Cervical Dystonia

DYSTONIAS

One way to classify the dystonias is according to the parts of the body they affect:

  • Generalized dystonia affects most or all of the body.
  • Focal dystonia is localized to a specific part of the body.
  • Multifocal dystonia involves two or more unrelated body parts.
  • Segmental dystonia affects two or more adjacent parts of the body.
  • Hemidystonia involves the arm and leg on the same side of the body.

Some patterns of dystonia are defined as specific syndromes:

Torsion dystonia, previously called dystonia musculorum deformans or DMD, is a rare, generalized dystonia that may be inherited, usually begins in childhood, and becomes progressively worse. It can leave individuals seriously disabled and confined to a wheelchair. Genetic studies have revealed an underlying cause in many patients – a mutation in a gene named DYT1. And it has been discovered that this gene is related not only to generalized dystonia, but also to some forms of focal dystonia. Note, however, that most dystonia, of any type, is not due to this gene and has an unknown cause.

Cervical dystonia, also called spasmodic torticollis, or torticollis, is the most common of the focal dystonias. In torticollis, the muscles in the neck that control the position of the head are affected, causing the head to twist and turn to one side. In addition, the head may be pulled forward or backward. Torticollis can occur at any age, although most individuals first experience symptoms in middle age. It often begins slowly and usually reaches a plateau. About 10 to 20 percent of those with torticollis experience a spontaneous remission, but unfortunately the remission may not be lasting.

Blepharospasm, the second most common focal dystonia, is the involuntary, forcible closure of the eyelids. The first symptoms may be uncontrollable blinking. Only one eye may be affected initially, but eventually both eyes are usually involved. The spasms may leave the eyelids completely closed causing functional blindness even though the eyes and vision are normal.

Cranial dystonia is a term used to describe dystonia that affects the muscles of the head, face, and neck. Oromandibular dystonia affects the muscles of the jaw, lips, and tongue. The jaw may be pulled either open or shut, and speech and swallowing can be difficult. Spasmodic dysphonia involves the muscles of the throat that control speech. Also called spastic dysphonia or laryngeal dystonia, it causes strained and difficult speaking or breathy and effortful speech. Meige’s syndrome is the combination of blepharospasm and oromandibular dystonia and sometimes spasmodic dysphonia. Spasmodic torticollis can be classified as a type of cranial dystonia.

Writer’s cramp is a dystonia that affects the muscles of the hand and sometimes the forearm, and only occurs during handwriting. Similar focal dystonias have also been called typist’s cramp, pianist’s cramp, and musician’s cramp.

Dopa-responsive dystonia (DRD), of which Segawa’s dystonia is an important variant, is a condition successfully treated with drugs. Typically, DRD begins in childhood or adolescence with progressive difficulty in walking and, in some cases, spasticity. In Segawa’s dystonia, the symptoms fluctuate during the day from relative mobility in the morning to increasingly worse disability in the afternoon and evening as well as after exercise. The diagnosis of DRD may be missed since it mimics many of the symptoms of cerebral palsy.

National Institute of Neurological Disorders and Stroke


CERVICAL DYSTONIA

Cervical dystonia is rare movement disorder affecting 300,000-400,000 people in North America, in which there is a tightening of the neck muscles -which can cause the head to move into different positions. However, the head may remain straight in some cases.

dystonia

Courtesy of Raymond Fiorini, MD at musclepaindoctor.com

Quick Facts about Cervical Dystonia (Spasmodic Torticollis)

  • Cervical dystonia is also known as spasmodic torticollis.
  • Cervical dystonia is a focal dystonia that affects the neck and sometimes the shoulders.
  • Symptoms include involuntary contracting of the neck muscles, causing abnormal movements and awkward posture of the head and neck.
  • The movements may be sustained (“tonic”), jerky (“clonic”), or a combination.
  • Cervical dystonia may result in considerable pain and discomfort.
  • Treatments may include oral medications, botulinum toxin injections, surgery, and complementary therapies.
  • Cervical dystonia may be primary or secondary.

Symptoms
In cervical dystonia, the neck muscles contract involuntarily. If the contractions are sustained, they may cause abnormal posture of the head and neck. If the spasms are periodic or patterned, they may produce jerky head movements. The severity of cervical dystonia varies from mild to severe. Movements are often partially relieved by a “sensory trick” (also known as geste antagoniste) such as gently touching the chin, other areas of the face, or back of the head. Cervical dystonia may begin in the neck and spread into the shoulders, but the symptoms usually plateau and remain stable within five years of onset. This form of focal dystonia is unlikely to spread beyond the neck and shoulders or become generalized dystonia. Occasionally, people with cervical dystonia develop other focal dystonias.

