INTRODUCTION	Section 2 of 11
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures Bibliography

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.

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.

	CLINICAL	Section 3 of 11
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures Bibliography

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.

Physical: 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.

Causes:

• 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.

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

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

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

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

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

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

• 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.