Dystonia

DYSTONIA

Dystonia is a very complex, highly variable neurological movement disorder characterized by involuntary muscle contractions or spasm involuntarily. The involuntary muscle contractions cause twisting, repetitive and patterned movements as well as abnormal postures.

It is a condition that knows no age, ethnic or racial boundaries – it can affect young children to older adults of all races and ethnicities.

Dystonia affects men, women and children of all ages and backgrounds. It can develop in childhood and is often particularly disabling for children. Dystonia may be genetic or caused by factors such as physical trauma, exposure to certain medications, or other neurological conditions.

Deep brain stimulation (DBS) is a surgical procedure in which two thin, insulated electrodes are inserted into the brain. These electrodes are then connected by a wire under the skin to a battery usually implanted in the chest or in the abdomen. The battery operates similarly to a pacemaker delivering targeted electrical pulses that block the signals that cause the symptoms of dystonia.

The carefully controlled, minute electrical currents delivered through the electrodes on both sides of the brain can have a beneficial effect on the involuntary muscle contractions caused by dystonia. As a result, the symptoms of dystonia such as abnormal movements and postures and/or dystonic tremor can be eased. In addition, DBS can reduce the pain caused by dystonia.

Deep brain stimulation works most effectively for people who have an inherited (genetic) form of dystonia or for those where the dystonia has no identified cause (this is called idiopathic). The treatment is provided for those with inherited or idiopathic dystonia who have a severe generalized dystonia, neck (cervical) dystonia or dystonic tremor when other treatment options (such as botulinum toxin injections and oral medications) have failed to provide adequate relief.

The DBS system can be activated 1–2 days or a few weeks after surgery. The stimulator will start to reduce symptoms a few days after activation, but the improvement is usually gradual and may take several months to reach its full extent. During this time, the healthcare professional who programs the device will go through several programming sessions adjusting the settings of the device to achieve optimal results. The wait for the stimulator to be fully effective can be a time of anxiety so it is important to be aware from the outset that patience is required.

 

Deep brain stimulation (DBS)

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