Symptoms of CD often worsen while walking or during period of stress. Symptoms typically improve with rest or sleep. In addition, CD is the most common focal dystonia that responds to a sensory trick or geste antagoniste. For example, patients with CD may find that placing a hand on the side of the face, chin, or the back of the head, temporarily alleviates the dystonic posture. Leaning the head back against a chair or placing a hand on the top of the head may also help to relieve CD symptoms. The reason that sensory tricks work for some patients is not fully understood.

Muscle hypertrophy is present in almost all CD patients. Over two-thirds or up to 80% of patients, particularly those with sustained head deviation, have associated neck pain. About 33% to 40% of these patients also experience head tremor (i.e., dystonic tremor), hand tremor, or both. Approximately 20% of patients with CD also have blepharospasm or dystonia in other muscles or in muscle groups of the arm or hand. In addition, about 15% of patients have hand tremor resembling essential tremor.

Evidence suggests that about 10% to 20% of patients with cervical dystonia may have brief, spontaneous remissions. Almost all affected individuals eventually experience a relapse of symptoms. An additional 10%, particularly patients with an earlier age at symptom onset, may have longer remissions of about two to three years, typically beginning during the first few years following disease onset.

Movement Disorder Virtual University

Cause
Cervical dystonia may be primary (meaning that it is the only apparent neurological disorder, with or without a family history) or be brought about by secondary causes such as physical trauma. Cases of inherited cervical dystonia may occur in conjunction with early-onset generalized dystonia, which is associated with the DYT1 gene.

Diagnosis

Diagnosis of cervical dystonia is based on information from the affected individual and the physical and neurological examination. At this time, there is no test to confirm diagnosis of cervical dystonia, and in most cases assorted laboratory tests are normal.

Cervical dystonia should not be confused with other conditions which cause a twisted neck such as local orthopedic or congenital problems of the neck, or ophthalmologic conditions where the head tilts to compensate for impaired vision. It is sometimes misdiagnosed as stiff neck, arthritis, or wryneck.

Breanna Rose Foundation

Treatment
One of the most effective treatments for cervical dystonia is regular botulinum toxin injections to the affected muscles. A multitude of oral medications have demonstrated some benefit. The categories of drugs reported to help relieve the symptoms associated with cervical dystonia include anticholinergic drugs such as Artane ®(trihexyphenidyl) and Cogentin® (benztropine); dopaninergic drugs such as Sinemet® or Madopar® (levodopa), Parlodel® (bromocriptine), and Symmetrel® (amantadine); and GABAergic drugs such as Valium® (diazepam).

Selective denervation surgery is an option for some cervical dystonia patients, and deep brain stimulation surgery is beginning to be explored for cervical dystonia as well. Gentle physical therapy with a physical therapist who specializes in neurological disorders may improve range of motion and help reduce pain. Complementary therapies should be explored and regular relaxation practices may significantly benefit discomfort and pain.

Worldwide Education and Awareness for Movement Disorders

This is a short documentary produced by Ryan Murphy. It shows the daily struggles of the lives of 3 people who have a neurological movement disorder called Dystonia. For more information on dystonia, please contact Dystonia Medical Research Foundation.

Notable Tweets:
@jeffaevans @ClinNeuroNews @Apfeiffer @aliciaault @MedicalNewsNet @elizmech
“FDA Approves Dysport for Both Cervical Dystonia and Glabellar Lines” http://tinyurl.com/cxo9tt

@brainlocked

I have Cervical Dystonia a rare incurable disease. Tweeting to others helps me stay positive.
I was diagnosed with Cervical Dystonia a yr ago. I am deathly afraid of Botox.
Dr. put me on another drug yesterday. Now I take 8 per day! It’s all trial and error since there is no cure for Cervical Dystonia

@dystonia2006 @TheKomodoDragon
Im a 28 yr old female, diagnosed with cervical dystonia/writers cramp then later told “prob” generalized  http://is.gd/tEK4

@TheKomodoDragon
I’m a 56 yr old female, and got cervical dystonia as a repetitive-motion injury in 2003. Botox helped, but now my neck’s wo http://is.gd/tBV5

@Spasmodic
Check us out on facebook/myspace for more information about cervical dystonia!

Spasmodic Facebook Page
National Spasmodic Torticollis Association

Advertisements

~ by marbleroad on May 1, 2009.

One Response to “Cervical Dystonia”

  1. neurological movement disorder and endometriosis
    are the hard diseases should be avoided. A person who has this thing suffers most.

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s

 
%d bloggers like this: