AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Torticollis is the common term for various conditions of head and neck dystonia, which display specific variations in head movements (phasic components) characterized by the direction of movement (horizontal, as if to say "no", or vertical, as if to say "yes"). Such to-and-fro movements of the head can be equal (as in a tremor) or unequal (ie, rapid clonic movements of the head and neck with slow recovery, termed "spasmodic").

Characteristic head tilt often occurs from a tonic component. One example is laterocollis, in which the head is displaced with the ear moved toward the shoulder from increased tone in the ipsilateral cervical muscles. Another is rotational torticollis, in which partial rotation or torsion of the head occurs along the longitudinal axis. In anterocollis, the head and neck are held in forward flexion with increased tone of anterior cervical muscles; in retrocollis, the head and neck are held in hyperextension with increased tone in the posterior cervical muscles.

No matter which term is preferred in communicating about these conditions, the implication is that they all represent differing degrees of the same phenomenon. Jankovic et al (1991) and Chan et al (1991) would like to avoid the popular term "spasmodic torticollis" and prefer "cervical dystonia," because many patients have neither simple rotation nor spasmodic movements. In fact, several patients have combinations of movements, not as simple tremors but as responses to dystonic motor control.

Pathophysiology

As a neurodegenerative disease, torticollis, or idiopathic cervical dystonia, is believed to arise from basal ganglia circuit abnormalities stemming from selective vulnerability of these structures to an abnormal biochemical process that leads to neuronal loss. Some indication of involvement of dopamine-secreting circuits comes from findings of low levels of metabolites of dopamine in the cerebrospinal fluid and some minor improvements reported from individual trials of levodopa and traditional neuroleptics, both of which possess equal D1 and D2 receptor-binding properties. Neither moderate-dose levodopa nor high-dose anticholinergics are as effective as in idiopathic torsion dystonias or inherited dystonias, which therefore have clearly different receptor responses and circuit abnormalities.

The use of selective D2 ligands with single-photon emission computerized tomography (SPECT) scanning in 10 patients with torticollis has shown reduced D2-receptor binding in the basal ganglia (Naumann, 1998). Similar results have been noted in focal hand dystonia by using SPECT (Horstink, 1996) and positron emission tomography (PET) scanning (Perlmutter, 1996). The implication is that underactivity occurs in the D2 dopamine receptors located in the indirect pallidal outflow pathway in both conditions. Such underactivity can be expected to cause disinhibited thalamocortical output and dystonic postures.

This relative imbalance between direct (D1-related) and indirect (D2-related) pallidal outflow pathways (see Image 1) explains the failure of levodopa to adequately improve torticollis and the transient improvement from traditional neuroleptics, which initially may reduce D1 activity and eventually both D1 and D2 activity in both pathways.

Pramipexole is a dopamine agonist with selective, highly potent binding properties to D2 and D3 receptors. The authors have tried pramipexole in an open label trial with 14 patients with idiopathic cervical dystonia who displayed uncomplicated torticollis (unpublished results). Reduction in stiffness of neck muscles and head movements was reported in 6 patients who received 1.5 mg 3 times per day for at least 2 years. Five of 8 patients improved on 5 mg olanzapine, a dopamine-receptor blocker with minimal D1-blocking potency compared to its major D2- and D3-blocking potency. Atypical neuroleptic action suggests a bilateral relative binding effect, in which blocking the D2 action on the opposite indirect pathway may enhance the ipsilateral D2 receptor effect by comparison. This observation may suggest a mechanism of bilateral rather than unilateral basal ganglia control of torticollis.

Another approach to increasing the inhibitory output of the indirect pathway (an alternative to increasing D2 receptor action in the same pathway) is to deplete or block glutamate action. Selective glutamate release inhibition can be achieved with riluzole or with glutamate receptor blocking with amantadine, lamotrigine, or memantine.

