Movement Disorders

Signs and Symptoms of Movement Disorders

To offer some insight into what it is to experience a movement disorder, consider an event that we’ve all experienced at some point: in the twilight stages of sleep, just as we’re dozing off, our body twitches. Another analogy would involve muscle cramps or spasms. Many movement disorders involve excessive muscle tone, also known as spasticity or hypertonia, that can lead to spasms. Now imagine your entire body or some extremity experiencing similar episodes of muscle spasms, involuntary movement or spasticity for an extended duration, and there was nothing you could do to stop it!

• Chronic and severe arching of the back, also called posturing
• Uncontrollable twitching, tics, or muscle spasms
• Flailing or scissoring of extremities. A movement disorder may be limited to one part of the body or may impact all extremities
• Jerky or vibrating movements
• Screaming in pain from possible muscle spasms brought on by the possible extreme muscle tone or spasticity
• A child may curl their toes as a result of muscle spasms associated with the movement disorder

What Parents Need to Know

Most parents of children who develop cerebral palsy leave the hospital with an initial diagnosis of some type of brain injury, although the extent of the damage is unknown. Other parents may be sent home with a vague concern about low Apgar scores and little else. Symptoms such as lack of suck and lethargy may send them frustrated and worried back to the pediatrician again and again. Eventually, failure to meet development milestones will lead to a diagnosis of cerebral palsy. Most all children with cerebral palsy are diagnosed by age 3.

Diagnosis of a movement disorder within cerebral palsy can follow the same frustrating path. Because of a number of factors, movement disorders are often misdiagnosed.

• Seizures, for example, occur in 30-50% of children with cerebral palsy. In some cases, severe movement disorders can mimic the same exaggerated muscular involvement as a seizure, with similar posturing, spasticity, and uncontrollable movements. An infant or a non-verbal child will be unable to communicate what they are feeling or experiencing during these episodes, making it extremely difficult for a health care provider to diagnose the condition.
• The same spastic or uncontrollable movements can also be triggered by such things as earaches, acid reflux, and certain medications, all of which will need to be treated and ruled out. Even an attempt by a person with cerebral palsy to control a movement can trigger the types of spasms seen in a movement disorder.

For these reasons, a non-verbal child with a movement disorder can go undiagnosed for years.  It’s common for physicians with no experience in the special needs or movement disorders population to misdiagnose a movement disorder as a seizure. In the instance of severe movement disorders such as dystonia, it usually takes a neurologist or developmental pediatrician who specializes in special needs children to make the diagnosis. An EEG or extended EEG with video would be helpful in reaching a diagnosis of movement disorder. It can confirm or rule out seizure activity in relation to the behavior. To better assist your healthcare provider in understanding your child’s disorder, follow these steps:

• Keep a detailed journal of when and how often the abnormal movements occur
• Note how long the behavior lasted
• Note as many details of the behavior as possible, such as what limbs were involved and the severity of the abnormal movements. Were they extreme and violent or mild? Were the movements jerky, or did they resemble tremors? Did your child appear to experience pain with the abnormal movements?  Write down anything and everything that will provide as much information as possible for your child’s physician. You want to attempt to paint a picture for the physician using words. Be as descriptive as possible.
• If possible, videotape the episode. This will provide your health care provider with a clear picture of what your child is experiencing.
• Use a neurologist or pediatrician who specializes in children with special needs.
• Request an order for an EEG to rule out seizure disorders.
• Stay on top of the latest information. Aggressive research is being conducted throughout the world involving the treatment and prevention of movement disorders
• Be persistent.  Movement disorders can be managed with a team approach and the right medications. Some movement disorders, left untreated can severely compromise a child’s quality of life and negatively impact a child’s development.

Misconceptions

It’s a common stereotype and misconception that prompts people with no knowledge of cerebral palsy or movement disorders to associate movement disorders with cognitive impairment or learning disabilities. Depending on the severity and nature of the cerebral injury, not all individuals with movement disorders are developmentally delayed.

Types of Movement Disorders

Movement disorders many times involve either excessive or reduced levels of muscle tone and can include a variety of involuntary gross motor movements

• Hyperkinetic — Means excessive tone. Also known as spasticity or hypertonia
• Bradykinetic — Indicates reduced tone or activity, and is often referred to as hypotonia
• Clonus — A common movement disorder associated with cerebral palsy. Clonus involves a series of involuntary muscular contractions and relaxation of skeletal muscles.
• Dyskinesia — Involves a variety of abnormal involuntary movements, classified as Tremors, Chorea and Ballism, Myoclonus, Tics, and Dystonias.
• Myoclonus — There are many forms of myoclonus. It is characterized by rapid, muscular jerking movements and can be triggered by attempts at coordinated movement.
• Chorea — A state of excessive, spontaneous movements, irregularly timed, non-repetitive, randomly distributed and abrupt in character. It may vary in severity from restlessness with mild intermittent exaggeration of gesture and expression to a continuous flow of disabling, violent movements.
• Dystonia — A movement disorder that causes sustained muscle contractions and can result in abnormal involuntary muscle movements in the form of twisting, repetitive movements or posturing.

Medications and Treatments

• Benzodiazepines — A class of medications that act upon the central nervous system to reduce communication between certain neurons, lowering the level of activity in the brain. Benzodiazepines are muscle relaxants such as diazepam, oral baclofen, and dantrolene and are frequently the first line of treatment in managing movement disorders.
• Spinal Cord Stimulation — Developed in the 1980s to treat spinal cord injury and other neurological conditions involving motor neurons, this procedure involves an implanted electrode which selectively stimulates nerves at the base of the spinal cord to inhibit and decrease nerve activity. The effectiveness of spinal cord stimulation for the treatment of cerebral palsy has yet to be proven in clinical studies. It is considered only when other conservative or surgical treatments have been unsuccessful at relaxing muscles or relieving pain.

Surgical Intervention

• Selective Dorsal Rhizotomy (SDR) — A surgical procedure recommended only for cases of severe spasticity when all of the more conservative treatments have proven ineffective. In this procedure, surgeons locate and selectively severe over activated nerves at the base of the spinal cord.
• Intrathecal Baclofen — Approved by the FDA in 1996 for treatment of cerebral palsy, this therapy is becoming the common choice of treatment in movement disorders that do not respond well to other forms of treatment. This form of therapy uses an implanted pump device to deliver baclofen (muscle relaxant) into the intrathecal space of the spinal cord. Inthrathecal Baclofen is most appropriate for children with chronic, severe hypertonia and uncontrolled movement disorders throughout the body.

Research and Progress

Although there is aggressive research being conducted throughout the world, researchers agree there is a critical need for larger, multicenter, controlled clinical trials to fully evaluate treatments for pediatric movement disorders. In regards to treatment and management of pediatric movement disorders, developments in new pharmaceuticals have begun to provide hope for those cases in which a genetic diagnosis can be made. Advances in therapies such as deep brain stimulation, gene therapy, epigenetic modulation, and stem cell therapy hold promise for improving outcomes in both primary and secondary causes of movement disorders.

Your child’s treatment will depend on the type and severity of the movement disorder involved. Movement disorders left untreated can severely impact a child’s development and quality of life. Speak with your child’s physician and neurologist to decide the best course of treatment for your child.