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Movement Disorders

Joanna Blackburn, MD

Joanna Blackburn is an Assistant Professor in Pediatrics at Northwestern Feinberg School of Medicine and an attending child neurologist at Ann & Robert H. Lurie Children’s Hospital of Chicago. She is fellowship trained pediatric movement disorders specialists and leads the pediatric movement disorders program and Lurie Children’s Hospital.  She has published several articles in the medical literature and has written book chapters on the topic of pediatric movement disorders.  In addition to her clinical practice, she is the program director for the child neurology residency program and is dedicated to medical education.

SUMMARY

Childhood movement disorders, taken together, are common.  It is difficult to estimate the number of children with movement disorders as statistics about the frequency of these disorders in children is limited.  The subspecialty field of pediatric movement disorders is expanding with both increased number of providers and increased knowledge about these disorders.  Once rare, pediatric movement disorders specialty clinics are becoming more common in academic medical centers.

A movement disorder is defined as one or more of the following:

  • Dysfunction in the target and velocity of an intended movement
  • Abnormal posturing
  • Presence of unintended excessive movement
  • Normal movements occurring at unintended or inappropriate times.

The causes of movement disorders in children are different than those in adults.  Despite the differences in causation, the classification of movement disorders in children is the same as in adults.  Movement disorders are classified based on the way they appear to the observer.  This is referred to as the phenomenology of the movement.  Movements disorders are divided into two major categories:

  1. Hyperkinetic movement disorders which are characterized by an excess of movement, such as: tics, stereotypies (all are in glossary except this word), dystonia, chorea, tremor and myoclonus
  2. Hypokinetic movement disorders which are characterized by a paucity or lack of movement.  The primary hypokinetic movement disorder is parkinsonism.

Hyperkinetic movement disorders are far more common than hypokinetic movement disorders in the pediatric population.

The main regions of the brain involved in movement disorders are the basal ganglia and the frontal cortex.  Normal movement requires a multitude of connections between these areas of the brain in addition to connections to the thalamus, cerebellum, spinal cord, nerves, and muscles.  Dysfunction in these exquisite networks leads to an array of abnormal involuntary movements.

The first step in the diagnosis of a movement disorder is to identify the type of abnormal involuntary movement that is present.  This is referred to as identifying the phenomenology.  To make this decision, the clinician relies on the description of the movement provided by the family, on the direct observation of the involuntary movement in clinic if possible and, when the movement is intermittent, on a home video recording of the involuntary movement.  Some movement disorders can be diagnosed by clinical observation alone.  Others require additional diagnostic testing including:

  • blood tests
  • genetic testing
  • cerebrospinal fluid analysis following a spinal tap (lumbar puncture)
  • imaging with magnetic resonance imaging (MRI) of the brain

The treatment of movement disorders depends on the type of movement disorder that is present and on the underlying cause.  Some movement disorders require no treatment at all.  Others are treated with various oral medications.  For some movement disorders, there are psychologically based interventions.  Most commonly, this involves cognitive behavior based therapy.  Less commonly, surgical options are available including the placement of intrathecal baclofen pumps (ITB) and deep brain stimulation (DBS).

DESCRIPTION

Movement disorders can be hyperkinetic (excess of movement) or hypokinetic (paucity or lack of movement).  Hyperkinetic movement disorders include tics, stereotypies, dystonia, chorea, tremor and myoclonus.  Hypokinetic movement disorders include parkinsonism.  Movement disorders can be the only presenting symptom in a child or they can occur in the setting of multiple neurologic and/or non-neurologic symptoms.  Movement disorders can occur in typically developing children or in children with developmental delay or developmental regression.  Children may have mixed movement disorders where more than one type of involuntary movement occurs in the same child.

Characteristic features of the involuntary movements help determine the phenomenology or type of involuntary movement.  In some instances, these features can also identify a cause for the movement disorder.  The first step is to classify the abnormal movement as hyperkinetic or hypokinetic.

The second is to identify whether the involuntary movement is normal or abnormal.  For example, an involuntary movement observed in an infant may be normal whereas the same involuntary movement observed in a teenager may be abnormal.  As brain maturation occurs, the spectrum of normal movements change.  Another example is a quick jerk while falling asleep may be normal but that same quick jerk while awake may be abnormal.

