Spasmodic Torticollis (ST) is a painful and debilitating neurological disorder. It is also known as "Cervical Dystonia," and many people also call it just "torticollis." Approximately 3 in every 10,000 people - about 6,000 people in the Australia - are likely to suffer from ST.
This movement disorder is caused by a dysfunction of the brain. The symptoms are caused by intermittent or sustained contractions of the muscles around the neck which control the position of the head. This causes the head to lean to one side, or be pulled forward or backward. The shoulders may also be uneven and some patients experience tremors in the head or arms. ST is usually accompanied by constant and extreme pain.
Although it's not related, ST can resemble other disorders including Parkinson's disease, epilepsy, muscular dystrophy and wry neck (an acute episode of pain and spasm in the neck that resolves itself in days or weeks.)
ST can limit a person's ability to function, but it's a localized disability which does not affect other body systems. Because it can be more severe during times of anxiety or stress, ST was once thought to be a psychiatric disorder. Research has shown, however, that while ST does indeed seem to originate in the brain, it is clearly a neurological disorder rather than a psychiatric one.
Signs and Symptoms
ST can occur any time in life, although the symptoms most frequently occur between 25 and 55 years of age. ST is more common in women than men. The exact cause is unknown. Onset sometimes comes after an injury to the head or neck, following an infection, or after taking certain medications. There also seems to be a genetic link, with 5 percent of patients reporting at least one relative with ST and 50 percent demonstrating a family history of tremors in the head or hands.
First symptoms generally occur slowly or intermittently and are usually noticed when a person tried to keep his or her head straight, for example, while driving. Family or friends are often the first to see the sign, asking questions like, “Why does your head look like it’s on crooked?” Within two to five years, progression of the disease reaches a plateau without further worsening. The pain associated with ST is almost always focused in one place, frequently the side of the neck or the back of the shoulders.
There are three distinct varieties of Spasmodic Torticollis: Tonic, in which the head turns to one side, Clonic, which involves the shaking of the head, and Mixed which involves both turning and shaking.
The turning of the head is generally considered to fall into one of four categories: Rotational, in which the head turns to one side or the other, Laterocollis in which the head is pulled toward the shoulder, Retrocollis in which the head is pulled to the back, or Anterocollis in which the head is pulled forward. In fact, most people’s ST is more complex, having a combination of “pulling” from among these four categories.
ST movements often disappear in sleep and may not reappear for a short time, any where between 10 minutes and 4 hours after waking. Because of this, many people find relief by taking breaks during the day to lie on their backs. Touching the opposite side of the face or chin may also cause spasms to cease temporarily.
Spontaneous recovery can be found in up to 20 percent of people within five years of the onset of symptoms, however this is more common in those whose symptoms begin before age 40 and/or who have a relatively mild case of ST.
There is no uniform treatment for ST, but oral medications are usually the first line of defense. Should these fail, the approved treatment of choice is Chemodenervation which involves Botulinum Toxin injections in the contracting muscles. Over the years, this has proven to be a safe and effective method of relieving pain and lessening spasms.
Surgery is not recommended as an initial defense but can be helpful for people who are unresponsive to other treatments. The approved procedure, known as Selective Denervation, is difficult to perform and sometimes does not provide relief. It should only be performed by a neurosurgeon with special expertise in this area. Surgically severing the muscles does not work.
Often treatment of ST is through a multidisciplinary approach combining medications, botulinum toxin injections, physical and occupational therapy, biofeedback and electrical stimulation.
Reproduced with kind permission of NSTA (USA) – www.torticollis.org
Copyright ASTA Victoria 2015