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Prisma Health Children's Hospital–Midlands

7 Richland Medical Park Dr.
Columbia, SC 29203

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Dystonia

Dystonia (an involuntary pattern of posture and movement) can affect many different parts of the body, and the symptoms are different depending upon the form of dystonia. A child with cerebral palsy may only have dystonia when they try to use the affected body part, but these affected muscles are relaxed in sleep.  Dystonia in children with cerebral palsy may be noticeable only after prolonged exertion, stress, or fatigue.   Dystonia can be associated with fixed postures and shortening of tendons.

Paroxysmal Autonomic Instability with Dystonia (PAID) is a condition that occurs after moderate to severe brain injury. There are intermittent periods of dystonia with changes in the autonomic system. The brain and autonomic system control heart rate, blood pressure, breathing rate, and temperature. Children with PAID have episodes high temperatures, increased heart and breathing rates, and changes in blood pressure accompanied by dystonia without any apparent cause.

Treatment

There are several treatment options that can ease some of the symptoms of dystonia based on each individual’s symptoms. In some cases, individuals are able to do “sensory tricks” to decrease the dystonia. These may include touching the affected part of the body or a nearby body part either before or during any movement known to trigger dystonia, stretching the affected limb or body part, or using splints and braces. Although this may not prevent or stop the dystonia, touching can distract or trick the brain and reduce the length and intensity of a muscle contraction. Massage and warmth may help relax tight muscles. Movement and exercise helps some people. Even moving in limited range,or having someone help move the child may be helpful. Yoga is a great choice if possible. Mindfulness and meditation helps many people, your child may be able to follow some simple techniques.

Pediatric physiatrists may use the below treatments in conjunction with therapies and bracing to maximize function.

  • Medications – Several classes of drugs that affect different neurotransmitters may be effective for various forms of dystonia. Commonly used ones in children with CP include diazepam and clonazepam. Other medications in PAID are used to stabilize blood pressures, heart rates, and manage triggers, including pain.
  • Botulinum toxin – Botulinum injections often are the most effective treatment for focal dystonias.  Injections of small amounts into affected muscles prevents muscle contractions and can provide temporary improvement in the abnormal postures and movements that characterize dystonia.

In rare cases:

  • Deep brain stimulation (DBS) – may be recommended for some individuals with dystonia, especially when medications do not sufficiently alleviate symptoms or the side effects are too severe. DBS involves surgically implanting small electrodes that are connected to a pulse generator into specific brain regions that control movement.  Controlled amounts of electricity are sent into the exact region of the brain that generates the dystonic symptoms and interfere with and block the electrical signals that cause the symptoms. This does not help PAID.
  • Other surgeries – aim to interrupt the pathways responsible for the abnormal movements at various levels of the nervous system. Some operations purposely damage small regions of the thalamus (thalamotomy), globus pallidus (pallidotomy), or other deep centers in the brain. Other surgeries include cutting nerves leading to the nerve roots deep in the neck close to the spinal cord (anterior cervical rhizotomy) or removing the nerves at the point they enter the contracting muscles (selective peripheral denervation).  Some patients report significant symptom reduction after surgery. This does not help PAID.

NIH: National Institute of Neurological Disorders and Stroke

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