Basics
Description
- Torticollis is a symptom, not a disease
- "Twisted neck " ¯ (L. tortus, twisted + collum, neck)
- A fixed or dynamic posturing of the head and neck
- Synonym(s): Cervical dystonia, wry neck
Etiology
Local ‚
- Acute wry neck:
- Develops overnight without provocation
- Most prevalent
- Self-limited, symptoms resolve in 1 to 2 wk
- Cervical spine disease
- Fracture
- Dislocation, subluxation
- Infections
- Spondylosis
- Tumor
- Scar tissue " “producing injuries
- Ligamentous laxity in atlantoaxial region
- Inflammatory disease causing muscular damage:
- Myositis
- Lymphadenitis
- Tuberculosis
- Myasthenia gravis
- Neuritis of the auriculotemporal branch of the trigeminal nerve
- Infections of surrounding soft tissues:
- Nasopharyngeal abscess
- Retropharyngeal abscess
- Cervical adenitis
- Tonsillitis
- Meningitis
- Mastoiditis
- Sinusitis
Compensatory ‚
- Tilt with essential head tremor (patient tilts head to suppress tremor)
- Ocular muscle palsy
Central ‚
- Idiopathic spasmodic torticollis:
- Female > male
- Onset 31 " “60 yr old
- Dystonias:
- Torsion dystonia
- Generalized tardive dystonia
- Wilson disease
- ˇ »-Dopa therapy
- Acute (neuroleptic drugs)
- Strychnine poisoning
Local ‚
- Congenital:
- Odontoid hypoplasia
- Hemivertebrae
- Spina bifida
- Arnold " “Chiari syndrome
- Pseudotumor of infancy
- Hypertrophy or absence of cervical musculature
- Otolaryngologic:
- Vestibular dysfunction
- Otitis media
- Cervical adenitis
- Retropharyngeal abscess
- Pharyngitis
- Mastoiditis
- Esophageal reflux
- Syrinx with spinal cord tumor
- Trauma:
- Cervical fracture/dislocation
- Clavicular fractures
- Pneumomediastinum
- Juvenile rheumatoid arthritis
Compensatory
- Strabismus (4th cranial nerve paresis)
- Congenital nystagmus
- Posterior fossa tumor
Central
Dystonias:
- Torsion dystonia
- Drug induced
- Cerebral palsy
Diagnosis
Signs and Symptoms
- Intermittent painful spasms of sternocleidomastoid (SCM), trapezius, and other neck muscles
- Head is rotated and twisted to 1 direction
- Pure flexion (anterocollis) or extension (retrocollis) is rare:
- Represents symmetric involvement of muscles
- Symptoms usually aggravated by standing, walking, or stressful situations
- Usually does not occur with sleep
History
- Obtain a complete medication history
- The majority of antipsychotic medication " “induced dystonic reactions occur between 12 and 23 hr
- Obtain a complete trauma history
Physical Exam
- Head is rotated and twisted to 1 direction
- Neck movements vary from jerking to smooth
- The presence of fever supports an infectious or inflammatory etiology
- If the neurologic exam is focal, consider spinal cord or CNS disease
- Congenital form:
- A firm, nontender enlargement of the SCM muscle visible at birth
Essential Workup
- Geared toward diagnosing life-threatening etiologies above
- Distinguish torticollis from other causes of neck stiffness (meningismus)
- Cervical spine films to evaluate for fracture except patients with chronic paroxysmal episodes
Diagnosis Tests & Interpretation
Lab
No specific tests helpful ‚
Imaging
- CT or MRI of cervical spine if retropharyngeal abscess or tumor suspected
- High-frequency and color Doppler ultrasonography is the test of choice for congenital muscular torticollis
- Plain films, lateral and AP view for acquired torticollis resulting from trauma
- CT scan of the neck or cervical spine in nontraumatic acquired torticollis
Diagnostic Procedures/Surgery
- Consider administering an anticholinergic medication if drug-induced etiology is suspected
- Consider performing the Tensilon test if myasthenia gravis is a consideration
Differential Diagnosis
- CNS infections
- Tumors of soft tissue or bone
- Basal ganglia disease
- Abscess of cervical glands
- Myositis of cervical muscles
- Cervical disk lesions
- Myasthenia gravis
Treatment
Pre-Hospital
- Ensure patent airway
- Cervical spine precautions for any history of trauma
- Support head
Initial Stabilization/Therapy
Cervical spine immobilization if fracture is suspected ‚
Ed Treatment/Procedures
- Drug (e.g., phenothiazine) induced:
- Diphenhydramine or benztropine
- Acquired:
- Soft collar and rest
- Physical therapy
- Massage
- Local heat
- Analgesics
Medication
- Benztropine (for drug-related dystonia): 1 " “2 mg IM or slow IV, followed by 3 " “5 days PO
- Clonazepam (2nd-line drug): 0.5 mg PO TID
- Diphenhydramine (for drug-related dystonia): 25 " “50 mg IV or IM followed by 3 " “5 days PO q6 " “8h; (peds: 5 mg/kg/24 h div. q6h IV, IM, or PO)
- Trihexyphenidyl (a 1st-line drug): 2 " “5 mg/d PO, advance to 30 mg/d
- Valium: 2 " “5 mg IV, 2 " “10 mg PO TID (peds: 0.1 " “0.2 mg/kg/dose IV or PO q6h)
- Botulinum toxin is the 1st-line agent for treating nondrug-induced torticollis, though this is not typically administered in the ED setting
Follow-Up
Disposition
Admission Criteria
- Cervical spine fracture
- Diagnosis in doubt
- Infectious causes
- Toxic appearance
- Inability to maintain adequate fluid intake
- Lack of support system
Issues for Referral
Some patients who fail medical treatment may benefit from surgical treatment, such as accessory nerve ablation or deep brain stimulation ‚
Follow-Up Recommendations
- Outpatient referral to an orthopedist, neurologist, or neurosurgeon who uses botulinum toxin in his or her practice
- Physical therapy and consider chiropractic care
- Return to ED for weakness or worsening symptoms
Pearls and Pitfalls
Exclude infectious, inflammatory, traumatic, spinal cord and CNS causes of torticollis. ‚
Additional Reading
- Haque ‚ S, Bilal Shafi ‚ BB, Kaleem ‚ M. Imaging of torticollis in children. Radiographics. 2012;32:557 " “571.
- Ropper ‚ AH, Samuels ‚ MA, eds. Adams and Victors Principles of Neurology, 9th ed. New York, NY: McGraw-Hill; 2009.
- Shanker ‚ V, Bressman ‚ SB. What's new in dystonia? Curr Neurol Neurosci Rep. 2009;9:278 " “284.
- Simpson ‚ DM, Blitzer ‚ A, Brashear ‚ A, et al. Assessment: Botulinum neurotoxin for the treatment of movement disorders (an evidence-based review): Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2008;70:1699 " “1706.
Codes
ICD9
- 333.83 Spasmodic torticollis
- 723.5 Torticollis, unspecified
- 754.1 Congenital musculoskeletal deformities of sternocleidomastoid muscle
- 781.93 Ocular torticollis
ICD10
- G24.3 Spasmodic torticollis
- M43.6 Torticollis
- Q68.0 Congenital deformity of sternocleidomastoid muscle
- R29.891 Ocular torticollis
SNOMED
- 70070008 torticollis (disorder)
- 74333002 Spasmodic torticollis (disorder)
- 268240006 Congenital torticollis
- 34241000119104 Ocular torticollis (disorder)
- 270476009 wry neck/torticollis (disorder)