Although the D3 activity of pramipexole has been linked to improvement in mood (Cummings, 1999), D3 receptors are also found in the striatum of the basal ganglia and may provide a role complementary to the expected increased activity in the indirect pathway provided by D2 action of the drug. The action of the atypical neuroleptics such as olanzapine or risperidone is quite interesting and needs far more extensive evaluation before a mechanism of rebalance can be offered.

Further studies of receptor binding are needed to clarify the unknown process leading to the slow evolution and progression of torticollis. Such understanding is also necessary in providing viable medication alternatives to repeated botulinum toxin injections every few months and the surgical alternatives offered in cases of injection failure.

Frequency

United States

Reports of incidence are available primarily from the United States and Canada. Posttraumatic cases account for 10-20% of cases; the others are idiopathic. Consky and Lang (1994) have reviewed several series to determine the relative frequency of torticollis types, with the following conclusions: (1) most cases of torticollis have mixtures of movements; (2) spasmodic features presumably dominate and relate to the classic descriptor of head jerks and spasms, hence the term spasmodic torticollis (no consensus exists regarding that description; the term cervical dystonia is preferred); (3) torticollis with some degree of rotation is the most common individual type; and (4) after torticollis come laterocollis and then retrocollis in frequency, with anterocollis being the rarest form.

International

No data are available except from Canada (see above).

Mortality/Morbidity

These conditions do not usually lead to death, and life span is normal. Morbidity concerns 3 areas that may require additional treatment.

• Chronic pain due to dystonia or strain in attempts to compensate for abnormal postures
• Cervical spondylosis from chronic abnormal dystonic posture, which can lead to radiculopathies and/or spinal stenosis
• Social embarrassment or the extreme of social isolation with depression

Race

No racial predominance is reported for torticollis.

Sex

Torticollis is reported twice as often in females as in males.

Age

Onset of idiopathic cervical dystonia typically occurs when patients are aged 30-50 years. Onset of posttraumatic cervical dystonia is within days of injury for the acute form and 3-12 months after injury for the delayed form.

CLINICAL

Section 3 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

Of patients with torticollis (cervical dystonia), 80-90% fall into the idiopathic category, typically without family history. A positive family history suggests that the case in question may in fact be a residual form of an inherited generalized dystonia. The remaining 10-20% of patients with torticollis (cervical dystonia) fall into the posttraumatic category.

Other neurologic problems can mimic torticollis, and the practitioner should be alert to a history of adversive seizures, homonymous hemianopsia, and various ocular disturbances that lead to head tilt, including a variety of cervical spinal deformities, ocular palsies, congenital nystagmus, labyrinthine disease, and probable cervical adenitis.

• Idiopathic cervical dystonia demonstrates a slowly progressive course initiated in patients aged 30-50 years.
• • Details of the extent of dystonia (including dystonic speech, involvement of upper limbs, other body parts with painful sustained contractures) may suggest a genetic, more generalized form of dystonia but can also occur as a natural progression of cervical symptoms over time.
  • Jahanshahi et al (1990) reported progression of dystonic symptoms to extranuchal but still cervical innervated sites (hand, arm, oromandibular region) in 32% of 72 patients with adult-onset cervical dystonia.
  • In addition, Comella et al (1992) observed both clinical dysphagia and subclinical swallowing motility disturbances in such patients.
  • Action-induced or activity-induced worsening of torticollis and dystonia are typical, as are variable periods of lessened symptoms in the morning (ie, morning benefit). Patients often discover certain sensory tricks (ie, gestes antagonistiques) that reduce head and neck movement (eg, touching the face in a particular spot with the thumb). The absence of sensory tricks can help distinguish acute traumatic torticollis and nondystonic torticollis from idiopathic and delayed dystonic torticollis.
  • A positive history of chronic neuroleptic drug use may call attention to possible tardive dystonia.
  • Of patients with cervical dystonia, 10-20% experience spontaneous self-limited remissions that may be quite brief or last as long as 2-3 years.
• Posttraumatic cervical dystonia is divided into 2 subtypes, acute onset (initiated immediately to a few days after head and neck trauma) and delayed onset (3-12 mo after head and neck trauma).
• • Characteristics of acute posttraumatic cervical dystonia include local pain immediately following trauma such as concussion or whiplash injury, followed within days by a marked limitation in range of motion of the neck and an abnormal posture of the head (without phasic components), elevation of the shoulder, and eventual hypertrophy of the trapezius. Two characteristics distinguish acute posttraumatic from idiopathic and delayed posttraumatic cervical dystonia: (1) no increase in symptoms with effort and (2) no inhibitory response to sensory tricks.
  • Delayed-onset posttraumatic cervical dystonia is nearly identical to idiopathic cervical dystonia and includes activation by effort and the ability to minimize symptoms by the use of sensory tricks.
• Whether occupational overuse or subacute recurring trauma can lead to cervical dystonia, as hypothesized with focal hand dystonia (writer's cramp) or musicians' syndromes, is uncertain (Jankovic, 2001).

