Dystonia: A Neurological Movement Disorder Caused by Dystonia and Modern Treatment Options for People with Dystonia

The term dystonia (Greek, meaning “abnormal muscle tone”) refers to an organic disorder of the brain. It causes involuntary, sometimes spasmodic movements or abnormal postures in specific parts of the body or the entire body. In Germany, at least 32,000 people suffer from dystonia.

Dystonia is caused by malfunctions in specific networks of the brain. Medical professionals believe that a disturbance occurs in the basal ganglia, a deep structure within the brain. The activity of the nerve cells located there is abnormally elevated.

To make an accurate diagnosis, the treating neurologist must determine whether the patient has an idiopathic or symptomatic form of dystonia. This is of crucial importance for further treatment.

In the vast majority of cases, the underlying condition is idiopathic dystonia (i.e., with no identifiable cause), which is currently incurable. However, effective treatment options are available in many cases.

Symptomatic dystonia can be caused by

• medications,
• tumors,
• cerebral circulatory disorders, or
• metabolic disorders (e.g., Wilson’s disease)

. It requires treatment of the underlying condition.

In dystonia, abnormal muscle tone can lead to an abnormal posture of the entire body or individual body regions.

Continuous or intermittent muscle contractions result in a postural deformity of the affected body part (e.g., torticollis). This deformity is visible to others and often leads to social stigmatization of the affected person. In addition, these uncontrolled muscle contractions in dystonia are often accompanied by pain.

The treating neurologist conducts a thorough medical history review and a clinical examination. Various additional tests are also performed. Structural imaging of the brain should always be performed to rule out “symptomatic” dystonia.

Currently, there are three distinct treatment options for dystonia:

• Oral medication
• Botulinum toxin therapy
• Deep brain stimulation

Oral Medication Therapy for Dystonia

When prescribing medication, the severity and onset of the dystonia should be taken into account. A range of different medications is generally available for treating dystonia:

• L-dopa,
• anticholinergics,
• baclofen,
• benzodiazepines, and
• dopamine depleting agents such as reserpine and neuroleptics.

The response to oral medication is often unsatisfactory and then requires further therapeutic measures.

Botulinum Toxin Therapy for Dystonia

Botulinum toxin therapy has been used very successfully for several years to treat dystonia. It is now considered the treatment of choice in most cases of dystonia.

Botulinum toxin is a neurotoxin that is injected in low doses into the affected muscles. There, it causes a mild paralytic effect on muscle activity. This often results in a reduction of the symptoms caused by dystonia. This effect typically lasts 2 to 4 months.

However, with long-term treatment, “immunization”—that is, a diminishing of the therapeutic effect—may occur.

Deep Brain Stimulation for Dystonia

Sometimes, dystonia does not respond adequately to either medication or botulinum toxin therapy. For these cases, “deep brain stimulation” has been available as an effective treatment method since the late 1990s. Deep brain stimulation is also known as brain pacemaker therapy.

In this procedure, two electrodes are implanted into the overactive region of the brain. There, they suppress the pathological signals by emitting precisely controlled electrical impulses, acting as a sort of “jamming transmitter.”