Torticollis

Torticollis (wry neck, or loxia) is one of a broader category of disorders that exhibit flexion, extension, or twisting of muscles of the neck beyond their normal position. The Latin definition of torticollis means "twisted neck." In torticollis, the neck tends to twist to one side, causing head tilt. The condition can either develop slowly if you have a family history of the disorder, or acutely from trauma, or as an adverse reaction to medications.

When the disorder occurs in people with a family history, it is referred to as spasmodic torticollis (also called cervical dystonia). The characteristic twisting of the neck is initially spasmodic (involuntary contractions of the neck muscles that lead to abnormal movements and awkward posture of the head and neck) and begins between 31-50 years of age. If the condition is left untreated, it likely will become permanent.

Bending or twisting your neck too far can lead to acute torticollis. This condition appears with few symptoms, although often you will appear uncomfortable and will hold your head straight or rotated to one side. It will hurt to move your head to the opposite side. Your neck muscles on the side that hurts often are tender to the touch. The doctor will check your nerve and motor function to rule out spinal cord injury.

Benign paroxysmal torticollis of infancy (BPTI) is a rare medical disorder in infants characterized by recurrent episodes (or attacks) of tilting of the head to one side.

In adults, acute torticollis can be caused by many different conditions. Occasionally, no specific cause is found.

Trauma to the neck or spine can lead to torticollis. Injuries to the cervical spine or neck muscles often result in spasm of the muscles, leading to the twisting of the head, characteristic of torticollis.

Other causes include infection of the head or neck. These infections can cause an inflammatory torticollis secondary to inflamed glands and lymph nodes in the neck. The muscles overlying these lymph nodes may contract. Torticollis may be associated with abscesses of the throat and upper airway, and those situations can be life-threatening. Other infections of the sinuses, ears, mastoids, jaw, teeth, or scalp also can lead to torticollis.

Rarely, tumors, scar tissue, arthritis of the cervical spine, or vascular abnormalities may also cause torticollis.

Certain drugs of abuse such as ketamine, amphetamines, and cocaine as well as commonly prescribed neuroleptic drugs such as prochlorperazine (Compazine), haloperidol (Haldol), and chlorpromazine (Thorazine) can cause acute dystonia (a lack of normal muscle control). This is a condition that involves the sudden onset of involuntary contractions of the muscles of the face, neck, or back.

In addition to bending of the head to one side (acute torticollis), people may experience other symptoms such as deviation of the eyes (oculogyric crisis) and protrusion of the tongue (buccolingual crisis).

In addition to the causes above, children, infants, and newborns may also acquire torticollis from congenital causes or trauma due to childbirth. Congenital muscular torticollis (CMT) is the most common cause of torticollis in infants. CMT is a postural physical deformity present at birth that results from a shortening and fibrosis of the sternocleidomastoid muscle on one side of the neck. Infants often sleep with their heads in the same position against the mattress, which can lead to plagiocephaly (flat head syndrome), which is why it often accompanies muscular torticollis.

Risk factors for torticollis include a family history of the disorder, congenital abnormalities of the cervical spine, taking drugs that predispose to muscular spasm, and trauma.

• Because spasmodic torticollis is an abnormal contraction of the muscle in one side of the neck, people will appear with their head turned to one side. Neck muscles and those between the neck and shoulder will be tense and tender, causing neck pain.
• People with acute torticollis will be unwilling to turn their head to one side or may have their head turned slightly away from the side of discomfort.
• Deviation of the eyes (oculogyric crisis) where the eyes involuntarily look upward and protrusion of the tongue (buccolingual crisis) where the tongue sticks out involuntarily may also occur.
• With spasmodic torticollis (cervical dystonia), there may be neck muscle spasms that are sustained (tonic) or jerky (clonic).
• Other symptoms may include shoulder pain, back pain, headache, neck cramps, muscle tightness, muscle pain, or burning sensations.
• Attacks of benign paroxysmal torticollis in infants may also be accompanied by symptoms such as vomiting, irritability, and drowsiness.

In general, acute torticollis is not life-threatening. If symptoms are limited to muscle stiffness and pain, see a doctor within one day of onset.

If you injure your neck and have spasm of the muscles, go immediately to a hospital's emergency department. Other medical conditions may appear to be torticollis and will need immediate medical attention. Anyone who experiences spasms of the neck muscles involved with swallowing or breathing or symptoms that might involve the central nervous system should be evaluated.

The following symptoms suggest injury or irritation to the central nervous system and should be treated as an emergency especially if they suddenly develop:

• Trouble breathing or swallowing
• Pins-and-needles feeling or numbness in your arms and legs
• Urinary hesitancy, urinary or fecal incontinence
• Weakness in your arms and legs
• Impaired speech
• Difficulty walking
• If the spasm of the neck muscles is associated with fever, swollen glands, headache, neck stiffness, mouth or tongue swelling, or trouble swallowing, seek medical attention immediately.

