Course:KIN366/ConceptLibrary/HyperkineticDisorder

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Movement Experiences for Children
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KIN 366
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Instructor: Dr. Shannon S.D. Bredin
Email: shannon.bredin@ubc.ca
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Movement disorders in children can vary in their symptoms, etiologies, and degrees of expression (Sanger, 2012). Difficulties can often arise in the identification of the many different types of movement disorders as many different disorders can arise from the same cause, or many different etiologies can cause the same disorder (Sanger, 2012). Therefore, due to the diverse yet overlapping nature of movement disorders, a thorough understanding of the various causes and symptoms can lead to earlier diagnosis and more effective clinical treatment for children (Sanger, 2012).

The majority of the movement disorders in children are generally classified based off of one of the three following classes: negative signs, hypertonic disorders, and hyperkinetic disorders (Sanger, 2012). Negative signs are movement disorders where there is insufficient muscle activity and/or control of voluntary muscle activity (Sanger, 2012). Hypertonic movement disorders are movement disorders where there is an increased resistance to movement around a joint, when the movement is externally forced (Sanger, 2012). Finally, hyperkinetic disorders are movement disorders that are primarily characterized by excessive involuntary movements (Sanger, 2012).

Hyperkinetic movement disorders fall under the spectrum of attention deficit hyperactivity disorders ( ADHD ) (Riley, 2012). Hyperkinetic disorders can often be further sub-divided into two different divisions of excessive involuntary movements (Sanger, 2012). The first division of excessive involuntary movements is the involuntary initiation of movement (Sanger, 2012). The second category is the addition of involuntary muscle contractions and/or movements to voluntary motor actions or posture (Sanger, 2012). The two divisions of excessive involuntary movements are unique to hyperkinetic disorders, which could be helpful to distinguish hyperkinetic disorders from other similar movement disorders (Sanger, 2012). The clear and thorough understanding of the various causes, symptoms, and defining characteristics is essential for the accurate classification, diagnosis, and treatment of hyperkinetic disorders in children (Sanger, 2012).

Influence on a Child's Life

Hyperkinetic disorder can affect many different areas of a child’s life. For example, hyperkinetic disorder can negatively influence a child’s confidence, behaviour, their ability to make relationships, and their performance in motor-driven activities like sports (Riley, 2012; Laufer & Denhoff, 1957). Although children with hyperkinetic disorder exhibit normal intelligence for their age group, the hyperactivity associated with hyperkinetic disorder can also negatively influence a child’s performance in school (Rosenthal & Allen, 1978). A child’s difficulty in school often arises from interruptions in their learning, which can be caused by a short attention span, low frustration tolerance, and an aggressive and/or impulsive behavior (Rosenthal & Allen, 1978). As a result, relationships can be hard for children to form and maintain due to the behavioural symptoms associated with hyperkinetic disorders (Rosenthal & Allen, 1978). In addition to the behavioural symptoms, children suffering from hyperkinetic disorders may also find it difficult to establish friendships as participation in normal childhood activities is often difficult due to the uncontrollable nature of their body movements (Rosenthal & Allen, 1978). As a result, children with hyperkinetic disorder often are limited in terms of their potential relationship pools and the types of opportunities (learning, motor, and/or social) that the external environment permits (Harvey et al., 2007)

Another important factor to take note of is that the difficulty in controlling voluntary movements can also impair a child’s long-term motor development (Harvey et al., 2007). For example, children that have persistent and/or chronic hand tremors often find it difficult to grip, throw, or catch objects, which can impair the development of hand-eye coordination, and the control and strength of their upper limb muscles (Harvey et al., 2007). It can also limit the degree of participation in sports that rely heavily on steady hand control and fine motor coordination (i.e. baseball, golf, bowling, etc.), therefore reducing the number of opportunities that are available to improve motor-deficits (Harvey et al., 2007). The above noted symptoms, and the associated negative influences on a child’s life, can persist all the way into adulthood if untreated, thus it can potentially lead to life-long learning and motor deficits in a child (Rosenthal & Allen, 1978).

