly on dopamine receptors D1 and D2, whereas only three cases were reported in association with selective D2 and D3 agonists. Epidemiological considerations and available data from animal models suggest that punding, drug-induced stereotypies, addiction and dyskinesias all share a common pathophysiological process. Punding may be related to plastic changes in the ventral and dorsal striatal structures, including the nucleus accumbens, and linked to psychomotor stimulation and reward mechanisms. Possible management guidelines are proposed.

Neurodevelopmental movement disorders - an update on childhood motor stereotypies.

AIM: The term 'stereotypies' encompasses a diverse range of movements, behaviours, and/or vocalizations that are repetitive, lack clear function, and sometimes appear to have a negative impact upon an individual's life. This review aims to describe motor stereotypies. METHOD: This study reviewed the current literature on the nature, aetiology, and treatment of motor stereotypies. RESULTS: Motor stereotypies occur commonly but not exclusively in autistic spectrum disorders. Similar movements are also found in otherwise healthy children and those suffering sensory impairment, social isolation, or severe intellectual disabilities; they may be persistent over time. Although often difficult, it is possible to define and differentiate stereotypies from other movement disorders such as tics through features of the history, such as earlier onset and examination, together with the presence or absence of associated neurological impairment or developmental difficulties. Co-occurrence with other disorders affecting frontostriatal brain systems, including attention-deficit-hyperactivity disorder, obsessive-compulsive disorder, and tic disorders, is common. INTERPRETATION: The underlying function of motor stereotypies remains unclear but may include the maintenance of arousal levels. A neurogenetic aetiology is proposed but requires further study. When treatment is sought, there are both pharmacological and behavioural options. Behavioural treatments for motor stereotypies may in time be shown to be most effective; however, they are difficult to implement in children younger than 7 years old.

Rethinking Stereotypies in Autism.

Stereotyped movements ("stereotypies") are semi-voluntary repetitive movements that are a prominent clinical feature of autism spectrum disorder. They are described in first-person accounts by people with autism as relaxing and that they help focus the mind and cope in overwhelming sensory environments. Therefore, we generally recommend against techniques that aim to suppress stereotypies in individuals with autism. Further, we hypothesize that understanding the neurobiology of stereotypies could guide development of treatments to produce the benefits of stereotypies without the need to generate repetitive motor movements. Here, we link first-person reports and clinical findings with basic neuroanatomy and physiology to produce a testable model of stereotypies. We hypothesize that stereotypies improve sensory processing and attention by regulating brain rhythms, either directly from the rhythmic motor command, or via rhythmic sensory feedback generated by the movements.

Brief Report: Mobile Technology to Support Parents in Reducing Stereotypy.

Although behavioral interventions have been known to effectively reduce stereotypy in children with ASD, these types of interventions are not accessible to all families. In response to this issue, we evaluated the effects of the iSTIM, an iOS application designed to support parents in the reduction of stereotypy in their child with ASD. We used a series of AB designs to determine the effectiveness of the iSTIM on stereotypy using parents as behavior change agents. The use of iSTIM by the parents led to a reduction in stereotypy for six of seven participants. Our results suggest that the use of technology may be a cost effective and easily accessible method for parents to reduce stereotypy in their child with ASD.

Autism and Access to Care During the COVID-19 Crisis.

