Spasmodic torticollis is characterized by involuntary tonic contractions or intermittent spasms of neck muscles. The cause is unknown. Diagnosis is clinical. Treatment can include physical therapy, drugs, and selective denervation of neck muscles with surgery or locally injected botulinum toxin.
In torticollis, contraction of the neck muscles causes the neck to turn from its usual position. It is the most common dystonia (see Movement and Cerebellar Disorders: Dystonias).
Spasmodic (or adult-onset) torticollis is usually idiopathic. About 5% of patients with spasmodic torticollis have a family history. One third of these patients have other dystonias (eg, eyelids, face, jaw, hand). Torticollis can also be congenital or secondary to other conditions such as lesions of the brain stem and basal ganglia.
Symptoms and Signs
Symptoms may begin at any age but usually begin between age 20 and 60, with a peak between age 30 and 50.
Symptoms usually begin gradually but may begin suddenly. Painful tonic contractions or intermittent spasms of the sternocleidomastoid, trapezius, and other neck muscles occur, usually unilaterally, and result in abnormal head position. Sternocleidomastoid muscle contraction causes the head to rotate to the opposite side and the neck to flex laterally to the same side. Rotation may involve any plane but almost always has a horizontal component. Besides rotational tilting (torticollis), the head can tilt laterally (laterocollis), forward (anterocollis), or backward (retrocollis). During sleep, muscle spasms disappear.
Spasmodic torticollis ranges from mild to severe. Usually, it progresses slowly for 1 to 5 yr, then plateaus. About 10 to 20% of patients recover spontaneously within 5 yr of onset (usually in milder cases with younger age onset). However, it may persist for life and can result in restricted movement and postural deformity.
The diagnosis is based on characteristic symptoms and signs and exclusion of alternative diagnoses, such as the following:
Spasms can sometimes be temporarily inhibited by physical therapy and massage, including sensory biofeedback techniques (slight tactile pressure to the jaw on the same side as head rotation) and any light touch.
Injections of botulinum toxin type A into the dystonic muscles can reduce painful spasms for 1 to 3 mo in about 70% of patients, restoring a more neutral position of the head. However, this treatment can lose effectiveness with repeated injections because antibodies develop against the toxin. Drugs can usually relieve pain, but they suppress dystonic movements in only about 25 to 33% of patients. Anticholinergics such as trihexyphenidyl 10 to 25 mg po once/day or bid may help, but adverse effects may limit their use; benzodiazepines (particularly clonazepam 0.5 mg po bid) and baclofen and carbamazepine may help. All drugs should be started in low doses. Doses should be increased until symptoms are controlled or intolerable adverse effects (particularly likely in the elderly) develop.
Surgery is controversial. The most successful surgical approach selectively severs nerves to affected neck muscles, permanently weakening or paralyzing them. Results are favorable when the procedure is done at centers with extensive experience.
Rarely, an emotional problem contributes to spasmodic torticollis; psychiatric treatment is indicated. Psychiatric prognosis is best if symptom onset coincided with exogenous stress.
Last full review/revision June 2008 by Sally Pullman-Mooar, MD