Classification of Hyperkinetic Movement Disorders

Due to the existence of multiple different movement disorders that are characterized by involuntary excessive movement in children, it is often difficult to discriminate amongst them (Sanger, 2012). In order for a child to be diagnosed with hyperkinetic impulse disorder, they will often need to exhibit most (if not all) of the following essential symptoms: hyperactivity, restlessness, short attention span and/or inability to concentrate, impulsiveness, inability to delay gratification, irritability, and explosiveness (Riley, 2012; Laufer & Denhoff, 1957). In particular, hyperactivity is most commonly used as one of the identifying symptoms of hyperkinetic disorder (Sanger, 2012; Taylor et al., 2004; Taylor et al., 1998; Laufer & Denhoff, 1957). Hyperactivity is described as the involuntary and constant over-activity of gross motor function (Rosenthal & Allen, 1978; Laufer & Denhoff, 1957). Hyperactivity can often be identified in infancy by instances of advanced motor development (Laufer & Denhoff, 1957). Historically, the most common identifier for hyperactivity was a child climbing out of the crib well before the age of one (Laufer & Denhoff, 1957). However, hyperactivity may not be noticeable until later in life, usually anywhere up to 6-7 years of age (Taylor et al., 2004, Taylor et al., 1998; Laufer & Denhoff, 1957).

It is important to note that the degrees of expression of these symptoms are highly variable and can vary tremendously from child to child (Laufer & Denhoff, 1957). It is also important to note that all children display some of the symptoms of hyperkinetic disorder and ADHD to a certain degree, but in order to fit the criteria of a hyperkinetic disorder, children must display the symptoms for a period of at least six months and the symptoms must be present in at least two different environments (i.e. home and school) (Riley, 2012).

In order to classify the type of hyperkinetic movement disorder a child may have, it is useful to identify the nature of the movement (i.e. dystonia, chorea, tremor, etc.), when the movement usually occurs (i.e. at rest, with posture, with all voluntary movements, with particular voluntary movements, etc.), and what body parts are affected (Sanger, 2012). The clinical course of treatment is highly dependent on the type of hyperkinetic disorder and the etiology; therefore, proper classification of the movement disorder is essential for effective treatment (Sanger, 2012).

Types of Hyperkinetic Movement Disorders

Hyperkinetic disorders describe a broad class of movement disorders (Rosenthal & Allen, 1978). All hyperkinetic disorders are more or less similar in the sense that they cause the voluntary skeletal muscles to produce excessive and uncontrollable movements (Sanger, 2012; Rosenthal & Allen, 1978). However, the various types of hyperkinetic disorders can vary tremendously in their symptoms, their treatment, and the overall affect on the quality of life and movement opportunities for a child (Sanger, 2012; Rosenthal & Allen, 1978; Laufer & Dennhoff, 1957). An example of a specific hyperkinetic disorder that affects children is chorea (Sanger, 2012).

Chorea

Chorea is an example of a hyperkinetic disorder that is seen in children (Sanger, 2012). Chorea is classified as variable and unpredictable movements that involve multiple muscle groups (Sanger, 2012). Chorea usually occurs when the skeletal muscles of the body are at rest (Sanger, 2012). As a result of the movements occurring at rest, the child appears to have a restless or fidgety quality (Sanger, 2012). For example, children with chorea often have difficulty sitting still in chairs due to their inability to hold a static position for any length of time (Sanger, 2012). In addition, chorea is often associated with motor impersistence, which makes it difficult for children to hold a steady grip for more than a few seconds (Sanger, 2012). The inability to hold a steady grip for more than a few seconds can impair a child’s fine motor-skill development, and can limit the types of motor activities a child can perform (Sanger, 2012). However, mild to moderate forms of chorea do not often impair balance or ambulation in children; therefore, children with chorea may still be able to walk stably from destination to destination (Sanger, 2012).