CASE: Brian is a 6-year-old boy who was diagnosed with autism spectrum disorder (ASD) and global developmental delay at age 2. He has no other health conditions of note. Brian lives with his parents and an older brother, who also has ASD, in a rural area 2 hours from the center where he was diagnosed. Brian has a history of intermittent self-injurious behaviors (head-banging, throwing himself onto the floor, etc.) that regularly result in bruising, intense and lengthy tantrums, and aggression toward family and teachers. Brian will occasionally indicate items that he wants, but otherwise has no functional communication skills. Over the past 18 months, Brian's challenging behaviors have waxed and waned. The regional special education program is not equipped to safely manage his behaviors, and there are no in-home or center-based agencies that provide applied behavior analysis (ABA) available. Brian's developmental pediatrician initiated guanfacine (eventually adding a small dose of aripiprazole) and referred the family to psychology for weekly telehealth behavioral parent training to address behavioral concerns using the Research Units in Behavioral Intervention curriculum.1Brian's behavioral problems decreased during the initial weeks of the COVID-19 crisis, when he no longer had to leave home or attend special education. However, as summer continued, his behaviors worsened substantially (regular bruising and tissue damage, numerous after-hours consultations with his psychologist and developmental pediatrician, and one trip to the emergency department). The intensity of Brian's behaviors (maintained primarily by access to tangible items and escape from demands) made progress with behavioral supports slow and discouraging for his parents. Other psychosocial stressors coalesced for the family as well, including employment loss, limited social support because of social distancing requirements, and illness of one of his parents. The developmental pediatrician continued to modify the medication regimen over the summer, transitioning Brian from guanfacine to clonidine and increasing his aripiprazole incrementally (with clear increased benefit); hydroxyzine was also used as needed during the episodes of highest intensity.Despite the availability of best-practice guidelines for children with Brian's presenting concerns,2 a confluence of barriers (geographic, economic, ABA work force, global pandemic, etc.) present serious questions for his family and care team related to the next steps in Brian's care. Should he attend in-person school in the fall, knowing that the available program may have limited educational benefit and increase his risk of COVID-19 exposure (not to mention self-injury)? Would the potential benefits of cross-country travel to an intensive behavioral treatment program outweigh the associated psychosocial and economic stressors? How else can the virtual care team support this family? REFERENCES: 1. Bearss K, Johnson C, Smith T, et al. Effect of parent training vs parent education on behavioral problems in children with autism spectrum disorder: a randomized clinical trial. JAMA. 2015;313: 1524-1533.2. Hyman SL, Levy SE, Myers SM, et al. Identification, evaluation, and management of children with autism spectrum disorder. Pediatrics. 2020;145:e20193447.

Evaluating a treatment without extinction for elopement maintained by access to stereotypy.

Elopement is a common and potentially dangerous form of problem behavior. Results of a functional analysis found that the elopement of a child with autism was maintained by access to stereotypy in the form of door play. We implemented functional communication training and contingency-based delays dependent on the absence of elopement and increased the amount of time the participant waited prior to engaging in stereotypy. We also conducted treatment-extension probes, with the participant waiting up to 10 min without elopement.

To What Extent Do Practitioners Need to Treat Stereotypy During Academic Tasks?

Researchers frequently argue that a child's engagement in stereotypy may compete with his ability to acquire academic skills, engage in appropriate social interactions, or both; however, few studies have directly tested these suppositions. We used a five-phase assessment to evaluate the extent to which behavioral interventions with a progressively greater number of components were necessary to decrease stereotypy and increase correct responding during academic instructions for five children diagnosed with autism spectrum disorders. For one participant, stereotypy decreased when instructors provided standard instruction without specific intervention for stereotypy. For two participants, stereotypy decreased when instructors provided standard instruction plus antecedent intervention for stereotypy with continuous music. For another participant, stereotypy decreased when instructors provided enhanced consequences for correct responding during standard instruction without either antecedent or consequent intervention for stereotypy. For the final participant, stereotypy decreased and correct responding increased when instructors provided standard instruction and consequent intervention for stereotypy.

A comparison of differential reinforcement procedures for treating automatically reinforced behavior.

Children diagnosed with autism spectrum disorder (ASD) often exhibit stereotypy, which can be socially stigmatizing, interfere with daily living skills, and affect skill acquisition. We compared differential reinforcement of alternative behavior (DRA) and differential reinforcement of other behavior (DRO) when neither procedure included response blocking or interruption for (a) reducing stereotypy, (b) increasing task engagement, and (c) increasing task completion. DRA contingencies yielded superior outcomes across each measure when evaluated with 3 individuals with autism spectrum disorder.

Motor stereotypy disorders.

PURPOSE OF REVIEW: This review highlights recent advances in understanding the clinical features, prevalence, and outcomes of motor stereotypy disorders in typically developing children. RECENT FINDINGS: Longitudinal data indicate that stereotypies in children with normal intelligence show an early age of onset, chronicity, and high prevalence of comorbid difficulties, including tics, obsessive-compulsive behaviors, and attention deficit hyperactivity disorder. The underlying abnormality remains unknown, but there is increasing evidence for Mendelian inheritance and a neurobiological mechanism. SUMMARY: Primary motor stereotypies are relatively common in childhood and can be subdivided into three groups (common, head nodding, and complex motor). Movements are similar to those seen in children with autistic spectrum disorders, mental retardation, and sensory deprivation. The role of pharmacotherapy is not established and behavioral therapy can be beneficial.

