Torticollis, Emergency Medicine

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Torticollis, Emergency Medicine

Basics

Description

No specific tests helpful ‚

Some patients who fail medical treatment may benefit from surgical treatment, such as accessory nerve ablation or deep brain stimulation ‚

Exclude infectious, inflammatory, traumatic, spinal cord and CNS causes of torticollis. ‚

• 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

• Acute wry neck:Develops overnight without provocationMost prevalentSelf-limited, symptoms resolve in 1 to 2 wkCervical spine diseaseFractureDislocation, subluxationInfectionsSpondylosisTumorScar tissue " “producing injuriesLigamentous laxity in atlantoaxial region
• Inflammatory disease causing muscular damage:MyositisLymphadenitisTuberculosisMyasthenia gravisNeuritis of the auriculotemporal branch of the trigeminal nerve
• Infections of surrounding soft tissues:Nasopharyngeal abscessRetropharyngeal abscessCervical adenitisTonsillitisMeningitisMastoiditisSinusitis

• 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

• Myositis
• Lymphadenitis
• Tuberculosis
• Myasthenia gravis
• Neuritis of the auriculotemporal branch of the trigeminal nerve

• Nasopharyngeal abscess
• Retropharyngeal abscess
• Cervical adenitis
• Tonsillitis
• Meningitis
• Mastoiditis
• Sinusitis

• Tilt with essential head tremor (patient tilts head to suppress tremor)
• Ocular muscle palsy

• Idiopathic spasmodic torticollis:Female > maleOnset 31 " “60 yr old
• Dystonias:Torsion dystoniaGeneralized tardive dystoniaWilson disease ˇ »-Dopa therapyAcute (neuroleptic drugs)Strychnine poisoning

• Female > male
• Onset 31 " “60 yr old

• Torsion dystonia
• Generalized tardive dystonia
• Wilson disease
• ˇ »-Dopa therapy
• Acute (neuroleptic drugs)
• Strychnine poisoning

• Congenital:Odontoid hypoplasiaHemivertebraeSpina bifidaArnold " “Chiari syndromePseudotumor of infancyHypertrophy or absence of cervical musculature
• Otolaryngologic:Vestibular dysfunctionOtitis mediaCervical adenitisRetropharyngeal abscessPharyngitisMastoiditisEsophageal refluxSyrinx with spinal cord tumor
• Trauma:Cervical fracture/dislocationClavicular fracturesPneumomediastinum
• Juvenile rheumatoid arthritisCompensatory

• Odontoid hypoplasia
• Hemivertebrae
• Spina bifida
• Arnold " “Chiari syndrome
• Pseudotumor of infancy
• Hypertrophy or absence of cervical musculature

• Vestibular dysfunction
• Otitis media
• Cervical adenitis
• Retropharyngeal abscess
• Pharyngitis
• Mastoiditis
• Esophageal reflux
• Syrinx with spinal cord tumor

• Cervical fracture/dislocation
• Clavicular fractures
• Pneumomediastinum

Compensatory

• Strabismus (4th cranial nerve paresis)
• Congenital nystagmus
• Posterior fossa tumorCentralDystonias:

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

• Represents symmetric involvement of muscles

• Obtain a complete medication history
• The majority of antipsychotic medication " “induced dystonic reactions occur between 12 and 23 hr
• Obtain a complete trauma history

• 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

• 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

• 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

• 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

Ed Treatment/Procedures

• Drug (e.g., phenothiazine) induced:Diphenhydramine or benztropine
• Acquired:Soft collar and restPhysical therapyMassageLocal heatAnalgesics

• Diphenhydramine or benztropine

• 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

• Cervical spine fracture
• Diagnosis in doubt
• Infectious causes
• Toxic appearance
• Inability to maintain adequate fluid intake
• Lack of support system

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

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)