Etiology

Chorea is essentially caused by an imbalance in the indirect and direct circuits of the basal ganglia (Barton & Shannon, 2010). The indirect and direct circuits of the basal ganglia normally work together to prevent unwanted muscle contractions from out-competing wanted muscle contractions, thus helping to coordinate efficient voluntary movements (Barton & Shannon, 2010). The imbalance in the basal ganglia circuitry is associated with either under-activity of the indirect pathway or excess activity of the direct pathway (Barton & Shannon, 2010). As a result of reduced inhibitory output from the indirect pathway, the subthalamic nucleus is excessively inhibited by the globus pallidus externa (Barton & Shannon, 2010). In result, there will be an increase in extraneuous movements due to a lack of excitatory outflow from the subthalamic nucleus inhibiting the thalamus (Barton & Shannon, 2010). Alternatively, excess activity of the direct pathway will also increase thalamocortical motor drive due to increased excitation of the globus pallidus interna/substantia nigra and subsequently, the thalamus (Barton & Shannon, 2010).

The most common form of Chorea in children is Sydenham’s chorea (Sanger, 2012). Sydenham’s chorea is due to an autoimmune response in children to the antigens in the basal ganglia, which is often due to the cross-reactivity to streptococcal antigens (Sanger, 2012). As a result, there is an abnormal production of anti-basal ganglia antibodies in the cerebrospinal fluid (Sanger, 2012). Since the basal ganglia is involved in the smooth coordination of voluntary movements, impairing basal ganglia function can result in lack of coordination or control over skeletal muscle movements (Barton & Shannon, 2010). The most common movement resulting from Sydenham’s chorea is the “piano playing” chorea, which involves the involuntary and unpredictable movement of the fingers of both hands (Sanger, 2012).

In addition to Sydenham’s chorea, severe but transient chorea is often seen following an episode of acute encephalitis (Sanger, 2012). In the case of chorea being a side effect of an encephalitic episode, the basal ganglia is usually without apparent injury (Sanger, 2012). Instead, the injury is usually confined to the cerebral cortex and the subcortical white matter, which suggests that the chorea is either sourced from the cerebral cortex or caused by a lack of inputs from the striatum and sub-thalamic nucleus to the basal ganglia, thus impairing normal basal ganglia function (Sanger, 2012).

Practical Applications and Treatment

What Can Parents Do?

Preventative Applications

For children suffering from hyperkinetic disorder, the impairment of their ability to focus and control their attention skills and motor behaviour often can stem from disrupted early experiences caused by a lack of attachment (Haddad & Garralda, 1992). Attachment is often defined as the biological tendency for a child to behave in ways that promote contact with their mother, or a mother figure (Haddad & Garralda, 1992). In addition, motor activity and exploration are often intrinsic factors of the attachment process (Haddad & Garralda, 1992). The child often uses the mother (or mother figure) as a point of security from where they gradually explore their environment to more distant targets (Haddad & Garralda, 1992). Development of the attachment process occurs during the first year of life, therefore adequate availability of the mother, or primary “mother-like” figure, during the first year of a child’s life is critical for appropriate development of search and exploratory behaviours (Haddad & Garralda, 1992). Deprivation can result in deep psychological injury, which often leads to the development of hyperkinetic disorder-like behaviours later in a child’s life (Haddad & Garralda, 1992). In addition, deprivation can also result in impaired development of sensory-motor competences that are promoted by exploration and search behaviours (Haddad & Garralda, 1992). Due to the strong link between deprivation and the development of hyperkinetic disorder, preventative tips for parents would be ensuring that parents provide sufficient time to allow for completion of the attachment process, and to have a safe environment for children to explore and play (Haddad & Garralda, 1992).

