Torticollis - kau



Congenital Muscular Torticollis

Synonyms and related keywords: idiopathic spasmodic torticollis, IST, involuntary contractions of neck muscles, focal dystonia, congenital torticollis, birth trauma, intrauterine malpositioning, acquired torticollis, acute wryneck, painful neck spasms, cervical muscle spasm, tonic head deviation, clonic head movements, head torsion, spasmodic torticollis, head shaking, cervical osteomyelitis, retropharyngeal space infection, occipital condyle fracture, cervical muscle spasm following motor vehicle accident, odontoid fractures, cervical disk disease, L-dopa, neuroleptics, ocular condition, palsy of the inferior oblique muscle, essential head tremor.

Torticollis Overview

Torticollis is one of a broader category of disorders that exhibit flexion, extension, or twisting of muscles of the neck beyond their normal position. In torticollis your neck tends to twist to one side. The condition can either develop slowly if you have a family history of the disorder, acutely from trauma, or as an adverse reaction to medications.

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Child with right SCM

When the disorder occurs in people with a family history, it is referred to as spasmodic torticollis. The characteristic twisting of the neck is initially spasmodic and begins between ages 31-50 years. If you leave the condition 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.

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), you may experience deviation of the eyes (oculogyric crisis) and protrusion of the tongue (buccolingual crisis).

Background:

Torticollis (from the Latin torti, meaning twisted and collis, meaning neck) refers to presentation of the neck in a twisted or bent position. Torticollis manifests in involuntary contractions of the neck muscles, leading to abnormal postures and movements of the head. Idiopathic spasmodic torticollis (IST) is considered a focal dystonia.

What is torticollis?

Torticollis (also called congenital muscular torticollis) is a condition that causes a baby's head and neck to tilt to one side. It affects about 2 percent of newborns. The cause is unknown, but doctors suspect that when a baby is positioned in the uterus so that her head is tilted to the side and her neck is down, the blood supply to the neck is cut off. This results in some tightness in one of the two strap (or sternocleidomastoid) muscles that connect the breastbone, head, and neck, and allow a baby to turn her neck. Though the baby may look uncomfortable, this condition causes no pain. About 8 percent of babies born with torticollis will also have other problems such as hip dislocation.

Pathophysiology:

Torticollis is a symptom, as well as a disease, and it has a host of underlying pathologies. Torticollis can be divided into 2 types: congenital and acquired.

Congenital torticollis is usually not encountered in the ED, but it is worthy of mention because of its presentation. Infants born with torticollis appear healthy at delivery, but over days to weeks, they develop soft-tissue swelling over an injured sternocleidomastoid. Injury may be due to birth trauma or intrauterine malpositioning. This mass, which may be confused with a cystic hygroma or branchial cleft cyst, regresses and leaves a fibrous band in place of the sternocleidomastoid muscle, causing contracture of the neck.

Acquired torticollis has an identical presentation, but it has a host of underlying pathologies that must be excluded before diagnosis of IST can be made.

An acute form of torticollis, known as acute wryneck, is the type most frequently encountered in the ED. Acute torticollis develops overnight in young and middle-aged adults. Patients present with painful neck spasms. On examination, cervical muscle spasm is visible and palpable. Symptoms usually resolve spontaneously within 2 weeks. Treatment is symptomatic and consists of the use of heat, massage, supportive cervical collar, muscle relaxants, and analgesics.

IST is classified in a broad category of dystonic states, as a type of focal dystonia (ie, dystonic movements in a single body part). Torticollis may be associated with other forms of focal dystonia, such as blepharospasm, writer's cramp, spasmodic dysphonia, or orobuccal dystonia. Patients may present with tonic head deviation, clonic head movements, or both. Head deviation can be subdivided into lateral tilt or torsion. Patterns are not fixed and may change over time. The operational definition of IST contains the following elements:

• Acquired, nontraumatic origin

• Adult or childhood onset

• Clonic and/or tonic involuntary contractions of multiple neck muscles

• Sustained head torsion and/or tilt

• Duration of 6 months or longer

• Often associated with postural limb tremor

• No history of chronic neuroleptic treatment

• No associated ataxia, weakness, spasticity, or reflex changes

• Normal brain CT scan

Frequency:

• In the US: The exact incidence of IST is unknown, but it is thought to be about 3 per 10,000 individuals.

Mortality/Morbidity: Stress and emotional events may exacerbate symptoms of torticollis.

• Persistent neck deviation occasionally elicits avoidance behaviors.

• Considerable somatic and psychological disability may accompany chronic torticollis.

Sex: IST affects women more often than men, with a 4.5:1 ratio.