The third step used to classify a movement disorder is to identify the pattern of the involuntary movement.  It is crucial to determine whether the abnormal movement comes and goes, is repetitive, or is continuous.

The fourth step is to determine the time course of the movement disorder.  This involves establishing some of the following features:

  • Is the abnormal movement new or longstanding?
  • Did is start suddenly or did it have an insidious onset?
  • Has it been present since birth?
  • Has the movement been stable, slowly progressive, or rapidly progressive?
  • Has it changed over time?

These key pieces of information will help narrow down the possible causes of the movement disorder.  Additional helpful features to consider include factors that make the movement better or worse, an ability to suppress or control the movement, the presence or absence of the movement in sleep, and whether there are other family members with similar symptoms.  Finally, a complete examination, including a detailed neurologic examination, needs to be completed in every child with a movement disorder.  After the information is obtained from this detailed movement disorder history and examination, it may be possible to determine the type and cause of the movement disorder.  In some cases, additional testing will be indicated.

Movement disorders have a wide range of severity.  The severity is dependent upon the type and cause of the movement disorder.  On one end of the spectrum, movement disorders can be mild and go  unnoticed.  On the other end of the spectrum, movement disorders can be severe or even life threatening.  Many children with movement disorders fall somewhere in between these extremes.

TYPES OF MOVEMENT DISORDERS (SYMPTOMS)

Developmental and Benign Movement Disorders of Childhood

There are many abnormal movements that present in infancy and childhood that are not the sign of a serious underlying neurologic condition (Table 1).  In fact, most of these involuntary movements are thought to be related to normal brain development and resolve over time with no intervention.  While these movements often alarm parents and cause them to seek medical attention, it is important for them to be recognized.  Accurate recognition allows physicians to provide parental reassurance and to minimize unnecessary diagnostic testing.

One example is the jitteriness of newborns.  Jitteriness is the shaking of the body, limbs or chin that is commonly seen in newborns.  It is often triggered by a startle and can be stopped by gently flexing the infant’s limb.  This type of tremor can occur in an otherwise healthy infant but in some cases, jitteriness can be the sign of a more complicated medical condition.  In otherwise healthy infants, the jitteriness usually stops within the first few weeks of life.

Another example is shuddering attacks.  Shuddering episodes are quick periods of a rapid tremor of the head, shoulders and arms that resembles shivering.  The episodes only last seconds but can happen numerous times per day.  They can have anemotional trigger such as excitement or frustration.  These episodes stop on their own by early childhood and do not require treatment.


Tics

Tics are involuntary, repetitive, discrete, stereotyped movements or sounds.  Tics may be transient, recurrent, or chronic and are divided into motor tics and phonic or vocal tics.  Motor tics are tics that make movements.  Phonic or vocal tics are tics that make sounds.  Tics involve the face, head and shoulders but any body part or combination of body parts is possible.  Common motor tics include eye blinking, facial grimacing, and shoulder shrugging.  Common phonic tics include sniffing and throat clearing.  It is important to note that coprolalia (saying/shouting obscenities) is rare.  Tics are termed chronic when they have been present for more than a year.   Tourette syndrome is a specific subset of chronic tics where children have multiple motor tics and a phonic tic that have been present for more than one year.

Tics occur multiple times per day on most days.  They can wax and wane in frequency, intensity and type over time.  Some children, particularly younger children, are unaware of their tics.  Older children can often describe a premonitory urge which is a feeling they get before they have their tic.  Some descriptions of the urge are an itch, a tickle, a feeling of discomfort or anxiety which is temporarily relieved after the tic is completed.  Children can suppress their tics for a period but it is often uncomfortable to do so.  Tics tend to be worse during times of stress, anxiety, excitement, sleep deprivation, and illness.  They can also be worse in the setting of relaxation after a time of stress or with a change in routine such as at the start of the school year.  Tics tend to decrease during focused concentration.  Tics may persist in sleep.

Tics tend to begin in school-age children, peak around age 10-12, then decrease or disappear in adolescence or early adulthood.  Tics are associated with additional neuropsychiatric symptoms including:

  • attention deficit hyperactivity disorder (ADHD)
  • anxiety
  • obsessive compulsive disorder (OCD)
  • behavioral outbursts
  • mood disorders
  • learning disabilities

Sometimes these comorbidities are more disruptive to their day-to-day life than the tics themselves.