Motor-Support Applications

In addition to preventative treatments, parents can also help their children with the development of their locomotor and object control skills by providing them with opportunities to participate in physical activity (Harvey et al., 2007). The lack of physical skills in children with mild cases of hyperkinetic disorders, and other movement disorders, often stems from minimal accessible movement opportunities due to environmental and situational demands that restrict participation (Harvey et al., 2007). In order to promote motor proficiency and control, parents should provide accessible alternatives so their children can participate in as many physical activities as possible (Harvey et al., 2007). By motivating children to experience play and activity in a way like other children of their age and gender, they can learn basic motor skills that can translate to better locomotor proficiency later in life (Harvey et al., 2007). A few examples of locomotor skills that parents should focus on developing throughout their child’s life include the following: running, galloping, hopping, leaping, horizontal jumping, sliding, striking a stationary ball, stationary dribble of a ball, catching, kicking overhand throwing, and underhand rolling (Harvey et al., 2007). Overall, the most important tip for parents is to help provide their children with the same movement and play opportunities as other children of their age to prevent their mental, social, sensory, and motor development being hindered due to the limitations forced by environmental and structural demands (Harvey et al., 2007).

What Can Doctors Do?

Effective management of hyperkinetic movements disorders demands that physicians, and other health care professionals, know about all pharmacological and surgical treatments that are available (Jankovic, 2009). In many cases, treating the etiological source of the hyperkinetic disorder can be curative; however, most forms of treatment that are available tend to treat the symptoms of a hyperkinetic disorder rather than the underlying etiology (Sanger, 2012). It is important to note that symptomatic treatment of a hyperkinetic disorder may not eliminate symptoms in their entirety, but can significantly reduce them (Sanger, 2012). Significant reductions in the symptoms associated with hyperkinetic disorders can ultimately lead to improvements in function, motor ability, and the quality of life of affected children (Sanger, 2012). Therefore, physicians should opt to provide pharmacological and surgical symptomatic treatment options to children with hyperkinetic disorders in circumstances, where the hyperkinetic disorders are significantly disabling (Sanger, 2012; Barton & Shannon, 2010). If the hyperkinetic disorders are not particularly disabling, physicians should recommend non-pharmacological treatment options that implement multi-disciplinary support (i.e. physical therapy, occupational therapy, patient/caregiver education) (Barton & Shannon, 2010). Since treatment of all the various hyperkinetic disorders can differ greatly, a more detailed example of some treatment options for only chorea is listed below.

Treatment of Chorea

Medical Therapy Options

When treating chorea, the general approach to treatment should be the following: careful evaluation of the medical history of the child with a physical examination, introduce symptomatic treatment options if the chorea and associated symptoms are disabling to a child, consider surgery if the chorea is very severe, and implement multidisciplinary and supportive non-pharmacological treatment options throughout each step of the treatment process (Barton & Shannon, 2010). When doing the first step of carefully evaluating the medical history and performing a physical examination, it is important for a physician to identify possible underlying, reversible, and/or treatable symptoms (Barton & Shannon, 2010). For example, a physician would want to remove any drugs that cause chorea if a child is currently taking them, provide anti-infective treatments if the chorea is due to an infection, or provide immunomodulatory treatment if the chorea is due to an autoimmune disease (Barton & Shannon, 2010). Some notable treatable factors that a physician should investigate include vitamin B12 levels, thyroid function, drug investigations, and prior or current infections (Barton & Shannon, 2010).

When considering pharmacological treatment of chorea, it is often useful to identify the underlying etiology, and then treat the associated symptoms. If the cause of chorea is due to injury or damage to the basal ganglia, the first choice of treatment is treatment with low dosages of atypical neuroleptics (Sanger, 2012). Atypical neuroleptics are a form of antipsychotic medications, which can promote the development of more control in psychomotor activities (Sanger, 2012). Some examples of atypical neuroleptics include risperidone, olanzapine, or quetiapine (Sanger, 2012). The use of atypical neuroleptics also seems to reduce the risk of extrapyramidal disorders that could result from the administration of medication (Sanger, 2012). On the other hand, if the chorea is due to cortical injury and the basal ganglia is relatively intact, the use of low dosages of valproic acid and clonazepam are often more effective in treating symptoms than neuroleptics (Sanger, 2012).