Age: IST may occur in children or adults. In 90% of cases, however, symptom onset occurs in patients aged 31-60 years.

Causes:

The most common cause of torticollis in the first year of life is Congenital Torticollis which is more commonly called Congenital Muscular Torticollis or CMT. Research studies have put the incident rate of CMT to be 0.04% to as high as 1.9% (1,2).

There are three different ways that CMT can present itself:(3)

(1) A sternomastoid tumor that can be felt upon examination (fibromatosis colli)

(2) Tightness or thickening of the sternomastoid muscle

(3) Torticollis caused by posture preference with no tightness or tumor.

This exact cause of CMT has not been identified but some theories have been made that link CMT to bad inutero positioning or low amniotic fluid levels that restrict the environment of the growing fetus and prohibit the ability of the SCM muscle to stretch and grow which results in a contracted SCM muscle (also called perinatal compartment syndrome)(4). It is thought that the shortened muscle may actually be a contributing factor to a difficult birth and not its consequence. CMT is the third most common musculoskeletal anomaly(5) after hip dysplasia and clubfoot.

• IST has no clear etiology, although a lesion of the thalamus has been suspected.

• In some patients, IST is seen in several generations of their families.

• Many have relatives with other extrapyramidal disorders, usually tremor.

• Consider other causes before establishing a diagnosis of IST.

o Infection: Cervical osteomyelitis and retropharyngeal space infection may be confused with IST.

o Tumors: Tumors may cause IST-like symptoms by producing a mass effect on the muscle.

o Trauma: Occipital condyle fractures, cervical muscle spasm following motor vehicle accident, and odontoid fractures may simulate torticollis.

o Cervical disk disease: Subluxation and/or herniation can cause deviation to one side.

o Drug-induced condition: L-dopa and neuroleptics may induce focal dystonias. Alcohol may worsen underlying torticollis.

o Ocular condition: Patients with palsy of the inferior oblique muscle hold their heads at an angle to correct visual disturbances.

o Essential head tremor: Tremor may precede or coexist with IST and can be stopped with intentional turning of the head. As with IST, medication lists should be reviewed.

Imaging Studies:

• Plain cervical radiographs may be useful to exclude bony trauma or osteomyelitis.

• MRIs of the head may help rule out brain tumor. MRIs also may help if a herniated intervertebral cervical disk is suspected.

• CT scans are useful to identify and exclude retropharyngeal abscess or other neck mass. Brain CT scans may not equal brain MRIs, but they are more likely to be available to ED physicians.

CMT is most often diagnosed when an infant is 2-3 weeks old due to the presence of a mass or tumor (fibromatosis colli) in the belly of the SCM. In infants that do not have a mass, torticollis is unusually noticed around 2 months old when the neck muscles begin to develop and the head tilt becomes clear. Diagnosing this condition involves a physician taking a medical history (or reading the child's medical records), performing a physical exam, and if needed, scheduling of additional testing. Since there may be other causes for the torticollis posture, many physicians will have additional testing performed as a precaution. This testing includes x-rays of the cervical spine, ultrasound, MRI, or a CT scan. If the child has a lump/mass an ultrasound is usually performed with additional testing of a biopsy or fine needle aspiration to further study the cause of the lump/mass.

Symptoms:

• 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.

• 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.

• The infent may hold her head to one side and have limited neck movement, or she may have a small bump on the side of her neck. Torticollis is usually diagnosed within the first two months of a baby's life because even if parents don't spot it, a pediatrician will. Babies with torticollis may also develop positional plagiocephaly (asymmetrical head shape) because they'll often sleep with their head turned to the affected side. There are also a few other less common forms of torticollis. One, called wryneck, which happens when one of the vertebrae doesn't develop completely. Another, called acquired torticollis, is a serious condition that can come on anytime after birth and is usually an indication of a more serious problem, such as a brain or spinal cord tumor. Your doctor may need to take X-rays or perform other tests to determine which form your baby has.

How is it treated?

• If your baby is found to have congenital muscular torticollis, you'll want to get her started on a stretching routine to lengthen and stretch her tight neck muscles. The doctor may show you the proper exercises (or refer you to a physical therapist), and you'll be required to perform them on your child several times a day. The moves aren't complicated, but if you're not comfortable doing them, ask your physical therapist to help you. You'll also want to provide as many opportunities as possible for your baby to turn her head to the other side. To that end, you should approach her from the non-affected side, encouraging her to turn her head in that direction. You can also place your baby to sleep with her head turned to the non-affected side. When she's awake, it's very important to give your baby plenty of time on her stomach. This will help to develop the muscles in her neck.