Stereotypies

Stereotypies are stereotyped, intermittent, rhythmic, repetitive, purposeless movements involving the head and upper body.  The key feature of stereotypies is the movements look the same each time they occur and remain the same over time.  Some examples include hand flapping and rocking.  Stereotypies can involve more complex movements including posturing and grimacing.  Stereotypies start before the age of three and, for many children, resolve by early childhood.  Some children continue to have stereotypies later in childhood or even into adulthood.

Stereotypies tend to occur during times of excitement or boredom and often occur in certain situations.  They can last for seconds to minutes and tend to occur multiple times per day.  A key feature of stereotypies is that they are distractible, which can distinguish them from more serious conditions.  Some older children describe that they enjoy their movements.  Many children appear to be unaware that the movements are happening unless it is pointed out to him or her.  Stereotypies can be seen in typically developing children with normal neurologic examinations.  They are also seen in children with neurodevelopmental disorders such as autism spectrum disorder.  It is important to note that the presence of stereotypies does not imply a diagnosis of autism spectrum disorder.

Dystonia

Dystonia is characterized by abnormal, sustained muscle contraction which leads to unusual twisting and repetitive movements of the body and limbs or abnormal postures.  Dystonia can be classified by the age of onset, by the underlying cause, and by the areas of the body affected.  Dystonia can involve:

  • one body part (focal dystonia),
  • multiple contiguous body parts (segmental dystonia),
  • half of the body (hemi-dystonia) or
  • the entire body (generalized dystonia).

Dystonia has several key characteristic features which help differentiate is from other types of abnormal involuntary movements.

  1. Dystonia can be absent when the child is completely at rest.
  2. Dystonia becomes worse with voluntary action such as when reaching for an object. This sometimes causes people to think the child is faking the symptoms.
  3. The severity of dystonia may fluctuate over time and may worsen with stress.
  4. Dystonia resolves during sleep.
  5. It can occur during certain voluntary movements but not with others. For example, dystonia may be present while walking yet disappear while running.
  6. It may be present while walking forwards but resolve while walking backwards.
  7. It may occur only with a single specific task. An example of this is ‘writer’s cramp’ where dystonia is only present while writing and with no other activities.  An additional example is a musician’s dystonia where dystonia is only present while playing their instrument.
  8. The presence of a ‘sensory tick’ which occurs when a certain action significantly decreases their dystonia. For example, touching the back of a twisted and postured hand, opening and relaxing the fingers, or touching the side of the cheek to relieve twisting of the neck (cervical dystonia).

Dystonia can be mild and cause minimal impact on the child’s day-to-day life or it can be more severe, making it difficult for a child to use their hands, to eat, to speak, or to walk.  Rarely, dystonia can be life-threatening when it becomes extreme.  This is called status dystonicus.  The severe dystonia can cause muscle breakdown which may lead to kidney failure.

Tremor

Tremor is characterized by a rhythmic oscillation (back and forth movement) about a central point.  There are two main categories of tremor:

  1. Rest tremor: A rest tremor occurs with the limb is relaxed and supported. A rest tremor lessens or resolves with voluntary movement.  Rest tremor is characteristic of Parkinson’s disease which is exceedingly rare in children.
  2. Action tremor: Action tremor is a tremor that occurs during voluntary movement. There are three main subtypes of an action tremor.
    1. Postural tremor occurs when the limb is stationary but working against gravity, such as when you hold your arms outstretched in front of you.
    2. Isometric tremor occurs when the limb is stationary but is exerting a force against an object.
    3. Kinetic tremor occurs while the limb is moving. If the limb is moving towards a target, it is called an intention tremor.

In addition to determining if a tremor occurs at rest or with action, it is important to determine if the tremor is present on one side of the body (unilateral) or on both sides of the body (bilateral), if it started suddenly or if it has been present for a long time, and to assess what makes the tremor better or worse.  All this information together will help determine the need for additional testing and come to a specific diagnosis.

Chorea

Chorea is a near continuous movement disorder that can be described as chaotic, irregular and unpredictable movements that have a writhing or dancelike quality.  They appear to flow from one part of the body to another.  Chorea can have a low amplitude or a high amplitude.  When chorea has a low amplitude, it can resemble restlessness or fidgeting.  When chorea has a high amplitude, the child can appear to have flailing or flinging movements.  Athetosis is a term used to describe the slow writhing movements of the hands.  Ballism is the term used to describe the large amplitude flailing movements.  Chorea can occur on one side of the body (hemi-chorea) or can involve the entire body (generalized chorea).   Chorea cannot be suppressed or controlled by the child.