Physical

The primary goal in physical examination is to locate evidence for torticollis or cervical dystonia as the obvious primary finding representing the primary process, with additional dystonic features in the limb or hand being minimal and typically unilateral. Generalized dystonia does not reinforce the diagnosis but draws attention to idiopathic torsion dystonia or one of the genetic forms of dystonia.

• Characterization of head and/or neck posture (tonic components) and of dystonic head movements (phasic components)
• • Tonic head and neck posture (when chronic, may cause scoliosis)
    • Rotational torticollis: Head is turned around the long axis with nose and chin toward the shoulder; this is the most common head and neck deviation. This is not synonymous with torsion dystonia, a generalized dystonia named for rare athetoid components. Tone and bulk increase are appropriate in the sternomastoid contralateral to the direction of turn.
    • Simple torticollis: No head tilt is present. Document increased tone of neck muscles as symmetric or absent, hypertrophied or normal.
    • Laterocollis: Head tilts to one side with ear toward shoulder; asymmetric tone and muscle bulk also present.
    • Anterocollis: Head tilts forward with chin toward the chest, and anterior cervical muscles are increased in tone and bulk.
    • Retrocollis: Head tilts in hyperextension with increased tone and bulk in the posterior cervical muscles.
  • Phasic head components
    • Spasmodic jerks - Rapid irregular clonic jerks with less rapid recovery toward the neutral position
    • High-frequency oscillations - Horizontal, vertical, mixed, or irregular tremors
• Other dystonic features
• • Extranuchal dystonias may occur on the side ipsilateral to the cervical dystonia (if bilateral or contralateral, consider more generalized or torsion dystonias).
  • Oral, facial, or mandibular dystonias occasionally are associated with blepharospasm and laryngeal dystonia but not with neuroleptic use.
• Nondystonic findings
• • Swallowing difficulty (trouble initiating)
  • Cervical radiculopathies (secondary to bony changes)
  • Ulnar neuropathy secondary to performing sensory tricks
  • Reactive depression, self-consciousness

Causes

• Because idiopathic cervical dystonia is a neurodegenerative process, the confluence of etiologic factors in modern popular explanations applies here as it does in idiopathic Parkinson disease. Patients have a genetically determined susceptibility to environmental toxins, which, if encountered in threshold doses, activate free radical production in susceptible brain regions, leading to neuronal deterioration.
• The role of trauma in posttraumatic cervical dystonia is discussed in History and Medical/Legal Pitfalls.
• Because both idiopathic and delayed posttraumatic cervical dystonia wax and wane with emotional tone, the patient may believe an unjustified assertion that the dystonic problem is psychiatric in nature. This belief is easily reinforced by others who are not medically trained and actually was presumed by medical practitioners before the advent of synaptic chemistry and neurophysiology.
• Occasionally, torticollis with dystonic components or major cervical dystonia occurs as part of the overall clinical picture of Parkinson disease. The entire degenerative disease process should not be considered two processes but rather one process (ie, Parkinson disease). When head tremors without dystonic components occur with postural tremors of the upper extremities, consider the entire syndrome essential tremor. When torticollis with dystonic components occurs with postural tremors of the upper extremities, regard the entire syndrome as a form of cervical dystonia.
• Nondystonic torticollis can occur as an abnormal head position due to spinal deformity. In these patients, no palpable muscle hypertonus or hypertrophy and no record of sensory tricks should be present.