Some other general classes of drugs that are used in the pharmacological treatment of chorea include: dopamine receptor antagonists, dopaminergic drugs, NMDA receptor antagonists/anti-glutamatergic drugs, antiepileptic drugs, and disease-modifying/neuroprotective agents (Barton & Shannon, 2010). In terms of dopamine receptor antagonists, both atypical and typical antipsychotics are used (Barton & Shannon, 2010). Typical and atypical antipsychotics block dopamine from acting on their receptors by acting as antagonists (Barton & Shannon, 2010). By preventing dopamine from acting on their receptors, one can reduce the tendency of extraneous, uncontrollable movements (Barton & Shannon, 2010). In addition, the use of antipsychotics can treat neurological symptoms associated with neurodegenerative chorea, such as outbursts and irritability, which can improve the outcome of interaction experiences for a child (Barton & Shannon, 2010). An example of a typical antipsychotic that is commonly used is tetrabenazine (Barton & Shannon, 2010). Some side effects from tetrabenazine administration include: depression, parkinsonism (~30% probability), sedation, suicidal ideation, orthostatic hypotension, akathisia, anxiety, constipation, and neuroleptic malignant syndrome (Barton & Shannon, 2010). An example of an atypical antipsychotic that has shown promising efficacy in treating chorea is olanzapine (Barton & Shannon, 2010). However, some side effects from olanzapine administration include acute dystonic reactions, parkinsonism, sedation, tardive dyskinesia, prolonged Q-T interval, hyperglycemia, and fatigue (Barton & Shannon, 2010).

Surgical Therapy Options

Although surgical intervention has been effective in treating other forms of hyperkinetic disorder, surgery has not been effective in improving overall motor function and quality in life in patients suffering with chorea (Jankovic, 2009).

References

Barton, B & Shannon, K. M. (2010). Treatment of Chorea. The Differential Diagnosis of Chorea. New York: Oxford.

Haddad, P. M., & Garralda, M. E. (1992). Hyperkinetic syndrome and disruptive early experiences. The British Journal of Psychiatry. 161: 700-703.

Harvey, W. J., Reid, G., Grizenko, N., Mbekou, V., Ter-Stepanian, M., & Joober, R. (2007). Fundamental Movement Skills and Children with Attention-Deficit Hyperactivity Disorder: Peer Comparisons and Stimulant Effects. Journal of Abnormal Child Psychology. 35: 871-882.

Jankovic, J. (2009). Treatment of Hyperkinetic Movement Disorders. Lancet Neural. 8: 844-856.

Laufer, M. W., & Denhoff, E. (1957). Hyperkinetic Behavior Syndrome in Children. The Journal of Pediatrics. 50: 463-475. 22.

Riley, J. (2012). Attention Deficit Hyperactivity Disorder and Attention Deficit Disorder (ADHD and ADD; Hyperkinetic Syndrome; Hyperkinetic Impulse Disorder). Psychiatry. Retrieved from: http://psych.med.nyu.edu/patient-care/conditions-we-treat/hyperkinetic-syndrome.

Rosenthal, R. H., & Allen, T. W. (1978). An Examination of Attention, Arousal, and Learning Dysfunctions of Hyperkinetic Children. Psychological Bulletin. 85, 4: 689-715.

Sanger, T. D. (2012). Hyperkinetic Disorders in Children. Current Clinical Neurology. New York: Springer.

Taylor, E., Dopfner, M., Sergeant, J., Asherson, P., Banaschewski, T., Buitelaar, J., Coghill, D., Danckaerts, M., Rothenberger, A., Sonuga-Barke, E., Steinhausen, H. C., & Zuddas, A. (2004). European clinical guidelines for hyperkinetic disorder – first upgrade. European Child Adolescent Psychiatry. 13: 1/7 – 1/30.

Taylor, E., Sergeant, J., Doepfner, M., Gunning, B., Overmeyer, S., Mobius, H. J., & Eisert, H. G. (1998). Clinical Guidelines for Hyperkinetic Disorder. European Child & Adolescent Psychiatry. 7:184-200.