Myoclonus

Myoclonus is characterized by sudden, brief, shock-like jerking movements.  Myoclonus can occur at rest or appear when the child attempts to move.  These movements are not suppressible and children do not know when the jerk is going to happen.  It can affect a single body part (focal myoclonus), multiple connected body parts (segmental myoclonus), multiple body parts (multi-focal myoclonus) or involve the whole body (generalized myoclonus).

Parkinsonism

Features of parkinsonism include:

  • slow movements (bradykinesia)
  • stiffness of muscles (rigidity)
  • decreased spontaneous movement (akinesia)
  • postural instability
  • a tremor that occurs when the muscles are relaxed and not in use (resting tremor)

Psychogenic Movement Disorder

Psychogenic movement disorders occur when a child or adolescent presents with symptoms resembling a movement disorder but does not have an identifiable neurologic cause.  Psychogenic movement disorders can resemble any of the abnormal involuntary movements detailed above, especially dystonia, chorea, tics, tremor and myoclonus.  Often when a child has a psychogenic movement disorder, the abnormal movement does not fit with the neurologically based movement disorders.  They may change location, vary in intensity, and be distractible.  Psychogenic movement disorders tend to have an abrupt onset followed by a static course. Additional features include a physical or psychosocial trigger, spontaneous remissions, and selective disability.  An example would be a child who after getting bullied at school develops a movement resembling a tremor that makes it impossible to do his school work and attend class, but does not interfere with his ability to play video games.

PHYSICAL ABNORMALITIES

In primary movement disorders, the abnormal involuntary movement is the only symptoms and the only abnormality on examination.  In secondary movement disorders, children have additional symptoms and abnormalities on examination.  These can include other neurologic abnormalities or non-neurologic abnormalities.

CAUSATION

There are many causes of pediatric movement disorders.  The possible causes of the movement disorder depend upon the phenomenology (type).  In general, movement disorders can be primary or secondary.

  • In a primary movement disorder, the abnormal involuntary movement is the only symptom and the only abnormal finding on examination.
  • In secondary movement disorders, the movement disorder is part of a condition with additional symptoms and abnormalities on examination.

The cause of a movement disorder varies based on the type of movement.  There are both genetic and acquired causes to consider.  Acquired causes include:

  • hypoxic brain injury
  • infections
  • post infectious/autoimmune
  • strokes
  • toxins
  • medications

Tics

Most children who have tics have a primary tic disorder.  Tics can occur in isolation or in associated with neuropsychiatric comorbidities.  These include ADHD, OCD, anxiety, mood disorders, behavioral outbursts, or learning disabilities.  Tics and their comorbidities are thought to be heritable but a specific genetic cause has not been identified.   Tics are suspected to have a multifactorial causation with both genetic and environmental factors.

Secondary tic disorders exist but are rare.  In these situations, children will have additional symptoms or findings to suggest the presence of a condition other than a primary tic disorder.  Rare secondary causes of tics include:

  • infections
  • drugs
  • toxins
  • chromosomal disorders
  • neurodegenerative disorders
  • head trauma

Stereotypies

Stereotypies are primary when they occur in typically developing children.  In these situations, they can be part of normal infantile development.  Stereotypies can also be secondary when they occur in the setting of a neurodevelopmental disorder such as autism spectrum disorder.  A definitive cause of stereotypies has not been determined.   Both a psychogenic and neuro-biologic basis of this movement disorder have been proposed.

Dystonia

Dystonia can be primary (where dystonia is the only or predominant symptom) or secondary (where dystonia is due to another identified cause).  Primary dystonia is typically caused by a single gene mutation.  An example of this is DYT-1 dystonia which is a generalized dystonia that can present in childhood.  In this disease, dystonia is the only symptom.  In DYT-1 dystonia, children typically present with dystonia in a limb that then spreads and can involve their entire body.