How long does it take to see results?

• You'll probably see improvement within weeks. And as long as your baby's condition was discovered early enough — ideally by the age of 2 or 3 months — and she's following a rigorous stretching program, it should be fully corrected by age 1. (Your doctor may refer you to a physical therapist to help stretch the muscles.) If, however, the muscles do not return to their normal length and your baby doesn't have a normal range of motion by the time she's 18 months old, your doctor may refer you to an orthopedic surgeon, who may recommend surgery to lengthen the muscles. Surgery is necessary in about 15 percent of cases.

When to Seek Medical Care

In general, acute torticollis is not life threatening. If symptoms are limited to muscle stiffness and pain, see your doctor in at least a day.

If you injure your neck but cannot reach your doctor, go immediately to a hospital's emergency department. Other medical conditions may appear to be torticollis and will need immediate medical attention. For example, 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:

• 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

CLINICAL ASSESSMENT:

History:

• Signs and symptoms of torticollis change as the disease progresses.

• During later stages of the chronic disease, patients have more pain and difficulty performing motor activities.

• One sixth of patients report head trauma that occurred several days, or even months, before symptom onset.

• Definite spasmodic torticollis develops within 6 months of the first appearance of symptoms in 42% of patients, within 6-12 months in 20% of patients, and after 1 year in 35% of patients.

• Head turning is the most frequent symptom (80% of cases), and the patient often notes this finding while driving or attempting to perform a bimanual task involving direct visualization.

• One fourth of patients learn that their heads are turned only when others inform them.

• Neck pain occurs in 50% of cases.

• Head shaking occurs in 50% of cases.

• Abnormal posture occurs in 25% of cases.

Exams and Tests

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 of the neck, may require a CT scan of the neck.

Physical ASSESSMENT:

The physical examination should be directed at identifying underlying etiologies as well as documenting the degree of muscle involvement.

• Limited cervical range of motion and muscle contraction of sternocleidomastoid and paracervical muscles may be present.

• Severe muscle contraction and spasm may be present in the sternocleidomastoid, levator, splenius, and trapezius muscles.

• Head deviation to one side, with slight neck flexion, is the presenting posture.

TREATMENT

Treatment for torticollis is targeted to relax the contracted neck muscles involved. These treatments include medication, physical devices, botulinum toxin, 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.

Prehospital Care: Immobilize the cervical spine for any patient with acute torticollis and a history of neck trauma.

Emergency Department Care: Patients with torticollis may present to the ED because of adverse effects of therapy for another disease. Identify and appropriately treat the cause. Review the medication list of all patients with IST or essential head tremor.

Consultations:

• Refer most patients with symptoms suggestive of IST to a neurologist for follow-up.

• Since IST progresses slowly and is stable, immediate consultation is unnecessary.

• Referral to a neurologist specializing in movement disorders may hasten diagnosis and treatment.

Drugs of choice for IST include benzodiazepines, anticholinergics, and local intramuscular injections of botulinum toxin (BOTOX®). Emergency physicians, as standard practice, do not administer BOTOX® injections. Tertiary referral centers perform most injections.

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.

Drug Category: Benzodiazepines -- By binding to specific receptor sites, these agents appear to potentiate effects of gamma-aminobutyric acid (GABA), facilitate inhibitory GABA neurotransmission, and assist other inhibitory transmitters. Benzodiazepines may act in the spinal cord to induce muscle relaxation.

Surgery

Surgery is reserved only for a few selective cases. In this treatment, some of the upper neck nerves are selectively severed to prevent muscle contraction. Surgical treatment often helps, but frequently your neck will return to its twisting position after several months.

Correction consists of conservative [non-surgical] treatment of physical therapy with passive stretching and exercises. Exercises concentrate on relaxing tight muscles, lengthening shortened muscles, and improving the range of motion in the neck.

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Stretching right SCM

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Stretching of right SCM

[pic]

Stretching of right SCM

Chiropractic care, massage therapy, cranial sacral therapy, muscle taping, use of the Tubular Orthotic for Torticollis (TOT) collar, Botox injections, and alternative medicine approaches may also be used in addition to the physical therapy treatment of torticollis. You should not begin any type of alternative or home treatment program unless your child is under the direct supervision or guidance from a trained healthcare professional. Always discuss your intentions with your child's healthcare professional, and if needed, get a second opinion before making your decision.

Therapy is usually followed until the child is tilt free and in many cases, follow up visits to a therapist are recommended until a child has learned to walk because there may be regression as new milestones are reached.

 If therapy fails to produce satisfactory results, or if symptoms worsen, then surgery to lengthen the SCM might be considered.