DIFFERENTIALS

Section 4 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Spinal deformity - Early childhood "dropped head syndrome" seen in myopathies and myasthenia, may mimic anterocollis
Juvenile cerebral palsy with cervical dystonia
Phenothiazine-induced acute dystonic reactions of childhood
Juvenile-onset Wilson disease - Often dystonic rather than dyskinetic
Juvenile-onset Huntington disease - Often dystonic and cervical
Acquired dystonia of childhood, such as hematoma or other tumor of sternocleidomastoid muscle
Gastroesophageal reflux (Sandifer syndrome) producing rapid flexion and odd postures reminiscent of torticollis subtypes

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• When a positive family history suggests a familial dystonia rather than idiopathic cervical dystonia, DNA tests for specific genetic dystonias are available that use polymerase chain reaction to detect the DNA in blood samples.

Imaging Studies

• Plain cervical spine films are useful in distinguishing sequelae of bony buildup and scoliosis or spondylosis secondary to chronic dystonia from structural changes of the spine that may mimic cervical dystonia per se (ie, nondystonic torticollis).
• MRI of the cervical cord is useful in documenting cord impingement leading to either spinal stenosis or multiple radiculopathy, all of which can be secondary to bony changes from chronic dystonia.
• Contrast swallowing studies can be performed in consultation with a speech pathologist to evaluate and treat patients for swallowing disorders that accompany cervical dystonia. Indications are to plan botulinum toxin injections, which, if too extensive, may worsen the swallowing mechanism.
• Cranial imaging (CT or MRI) of cervical dystonias is indicated when the physical examination includes abnormal long tract findings (eg, in pyramidal tracts), ophthalmoplegia, and/or dementia.

Procedures

• Electromyography is useful in distinguishing myopathic from neuropathic processes.
• • Myopathic upper girdle muscles versus dystonic hypertrophied upper girdle muscles
  • Multiple cervical root entrapment (polyradiculopathy) versus brachial plexus or single nerve involvement versus combinations of the above associated with bony cervical changes from dystonia

TREATMENT

Section 6 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical Care

A comprehensive approach to the medical care of torticollis includes several treatment goals.

• Dystonia-reducing medication trials
• • In torticollis, the conventional dopamine agonists and antagonists are not effective (dopamine receptors D1=D2), as distinguished from the L-dopa–responsive dystonias (a set of familial generalized dystonias).
  • Anticholinergics (eg, trihexyphenidyl, benztropine) may be slightly effective but less so than in generalized or torsion dystonias.
  • Try unconventional dopamine agonists (dopamine receptors D2>D1).
  • Try glutamate release inhibitors (eg, riluzole) or glutamate receptor blockers (eg, high-dose amantadine, lamotrigine, memantine).
  • Botulinum toxin injection is the current popular treatment of choice.
• Selective medication choices
• • Clonazepam, especially if blepharospasm is part of the syndrome
  • Baclofen, especially if oromandibular dystonia is part of the syndrome
• Nonspecific medication choices
• • Propranolol or primidone if prominent oscillatory components
  • Nonsteroidal anti-inflammatory drugs (NSAIDs) for arthritic components
• Physical medicine options
  • Patients may respond well to sensory feedback training.
  • Muscle relaxation techniques may be tried.
  • Cervical braces occasionally are made that reproduce the tactile advantage of a sensory trick, which controls movement and/or reduces dystonia.

Surgical Care

Surgical care has been tried as a last resort for patients whose symptoms are refractory to botulinum injections.