Secondary dystonia has varied causes.  Dystonia can be a symptom of cerebral palsy.  An example of how this occurs is in the setting of hypoxic-ischemic brain injury. The brain does not get enough oxygen and blood during delivery.  This causes an injury to the basal ganglia which are the primary structures of the brain that control movement.  Sometimes this injury can be seen on brain imaging with an MRI.  Dystonia has many other causes.  These include neurodegenerative diseases, brain infections, structural brain lesions, and medications.  Dopamine-blocking medications can cause a specific type of dystonia called an acute dystonic reaction.  In this case, dystonia presents abruptly after the medication is administered.        

Tremor

There are many causes of tremor.  On the least severe end of the spectrum, tremor can be physiologic and occur in otherwise healthy children.  When tremor is more notable than the average healthy child, it is called an enhanced physiologic tremor.  These tremors are exacerbated by stress and anxiety.  Tremor can be hereditary as in the case with essential tremor.  Children with essential tremor typically have an action tremor involving the hands.  In some children, the head, voice, trunk, or legs can also be involved.  Tremor can be caused by strokes or brain lesion which most commonly cause a unilateral tremor.   Tremor can be caused by metabolic derangements such as low calcium, endocrine disorders such as hyperthyroidism, neurodegenerative diseases, medications, or toxins.

Chorea

Chorea can be seen in normal, healthy infants until they are about 6 month of age.  After this age, chorea is typically pathologic.  Chorea is rarely a primary disorder.  Examples of rare causes of primary chorea include benign hereditary chorea and Huntington’s disease.  Huntington’s disease is rare in children and does not typically present with chorea in children.

Secondary chorea has numerous causes.  In these cases, chorea is accompanied by additional neurologic symptoms and non-neurologic symptoms.  Chorea can be acquired in childhood.  The most common cause is Sydenham’s chorea which is the result of a streptococcal infection.  On average 1-2 months after the infection, children develop chorea.  This is thought to happen when antibodies that formed to fight off the strep infection incorrectly attack and injure the basal ganglia.  Chorea can be caused by metabolic derangements such as hypoglycemia or hyperglycemia (low or high sugar), hyponatremia or hypernatremia (low or high sodium), hypocalcemia (low calcium) and hyperthyroidism.  Chorea can be seen after heart surgery when children have cardiopulmonary bypass (post-pump chorea).  Additional causes of chorea include infections, systemic lupus erythematosus, toxins, and neurodegenerative disorders.  Chorea can be chronic as seen in a subtype of cerebral palsy.  In this setting, chorea often co-exists with dystonia due to injury to the basal ganglia.

Myoclonus

Myoclonus can arise from multiple areas in the nervous system including the cortex, basal ganglia, brainstem, and spinal cord. In fact, myoclonus can be normal, as with sleep myoclonus.  Most people have experienced this phenomenon where the body jerks as they are falling asleep, sometimes waking the person up while they are dozing off.

There are numerous causes of pathologic myoclonus.  Epileptic myoclonus must be differentiated from non-epileptic myoclonus.  Sometimes this requires an electroencephalogram (EEG) to differentiate. Once myoclonus is confirmed as a movement disorder, the location and the appearance of the myoclonus can be helpful in determining the cause.  Myoclonus can be seen in numerous inherited genetic diseases.   It can also be acquired by exposure to toxins, infections, metabolic derangement, and hypoxic events.

Parkinsonism

Primary parkinsonism is typically caused be a single gene mutation.  An example of this is juvenile Parkinson’s disease where Parkinson’s disease starts before the age of 21.  Secondary parkinsonism has a wide variety of causes.  It can present as a symptom of a larger neurodegenerative disorder as in mitochondrial diseases.  It can be caused by a brain infection (encephalitis) or an autoimmune inflammatory process (autoimmune encephalitis).  Parkinsonism can also be an adverse reaction to a medication.  This can be seen with a class of medications called dopamine-blocking medications.

LABORATORY EVALUATION

In some cases, a history and physical examination is all that is needed to diagnosis a movement disorder.  For example, in an otherwise healthy 5-yold with eye blinking and sniffing, a tic disorder can be diagnosed without laboratory evaluation.  When additional diagnostic testing is indicated, it may include the following:

  • Blood testing for metabolism and/or genetic studies
  • Lumbar puncture for cerebrospinal fluid analysis
  • Magnetic resonance imaging (MRI) of the brain
  • Electroencephalogram (EEG) to distinguish from seizures

DIAGNOSTIC STANDARDS

The diagnosis of movement disorders is dependent on identifying the phenomenology (what the disorder looks like to the observer).  In some cases, the movement disorder can be observed during the clinic visits.  If the movement disorder is intermittent, the physician must rely on the description of the movement from the parents; this can be challenging.   Parents and children can also attempt to mimic the abnormal movement during the visit.  If the abnormal movement in questions is intermittent, home videos are very helpful in identifying the phenomenology.  In these cases, a short video from a cell phone may be all that is needed to arrive at the correct diagnosis.