Tics and stereotypies: A comparative clinical review.

Tics and stereotypies are the most common pathological repetitive complex motor behaviors occurring during the neurodevelopmental period. Although they may appear transiently during development without acquiring a pathological status, when they become chronic they may be distressing, socially impairing, or even, in the case of malignant tics, potentially physically harmful. Despite a certain similarity in their phenomenology, physicians should be able to distinguish them for their different variability over time, topographical distribution, association with sensory manifestations, and relationship with environmental triggers. The complex phenomenology of tics and stereotypies is constantly enriched by the characterization of novel variants, e.g. tics triggered by auditory stimuli in association with misophonia and stereotypies associated with intense imagery activity. Their pathophysiology remains partially elusive, but both animal model and brain imaging studies confirm the involvement of all the three major loops (sensorimotor, associative and limbic) within the cortico-basal ganglia circuitry. From a management perspective, the greatest advances witnessed in the last decade involve the diffusion of behavioral strategies (e.g. habit reversal training or response interruption and redirection), including the development of protocols for telehealth on online training in order to optimise access. In the context of severe tics, e.g. in refractory Tourette syndrome, there is increasing experience with deep brain stimulation of the intralaminar thalamic nuclei or the globus pallidus internus, although more research is needed to fine tune target choice and stimulation setting definition.

Comparing response blocking and response interruption/redirection on levels of motor stereotypy: Effects of data analysis procedures.

Autism spectrum disorder (ASD) is marked by deficits in social communication and the presence of restrictive and/or repetitive behaviors or interests. Motor stereotypy is a form of repetitive behavior that is common in ASD. Response Interruption and Redirection (RIRD) and response blocking (RB) are two interventions found to be efficacious in reducing motor stereotypy. However, the current literature presents with inconsistencies regarding the relative efficacy of these two procedures. Thus, we sought to replicate and extend previous literature by evaluating the efficacy of both interventions on reducing motor stereotypy among 3 individuals with ASD. We also sought to evaluate how variations in data analysis affected the interpretation of treatment outcomes. Results indicated that both interventions were equally efficacious at reducing stereotypy when analyzing data exclusive of treatment-implementation time. However, when analyzing total session time data, RB produced greater and more sustained reductions in stereotypy across all participants. These results emphasize the importance of data analysis decision-making in evaluating intervention outcomes.

Using Mobile Technology to Reduce Engagement in Stereotypy: A Validation of Decision-Making Algorithms.

We developed an iOS app, the iSTIM, designed to support parents of children with autism spectrum disorders (ASD) in reducing common repetitive vocal and motor behavior (i.e., stereotypy). The purpose of our study was to preliminarily test the decision-making algorithms of the iSTIM using trained university students to implement the assessments and interventions. Specifically, we examined the effects of the iSTIM on stereotypy and functional engagement in 11 children with ASD within alternating treatment designs. Using the iSTIM reduced engagement in stereotypy for eight participants and increased functional engagement for four of those participants. Our results indicate that the iSTIM may decrease engagement in stereotypy but that some of the decision-making algorithms may benefit from modifications prior to testing with parents.

Nonautistic motor stereotypies: clinical features and longitudinal follow-up.

To characterize further the clinical features and long-term outcomes among children with motor stereotypies who do not manifest mental retardation or pervasive developmental disorders, a review of clinical records and semistructured telephone interviews were undertaken. The identified clinical cohort consisted of 100 typically developing children with motor stereotypies. The mean length of follow-up was 6.8 +/- 4.6 years. At most recent follow-up, movements had continued in 94% of the sample (62% for >5 years). Only six children reported complete cessation of movements, with four (3 of 4 with head nodding) doing so >1 year after their initial diagnosis. Thus the course of motor stereotypies, especially in children with arm/hand movements, appears chronic. Nearly half the children in this cohort exhibit other comorbidities, including attention-deficit-hyperactivity disorder (30%), tics (18%), and obsessive-compulsive behaviors/obsessive-compulsive disorder (10%). Twenty-five percent of children with motor stereotypies reported positive family histories of motor stereotypies, suggesting an underlying genetic abnormality. Finally, evidence is emerging that the clinical course of children who exhibit head nodding may differ from those whose motor stereotypy predominantly involves the hands and arms.