• Selective ramisectomy for cervical musculature
• Deep brain stimulator implants in the globus pallidus to control contralateral dystonia

Activity

Certain motor activities or prolonged postural vocational requirements may exacerbate pain. An ergonomics evaluation in the workplace can be helpful. Changing or selecting positions can also be beneficial (ie, sitting to the left or right of a speaker to avoid cervical strain).

MEDICATION

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• Follow-up
• Miscellaneous
• Multimedia
• References

The goals of pharmacotherapy are to reduce morbidity and prevent complications. Medication categories are as follows: (1) dystonia reducing (eg, trihexyphenidyl, pramipexole, glutamate release inhibitors and receptor blockers, botulinum toxin) and (2) selective adjunctive (eg, clonazepam for blepharospasm, baclofen for oromandibular dystonia, propranolol or primidone for prominent tremor).

Drug Category: Anticholinergics

These agents reduce dystonia.

Drug Category: Dopamine agonist

Agents with high potency at the D2 receptor, relative to lower potency at the D1 receptor, can be used to enhance activity in the indirect pallidal outflow pathway (see Image 1). This is especially useful in treating the cervical dystonias.

Drug Name	Ropinirole hydrochloride (Requip)
Description	Nonergot dopamine agonist that has high relative in vitro specificity and full intrinsic activity at D2 subfamily of dopamine receptors, binding with higher affinity to D3 than to D2 or D4 receptor subtypes. Has moderate affinity for opioid receptors. Metabolites have negligible affinity for dopamine D1, 5-HT1, 5-HT2, benzodiazepine, GABA, muscarinic, alpha1-, alpha2-, and beta-adrenoreceptors. Precise mechanism of action as treatment for Parkinson disease unknown. However, possibly related to stimulation of dopamine receptors in striatum. To avoid malignant hyperthermic complications when stopping the drug, discontinue gradually over 7-d period. Decrease frequency of administration from 3 tid to bid for 4 d. For the remaining 3 d, decrease frequency to once daily prior to complete withdrawal. Serves as alternative to pramipexole if that drug has objectionable adverse effects. Dopamine receptor profile similar to that of pramipexole.
Adult Dose	Ascending loading dose necessary, starting with 0.25 mg PO tid for week 1, followed by 0.25-mg increments PO tid per week until minimum effective dose achieved
Pediatric Dose	Not established
Contraindications	Documented hypersensitivity; paroxysmal sleep reactions; refractory visual hallucinations unresponsive to dose reduction; symptomatic hypotension
Interactions	None reported
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Withdrawal emergent malignant hyperthermia has been reported with abrupt discontinuation of similar dopamine agonists; fibrotic complications of long-term use of ergoloid drugs have been reported

Drug Category: Glutamate release inhibitors

Glutamate release inhibition and glutamate receptor blockade are alternatives to potentiating D2 receptors in the indirect pallidal outflow pathway by reducing the glutamate-related excitatory circuit in this outflow pathway (see Image 1).

Drug Name	Amantadine (Symmetrel)
Description	Inhibits N-methyl-D-aspartic acid (NMDA) receptor-mediated stimulation of acetylcholine release in rat striatum. May enhance dopamine release, inhibit dopamine reuptake, stimulate postsynaptic dopamine receptors, or enhance dopamine receptor sensitivity. Glutamate receptor inhibition occurs at high doses only. Use only at 100 mg PO tid (lower doses or frequencies only provide dopamine agonism).
Adult Dose	100 mg PO tid
Pediatric Dose	Not established
Contraindications	Documented hypersensitivity
Interactions	Drugs with anticholinergic or CNS stimulant activity increase amantadine toxicity; the concurrent administration of hydrochlorothiazide plus triamterene with amantadine may increase plasma concentrations of amantadine
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Caution in liver disease, uncontrolled psychosis, eczematoid dermatitis, seizures, and current use of CNS stimulant drugs; reduce dose in renal disease when treating Parkinson disease; do not discontinue this medication abruptly; stop use in mental changes, such as visual hallucinations, inattention, or night terrors; blotchy skin (ie, livedo reticularis) may occasionally occur (may be cosmetically objectionable in some patients and may lead to lower leg swelling if severe; in such cases, rimantadine at comparable doses can eliminate this problem)