Movement disorders may need to be distinguished from other conditions which lead to the appearance of abnormal movements.  The most common distinction that needs to be made is between a movement disorder and a seizure.  This can often be done by simple observation but in more challenging cases, may require addition diagnostic testing to make this important distinction.  The standard for diagnosis of a seizure disorder is an electroencephalogram (EEG).

In some cases, the identification of the type of movement disorder is all that is needed to come to a diagnosis.  For example, a primary tic disorder may be diagnosed on history, examination, and observation alone.  In other cases, additional diagnostic testing may be needed.  Additional testing may include blood and urine testing, imaging studies, and testing of the cerebrospinal fluid.  Diagnostic testing starts with attempting to identify the most common causes of the movement disorder as well as treatable forms of the movement disorder.  Increasingly, more extensive genetic testing has enhanced the ability to diagnose movement disorders as well as lead to the discovery on new genetic movement disorders diagnoses.

THERAPEUTIC INTERVENTION

The key to creating a treatment plan for pediatric movement disorders begins with the correct classification of the movement.  Some movement disorders are considered benign and do not require treatment.  Other movement disorders are disruptive to a child, impact their quality of life, and necessitate treatment.  Rarely, a pediatric movement disorder can be life-threatening and requires emergent treatment in a hospital.  With some movement disorders, the goal of treatment is to lessen the severity of the involuntary movement.  In other cases, the goal of treatment is to eliminate the abnormal involuntary movement.  Many movement disorders can be difficult to eliminate even with our current best treatments.  The benefit of the medication also must be weighed against the risk of potential side effects.  For example, a slight decrease in tics might not be worth the sedation caused by a medication used to reduce tics.  In cases of acquired movement disorders, it is important to treat the underlying cause.  Treatment of the underlying cause may eliminate the movement disorder and obviate the need to treat the movement disorder itself.

Tics

Regardless of treatment, one-third of children will outgrow tics in adolescence or early adulthood, one-third will have improvement of tics in adolescence or early adulthood, and one- third will have tics that continue to be disruptive in adulthood.  The decision to treat tics is based on whether the tics are bothersome or disruptive in day to day life.  Often counseling the family to show that the tics are not the sign of an underlying serious neurologic condition provides enough reassurance that medications are not pursued.  Providing information to teachers, classmates, and other family members can also reduce the stress caused by tics therefore minimizing the need for medication.

In older children, tics may be embarrassing or the children may be teased about their tics.  There are medications that can be used to treat tics.  These include alpha agonists (e.g. guanfacine), antiepileptic medications (e.g. topiramate), atypical neuroleptics (e.g. risperidone) or typical neuroleptics (e.g. haldol).  Medications decrease tics on average by one-third to one- half at best.  Medication choices can be based on whether the child has tics alone or tics in combination with comorbid conditions.  For example, if a child has tics and ADHD, the alpha agonists may be chosen as this medication can be helpful for both conditions.  In some children with tics, the associated psychiatric symptoms are more disruptive than the tics themselves.  For example, adequate treatment of ADHD may decrease stress at school and at home, secondarily decreasing tics.

An alternative to oral medication is a cognitive-based behavioral therapy training program called the comprehensive behavioral intervention for tics.  A key component of this behavioral approach is habit reversal therapy.  This process teaches tic awareness and the development of a competing response which is an action that replaces the current tic.  The child learns to stop the urge to have their tic with no movement at all.  The comprehensive behavioral intervention for tics has been shown to be as effective as medical treatment for tic reduction.

Stereotypies

Stereotypies do not require treatment unless the movements are bothersome to the child or are interfering with their day-to-day life.  They decrease or disappear in school age children without intervention.  If stereotypies are bothersome, especially when they persist in older children, the best treatment is cognitive behavioral therapy. Medications are not effective.