Prognosis:

• In most patients, IST gradually progresses over months to years.

• Complete remissions sometimes occur.

• Remission percentages have been reported in 12% or 21% of patients, particularly younger patients during the first 5 years of IST. In these reports, most remissions were only temporary.

Outlook

• 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.

• 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.

BIBLIOGRAPHY

• Bittar RG, Yianni J, Wang S, et al: Deep brain stimulation for generalised dystonia and spasmodic torticollis. J Clin Neurosci 2005 Jan; 12(1): 12-6[Medline].

• Claypool DW, Duane DD, Ilstrup DM, Melton LJ III: Epidemiology and outcome of cervical dystonia (spasmodic torticollis) in Rochester, Minnesota. Mov Disord 1995 Sep; 10(5): 608-14[Medline].

• Costa J, Espirito-Santo C, Borges A, et al: Botulinum toxin type B for cervical dystonia. Cochrane Database Syst Rev 2005; CD004315[Medline].

• Denislic M, Pirtosek Z, Vodusek DB, et al: Botulinum toxin in the treatment of neurological disorders. Ann N Y Acad Sci 1994 Mar 9; 710: 76-87[Medline].

• Duane DD: Spasmodic torticollis. Adv Neurol 1988; 49: 135-50[Medline].

• Gauthier S: Idiopathic spasmodic torticollis: pathophysiology and treatment. Can J Neurol Sci 1986 May; 13(2): 88-90[Medline].

• Hakkinen A, Ylinen J, Rinta-Keturi M, et al: Decreased neck muscle strength is highly associated with pain in cervical dystonia patients treated with botulinum toxin injections. Arch Phys Med Rehabil 2004 Oct; 85(10): 1684-8[Medline].

• Havaki-Kontaxaki BJ, Kontaxakis VP, Margariti MM, et al: Treatment of severe neuroleptic-induced tardive torticollis. Ann Gen Hosp Psychiatry 2003 Oct 17; 2(1): 9[Medline].

• Joyce MB, de Chalain TM: Treatment of recalcitrant idiopathic muscular torticollis in infants with botulinum toxin type a. J Craniofac Surg 2005 Mar; 16(2): 321-7[Medline].

• Lee LH, Chang WN, Chang CS: The finding and evaluation of EMG-guided BOTOX injection in cervical dystonia. Acta Neurol Taiwan 2004 Jun; 13(2): 71-6[Medline].

• Sa DS, Mailis-Gagnon A, Nicholson K, Lang AE: Posttraumatic painful torticollis. Mov Disord 2003 Dec; 18(12): 1482-91[Medline].

• Singh S, Goyal V, Prasad K, Behari M: Cervical dystonia responsive to levodopa. Neurol India 2004 Jun; 52(2): 276-8[Medline].

• Takeuchi N, Chuma T, Mano Y: Phenol block for cervical dystonia: effects and side effects. Arch Phys Med Rehabil 2004 Jul; 85(7): 1117-20[Medline].

• Truong D, Duane DD, Jankovic J, et al: Efficacy and safety of botulinum type A toxin (Dysport) in cervical dystonia: results of the first US randomized, double-blind, placebo-controlled study. Mov Disord 2005 Jul; 20(7): 783-91[Medline].

• van Herwaarden GM, Anten HW, Hoogduin CA, et al: Idiopathic spasmodic torticollis: a survey of the clinical syndromes and patients' experiences. Clin Neurol Neurosurg 1994 Aug; 96(3): 222-5[Medline].

(1) Ling CM &, Low YS. (1972) Sternomastoid tumor and muscular torticollis. Clin Orthop, 86, pp. 144-50.

(2) Suzuki S, Yamamura T & Fujita A. (1984) A etiological relationship between congenital torticollis and obstetrical paralysis. Int Orthop, 8, pp. 175-81.

(3) Cheng JCY, Wong MWN, Tang SP, Chen TMK, Shum SLF, & Wong EMC. (2001) Clinical Determinants of the Outcome of Manual Stretching in the Treatment of Congenital Muscular Torticollis in Infants : A Prospective Study of Eight Hundred and Twenty-one Cases. J Bone Joint Surg. (Am) 83, pp. 679-87

(4) Davids JR, Wenger D, & Mubarak SJ. (1993) Congenital muscular torticollis: Sequelae of intrauterine or perinatal compartment syndrome. J Pediatr Orthop. 13, pp. 141-7.

(5) Bredenkamp JK, Hoover LA, Berke GS, Shaw A. (1990) Congenital Muscular Torticollis. A spectrum of disease. Arch. Otolaryngol Head Neck Surg, 116, pp. 212-6.

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