Drug Name	Memantine (Namenda, Axura)
Description	N-methyl-D-aspartate (NMDA) antagonist.
Adult Dose	5 mg PO qd initially; gradually titrate to a 10-mg bid target dose (allow at least 1 wk between each dosage increase)
Pediatric Dose	Not indicated
Contraindications	Documented hypersensitivity
Interactions	Coadministration with drugs causing alkaline urine (eg, sodium bicarbonate, carbonic anhydrase inhibitors) may decrease clearance by 80%, thus accumulation and toxicity may occur; coadministration with other NMDA antagonists (eg, amantadine, ketamine, dextromethorphan) may increase toxicity risk; concurrent use with other drugs renally eliminated via tubular secretion (eg, hydrochlorothiazide, triamterene, cimetidine, ranitidine, quinidine, nicotine) may alter plasma levels of either drug
Pregnancy	B - Usually safe but benefits must outweigh the risks.
Precautions	Common adverse effects include dizziness (7%), headache (6%), and constipation (5%); predominantly excreted renally, no data support use with severe renal impairment

Drug Name	Lamotrigine (Lamictal)
Description	Blocks glutamate receptors and inhibits voltage-sensitive sodium channels, leading to stabilization of neuronal membrane. Back-up alternative to amantadine.
Adult Dose	25-100 mg PO tid
Pediatric Dose	Not established
Contraindications	Documented hypersensitivity
Interactions	Acetaminophen increases renal clearance of medication, decreasing effects; similarly, phenobarbital and phenytoin increase lamotrigine metabolism, causing a decrease in lamotrigine levels; administration of valproic acid with lamotrigine increases half-life; succinimide anticonvulsants (eg, methsuximide, phensuximide) decrease lamotrigine levels
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Caution in impaired renal or hepatic function

Drug Category: Adrenergic beta-blockers

These agents offer antitremor action when overt tremor complicates torticollis.

Drug Category: Anticonvulsants

Primidone is used in low doses for antitremor effect.

Drug Name	Benztropine (Cogentin)
Description	By blocking striatal cholinergic receptors, may help balance cholinergic and dopaminergic activity in striatum. Can be used as alternative to trihexyphenidyl.
Adult Dose	4-15 mg PO in divided doses
Pediatric Dose	Not established
Contraindications	Documented hypersensitivity; angle-closure glaucoma; stenosing peptic ulcers; prostatic hypertrophy or bladder neck obstruction; myasthenia gravis; pyloric or duodenal obstruction; achalasia (megaesophagus); megacolon
Interactions	Decreases effects of levodopa; increases effects of narcotic analgesics, phenothiazines, quinidine, tricyclic antidepressants, and anticholinergics
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	May exacerbate hypertension, tachycardia, cardiac arrhythmias, liver or kidney disorders, hypotension, prostatic hypertrophy, urinary retention, and obstructive disease of GI/GU tract; in extrapyramidal reactions resulting from phenothiazine treatment in psychiatric patients, toxic psychosis may occur

Drug Category: Nonsteroidal anti-inflammatory agents

These agents have analgesic, anti-inflammatory, and antipyretic activities. Their mechanism of action is not known, but they may inhibit cyclooxygenase activity and prostaglandin synthesis. Other possible mechanisms may include inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell-membrane functions.