The doctor will take a detailed history emphasizing specific medications that you may be taking. A physical examination will be performed.

When there is a history of trauma, the doctor may take X-rays of your neck to exclude a fracture or dislocation of the spinal bones in your neck.

• Often, X-rays are sufficient to make this determination.
• In a small number of cases, subtle abnormalities or preexisting conditions, for example, degenerative arthritis of the spine or of the neck may require a CT scan.

Treatment for torticollis is targeted to relax the contracted neck muscles involved. Treatments include medication, physical devices, botulinum toxin, physical therapy, stretching exercises, and surgery. In most people, torticollis resolves in several days to a few weeks. A few people will develop continuing neck problems for months to years. Persistent neck muscle spasms may require referral to a neurologist or surgeon.

You may initially be diagnosed with torticollis by your family practitioner or internist, or if the torticollis is due to an acute injury, you may be diagnosed by an emergency-medicine specialist in a hospital's emergency department. Infants and children will be diagnosed by their pediatrician.

For further care, you may be referred to a physical medicine and rehabilitation specialist (physiatrist) or orthopedist who specializes in musculoskeletal disorders and treatment. Some types of torticollis due to neurological conditions may be treated by a neurologist.

Physical therapy is often a primary treatment for torticollis and you may see a physical therapist or occupational therapist to help work on stretching and strength exercises.

In severe cases when surgery is needed, you may see an orthopedic surgeon or a surgical specialist in brain-stimulation techniques.

• If you have spasmodic torticollis that was caused by trauma or by medications, the doctor may prescribe muscle relaxants and anti-inflammatory drugs. These agents usually relieve the symptoms completely within a few days. Common medications to treat acute, spasmodic torticollis include benztropine (Cogentin) or diphenhydramine (Benadryl). These medications are usually given into the muscle or through the vein. The doctor may add muscle relaxant or benzodiazepines such as Ativan or Valium. The medications are continued in their oral form for 48-72 hours to avoid recurrent symptoms. Ice packs and massage therapy may also be used for relief.
• For chronic neck muscle spasms, a neurologist may give a local injection of botulinum A toxin (Botox). This toxin comes from Clostridium botulinum bacteria. It acts locally to prevent muscle contraction in the muscles where it is injected. The toxin often can stop torticollis from progressing and may lead to complete recovery.

Surgery is reserved only for a few selective cases. In this treatment, some of the upper neck nerves and/or muscles are selectively severed to prevent muscle contraction. Surgical treatment often helps, but frequently the neck will return to its twisting position after several months. Rarely, deep brain stimulation is done by inserting a wire into the brain where movement is controlled and then sending electrical signals to disrupt brain signals causing torticollis.

Once you have been diagnosed with torticollis, there are some home remedies that may help relieve symptoms.

• Lay on your back. Symptoms often disappear during sleep, so taking a break to lie on your back may provide relief.
• Touch the opposite side of the face, chin, or neck. This tricks your body and may help spasms stop temporarily.
• Apply heat. Heat packs or hot water bottles applied to the neck may help loosen tight muscles.
• Stress-reduction techniques: Know what causes you stress or anxiety because this can lead to tension and worsening of symptoms.

For infants, passive stretching (stretches done with the assistance of a parent or caregiver) may be performed. In older babies, facilitating active movement may be helpful, for example, using sights or sounds to get a child to turn their head in a certain direction. A physical therapist will recommend stretches and other exercises and show you how to do them properly.

Physical therapy exercises for adults with torticollis may include graded (step-by-step) neck exercises. You may first start by trying to gradually move your head a little further in each direction each time. Then, try to keep your head in the final position for longer periods of time. If you cannot perform these exercises on your own, you may have another person assist you with gentle passive movements. Do your exercises several times a day, in front of a mirror when possible to see if you have been able to move your head farther. Consult a physiatrist or physical therapist first for instructions on how to do movements and exercises correctly.

• Prevention of torticollis may not be possible, but the prognosis for torticollis is generally good. For the great majority of people with acute torticollis, the condition goes away in several days to a few weeks. A small number of people will go on to develop continuing problems with their neck for months to years.
• For children with congenital muscular torticollis, physical therapy is often successful and early intervention can prevent future problems.
• Most people with acute torticollis are successfully treated with medication. If a drug caused the spasm, it should be stopped.
• Spasmodic torticollis is successfully treated with local injections of botulinum A toxin in combination with medications.
• If these conservative measures are unsuccessful, surgery on the nerves of the neck can be attempted. After surgery, many people will often have initial relief, but most relapse after several months.
• Complications of torticollis include neck deformity and constant neck stiffness and pain. This constant tension may result in muscle swelling and neurological symptoms due to pressure on the nerve roots.
• In some cases, people who become disabled from the pain of torticollis may have difficulty performing daily activities, may no longer be able to drive, and may develop depression.