Dystonia

Dystonia can be treated with a variety of oral medications (trihexyphenidyl, carbidopa/levodopa, baclofen, benzodiazepines).  The effectiveness of these medications varies based on the underlying cause of the dystonia.  A notable specific type of dystonia is dopa-responsive dystonia.  These children have a dramatic and sustained response to low-dose levodopa.  Often, multiple medications are tried in children with dystonia to find the best response.

Botulinum toxin injections can be used to treat dystonia.  Botulinum toxin injections cause temporary weakness in the injected muscle.  The resulting weakness decreases the ability of the muscle to generate a dystonic contraction.  There are surgical options for a certain subset of patients with dystonia.  Surgical interventions include an intrathecal baclofen pump and deep brain stimulation (DBS).

Tremor

In some cases, tremor is not disruptive to day-to-day life and does not require treatment.  In more mild cases, occupational therapy and adjustments at school are enough to mitigate the effects of the tremor (having extra time in class to complete a written assignment if tremor impairs writing or having permission to type rather than write assignments).  If the tremor is disruptive, it can be treated with medications.  Beta blockers and primidone are the main medications used to treat tremor.  Clonazepam is also used with some success.  In cases of severe tremor, deep brain stimulation (DBS) has been effective in reducing and/or eliminating tremor.

Chorea

In some cases, chorea may be mild and self-limiting.  In these cases, treatment may not be necessary.  Chorea can be treated with oral medications.  Tetrabenazine is an oral medication that is approved for the treatment of Huntington’s chorea.  It can be used off-label for the treatment of chorea in children.  Haloperidol has also been shown to be effective in some children.  Certain anti-epileptic medications can be used to treat chorea.  These include carbamazepine and valproate.  Benzodiazepines can be helpful for some children with chorea.  Treatment of chorea may depend on the underlying cause or chorea.  For example, Sydenham’s chorea may be treated with oral or IV steroids as well as daily penicillin to prevent future streptococcal infections.

Myoclonus

Myoclonus is sometimes epileptic and sometimes a movement disorder. In both cases, the treatment of choice is anti-epileptic medication.  Some medications that have been used to treat myoclonus include valproate, carbamazepine, levetiracetam, and clonazepam.

PROGNOSIS

There is a wide range of outcomes for children with movement disorders.  It is dependent upon the type of movement disorder as well as the underlying cause of the movement disorder.  On the least severe end of the spectrum, movement disorders can be benign and self-limited.  Movement disorders may wax and wane and cause variable impairment in day–to-day life such as with tic disorders.  They may appear in infancy and remain unchanged over a lifetime as in cerebral palsy.  On the more severe end of the spectrum, movement disorders can cause significant disability affecting eye movements, ability to speak and swallow, and the ability to walk.  In some cases, symptoms can be progressive over time leading to other complications which limit life expectancy.  The movement disorder itself is rarely life threatening.

GLOSSARY

  • Akinesia – Loss or impairment of voluntary activity
  • Athetosis – Continuous, involuntary, slow, sinuous, writhing movements especially of the extremities
  • Attention deficit hyperactivity disorder – disorder including a combination of persistent problems such as difficulty sustaining attention, hyperactivity and impulsive behavior
  • Ballism – Abnormal swinging jerking movements
  • Bradykinesia – Extreme slowness of movement
  • Chorea – Involuntary spasmodic movements, especially of the face and limbs, with continuous, jerky or flowing ‘dancelike’ quality
  • Dyskinesia – Distortion or impairment of voluntary movement resulting in fragmented or jerky motions
  • Dystonia – Abnormal posture due to the co-contraction of agonist and antagonist muscle
  • Hyperkinetic – Excess of movement
  • Hypokinetic – Decrease of movement
  • Myoclonus – Rapid, lightning-like muscle jerk
  • Obsessive compulsive disorder – disorder of unwanted or repeated thought, feelings, ideas and behaviors that drive them to do something over and over.
  • Phenomenology – Appearance of an abnormal involuntary movement for classification of a movement disorder
  • Rigidity – Increase in muscle tone leading to stiffness of movement
  • Stereotypy – Involuntary repetitive or ritualistic movements or sounds that occur in the same fashion every time
  • Tics – Involuntary stereotyped repetitive movements or sounds
  • Tremor – Oscillatory movement about a fixed point

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