Drug Name	Ibuprofen (Motrin, Ibuprin)
Description	DOC for patients with mild to moderately severe pain. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.
Adult Dose	400-800 mg PO tid
Pediatric Dose	Not established
Contraindications	Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency; high risk of bleeding
Interactions	Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; anticoagulants may increase PT; instruct patients to watch for signs of bleeding; may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently
Pregnancy	B - Usually safe but benefits must outweigh the risks.
Precautions	Category D in third trimester of pregnancy; caution in congestive heart failure, hypertension, or decreased renal and hepatic function; caution in coagulation abnormalities or during anticoagulant therapy

Drug Category: Neuromuscular blockers

Paralysis of dystonic muscles by direct injection is used to reduce pain and abnormal posture.

Drug Category: Antispastic/gamma-aminobutyric acid inhibitors

As an inhibitor of the neurotransmitter GABA, baclofen can be used as an adjunctive medication when torticollis is complicated by oromandibular dystonia.

Drug Category: Benzodiazepines

These agents provide adjunctive treatment for patients with blepharospasm.

FOLLOW-UP

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Outpatient Care

• Regular outpatient visits are needed for routine medication checkups and repeat botulinum toxin injections.

Patient Education

• Patients must understand that their condition is expected to wax and wane with emotions and that this phenomenon does not make their condition a psychological problem. Failure to understand this results in a poor self-image and an unnecessary interest in tranquilizers.
• For excellent patient education resources, visit eMedicine's Back, Ribs, Neck, and Head Center. Also, see eMedicine's patient education articles Torticollis and Whiplash.

MISCELLANEOUS

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• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Distinguishing acute cervical trauma from traumatic torticollis may be difficult but is a recurring theme for car accident victims with persisting whiplash symptoms or for patients with industrial injuries when legal interest or chronic pain is an issue. Precise chronologic history is required in providing testimony to distinguish acute cervical trauma from posttraumatic torticollis. To maintain credibility during testimony, consistent statements of chronology are critical and must be prepared by careful review of the medical record by the physician giving testimony.
• • Postconcussive syndrome: Whiplash head and neck injury from rapid acceleration and/or deceleration involves sprained and painful neck muscles, usually on both sides and the posterior muscles, along with global headache, inability to concentrate, and often dizziness and blurred vision.
  • Acute posttraumatic torticollis: Although beginning a few days or immediately following whiplash or other trauma, this can be defined clearly only when the postconcussive syndrome is minimal. When the postconcussive syndrome is of great magnitude and persistent, acute posttraumatic torticollis can be identified clearly only after the acute strain and other postconcussive symptoms are eliminated in time or by analgesic medication (short-term narcotics or NSAIDs). "Residuals" of consistent abnormal head and neck posture with marked limitation of motion are not from the postconcussive syndrome (which is self-limited) but rather from acute posttraumatic torticollis (which is likely to be a chronic syndrome requiring botulinum toxin or a D2 agonist for long-term treatment).
• Delayed posttraumatic torticollis: This is not a recurrence of the postconcussive or whiplash syndrome in the absence of a new injury but an identifiable torticollis syndrome with persistent abnormal posture of head and neck with major limitation in motion. The history of a previous whiplash or postconcussive syndrome establishes the original trauma that may eventually lead to torticollis due to intracranial brain changes in physiology as a delayed response to the original trauma.

MULTIMEDIA

Section 10 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Pallidal outflow pathways from basal ganglia to thalamus. Image courtesy of Norman C. Reynolds, MD, and Wisconsin Medical Journal.
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Media type:  Image

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

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• Comella CL, Tanner CM, DeFoor-Hill L, Smith C. Dysphagia after botulinum toxin injections for spasmodic torticollis: clinical and radiologic findings. Neurology. Jul 1992;42(7):1307-10. [Medline].
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• Jankovic J, Leder S, Warner D, Schwartz K. Cervical dystonia: clinical findings and associated movement disorders. Neurology. Jul 1991;41(7):1088-91. [Medline].
• Jankovic J. Can peripheral trauma induce dystonia and other movement disorders? Yes!. Mov Disord. Jan 2001;16(1):7-12. [Medline].
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Article Last Updated: Sep 28, 2006