Cervical Dystonia

Cervical Dystonia

Article
Ear, Nose, & Throat (ENT)
Brain & Nerve
+3
Contributed byKrish Tangella MD, MBAFeb 21, 2021

What are the other Names for this Condition? (Also known as/Synonyms)

  • Spasmodic Torticollis (ST)

What is Cervical Dystonia? (Definition/Background Information)

  • Cervical Dystonia (CD) is an abnormal neuromuscular disorder affecting the neck and head muscles. It is marked by painful muscular spasms that result in involuntary head movement to the sides, front, or back. It is also known as Spasmodic Torticollis
  • A majority of Cervical Dystonia occurs spontaneously, with no explanation as to why they arise. However, a few cases are thought to occur due to certain underlying neurological abnormalities or diseases
  • The muscular condition cannot be cured completely in most cases. But, medications, therapy, and exercises, may help manage the condition and create a measure of comfort for those with Cervical Dystonia

Who gets Cervical Dystonia? (Age and Sex Distribution)

  • Cervical Dystonia affects all individuals (children, adults) regardless of race or ethnicity. Though, young and old adults, in the 30-60 year age range are more prone to this disorder
  • The general incidence of this condition varies from around 1 in 3,500, to 1 in 17,000
  • A 2:1 male-female gender inequality is observed

What are the Risk Factors for Cervical Dystonia? (Predisposing Factors)

Risk factors for Cervical Dystonia include:

  • Genetic abnormalities; children born in families with a medical history of the condition have a higher risk
  • Individuals with inherited, early onset neurological movement disorder, called Generalized Dystonia are prone to Cervical Dystonia
  • It is thought that external traumatic injuries, which affect the head or neck; or repeated abuse/overuse of the muscles located at that region, could increase vulnerability to the disorder
  • Use of psychiatric medication and drugs used to control nausea, which increases incidence of the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Cervical Dystonia? (Etiology)

  • Cervical Dystonia is uncontrolled movement of the head due to impulsive contractions or shortening of the neck muscles. This causes the head to tilt, slant, or have jerky/spasmodic motions. The tilt can be either to the right or left side, or random (to any side)
  • It is speculated that genetic factors, underlying disorders, external environmental conditions (including an injury, or use of certain drugs), or a combination of these, could be responsible for Cervical Dystonia. However, these are still a subject matter of medical research
  • Most causes of neurological movement disorders (Cervical Dystonia) are unknown

What are the Signs and Symptoms of Cervical Dystonia?

Individuals with Cervical Dystonia may suffer muscular spasms in rapid bouts, for a short duration of time, or may periodically suffer sustained holding of a distorted posture. It could be mild or severe; and Cervical Dystonia may present the following signs and symptoms:

  • Jerky movements of the head that are abrupt and spontaneous; head may move randomly back and forth to any direction (even front or back)
  • Abnormal head posture retained for longer periods due to prolonged muscle contraction or distortion. The neck may be twisted, stretched, bent forward, or arched backward
  • Severe pain in the neck muscles that slowly affect the shoulders too
  • Exhaustion due to such an intense sporadic activity may cause an individual to feel incapacitated
  • If CD persist for many years (around 5 years), then the movement may spread to the shoulder muscles too

How is Cervical Dystonia Diagnosed?

A simple physical examination of the condition, with study of patient (and family) history and a neurological evaluation may be conclusive enough. However, in order to determine the extent and type of the condition, and the nature of muscles involved with Cervical Dystonia, certain diagnostic tests are performed:

  • MRI scans of the head and brain
  • Electromyography; test to measure nerve electrical activity
  • Ultrasound scan of the neck
  • Blood & urine analysis to check for any drug/medication that can possibly provoke the condition
  • Biopsy of muscle or nerve tissue (sometimes of the skin too)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Cervical Dystonia?

Complications of Cervical Dystonia are mainly linked to the extent of the condition (i.e. severely twisted neck situation). These include:

  • The condition spreads to affect the chest/shoulder muscles
  • Intense pain causing serious discomfort, disability, and depression
  • CD can continue for many years and they are prone to recur. Chances of remission of the condition is high

How is Cervical Dystonia Treated?

The treatment measures for Cervical Dystonia are basically meant to prevent worsening of the condition and address the symptoms. The interventions could be surgical or non-surgical. Surgical methods are mainly used if the condition is classified as severe by the physician, and do not improve even after physical therapy. Non-surgical procedures form the usual line of treatment. The management measures include:

  • Use of medications to control the involuntary muscular movements (such as those used for Parkinson’s disease), muscle relaxants, and drugs to relieve pain
  • Botox injections (administered on the neck) are used to improve the muscle condition and strengthen them
  • Physical therapy is helpful in gently increasing the range of motion. It also prevents further muscle contracture and loss of muscle function. Using suitable stretching and musculoskeletal strengthening exercise program training, to control muscle fatigue and stress, can help decrease the burden of symptom
  • Use of special neck collars, neck braces
  • Application of warm compress, or heat
  • Use of surgical techniques like; deep brain simulation (providing electrical impulses to the brain directly, by drilling a tiny hole in the skull), or selective denervation surgery (cutting certain muscles and nerves that cause the specific condition). This is then followed by post-surgical physiotherapy

How can Cervical Dystonia be Prevented?

  • Cervical Dystonia is a neuromuscular disorder affecting the head and neck area that cannot be prevented

What is the Prognosis of Cervical Dystonia? (Outcomes/Resolutions)

  • Cervical Dystonia cannot be cured completely in most cases. But, medications, therapy, and exercises, may help manage the condition and create a measure of comfort for those suffering from the muscular condition
  • Cervical Dystonia can be treated to a near complete recovery in some cases, and sometimes they automatically disappear (though this is infrequent). But, with such a complete recovery, chances are that they could return (as observed 10-20% of the time)
  • The severity of the condition gradually builds-up over many months or years, and then slowly stabilizes. In some rare cases, it might spread to other regions too

Additional and Relevant Useful Information for Cervical Dystonia:

  • There are voluntary organizations and support groups that provide help, encouragement, and understanding, to the people affected by Cervical Dystonia.
  • Those with severe forms of CD have to live with a constant pressure of handling their daily personal, professional, and social life. This can cause deep emotional and psychological distress
  • To relieve severe pain and stress, alternative medicines are sometimes explored
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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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