Pediatric dystonia can cause twisting, often painful muscle contractions and abnormal postures. Specific actions, such as writing or walking, can act as a trigger and muscles often have a hard time relaxing once the contractions start.
What is Pediatric Dystonia?
Dystonia is a movement disorder that causes muscles to be too active. This can look like ongoing twisting movements. When muscles can’t relax, it leads to cramping. In dystonia, abnormal brain signals trigger a push-and-pull motion that causes muscles to twist painfully in opposition to each other.
Dystonia causes the body to awkwardly contort in response to voluntary movements, such as walking, resting, writing or talking. Movements can start in one area of the body, like the hand, and spread to other parts of the body. Dystonia can be diagnosed at any age, but is most common from preschool age and older.
What are the different types of Pediatric Dystonia?
There are two main categories of dystonia:
Primary dystonia is a genetic (inherited) disorder that occurs without a known brain injury.
Secondary dystonia is the most common form in children and is a result of a brain injury, such a cerebral palsy
Whether primary or secondary, dystonia impacts a number of bodily functions and locations, as described below:
- Cervical – neck
- Cranial – head and neck
- Focal – one specific location
- Generalized – most or all of the body
- Hemidystonia – arm and leg on the same side
- Multifocal – multiple locations
- Oromandibular – jaw, lips and tongue (speech and swallowing)
- Paroxysmal – sudden onset, usually temporary, locations vary
- Segmental – affects body parts that are next to each other
- Spasmodic dysphonia – throat muscles (speech)
- Tardive – usually the result of a medication exposure, face, neck, torso, and limbs can be affected
- Writer’s cramp – hand and forearm
What are the signs and symptoms of Pediatric Dystonia?
The main symptom of dystonia is muscle overactivation, which is the spread of muscle contraction from the desired muscle to the surrounding muscles, and the push and pull of muscles at the same time.
Symptoms are related to the area of the body that is affected. Some common areas are the leg, hand, tongue and lips. Dystonia is usually triggered by a specific voluntary movement, such as writing, walking or speaking. Patients may feel that their body part is pulling in an abnormal direction or that their body part is stuck in an abnormal position.
How is Pediatric Dystonia diagnosed?
Testing used to diagnose dystonia includes:
- Blood tests
- Electromyography (EMG) (measures how a muscle responds after a nerve stimulates a muscle)
- Genetic testing
- Magnetic resonance imaging (MRI) (imaging test that produces a detailed, 3-D image of the body using a magnetic field)
What are the causes of Pediatric Dystonia?
Dystonia can occur due to several possible triggers, including:
- Chemical or heavy metal poisoning (such as carbon monoxide or lead)
- Damage to the basal ganglia (deep structures within the brain that are primarily responsible for motor control)
- Head trauma
- Hypoxia (lack of oxygen to the brain), usually due to injury at birth
- Side effect of medication
- Brain tumor
How is Pediatric Dystonia treated?
Doctors can treat pediatric dystonia using the following:
- Botox – Therapeutic botulinum toxin can be used to relax muscles.
- Bracing and splinting – This can be used to support weak or stiff body parts or guard against joint deformity.
- Deep brain stimulation – With DBS therapy, surgeons implant a small device under the skin in the chest called a neurostimulator. This sends impulses to electrodes placed in the parts of the brain responsible for involuntary movements to help control them.
- Medications – A variety of oral medications can be used to treat dystonia.
- Physical and occupational therapy – These can be used to maintain the full range of motion in the affected body part and prevent muscles from getting rigid.