Rosen & Barkin's 5-Minute Emergency Medicine Consult (719 page)

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
    
  • λ-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

• 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
  

• 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
    

• 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
  

No specific tests helpful

• 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
  

• 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

• Ensure patent airway
  
• Cervical spine precautions for any history of trauma
  
• Support head
  

Cervical spine immobilization if fracture is suspected

• Drug (e.g., phenothiazine) induced:
  
  • Diphenhydramine or benztropine
    
• Acquired:
  
  • Soft collar and rest
    
  • Physical therapy
    
  • Massage
    
  • Local heat
    
  • Analgesics
    

• 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

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

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

• 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
  

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

• Haque S, Bilal Shafi BB, Kaleem M. Imaging of torticollis in children.
  Radiographics
  . 2012;32:557–571.
  
• Ropper AH, Samuels MA, eds.
  Adams and Victor’s 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

• 333.83 Spasmodic torticollis
  
• 723.5 Torticollis, unspecified
  
• 754.1 Congenital musculoskeletal deformities of sternocleidomastoid muscle
  

BASICS

• One of the most fulminant and potentially fatal of all dermatologic disorders
  
• Skin sloughing at the dermal–epidermal interface results in the equivalent of a 2nd-degree burn
  
• Can affect up to 100% of total body surface area (BSA)
  
• May extend to involve:
  
  • GI mucosa
    
  • Respiratory mucosa
    
  • Genitourinary/renal epithelium
    
• Mechanism unclear, research indicates immunologic, cytotoxic, and delayed hypersensitivity may be involved as well as genetic susceptibility
  
• Current classification system proposes 3 categories within the spectrum of Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), distinct from erythema multiforme major and based on percentage of total BSA:
  
  • SJS: <10% of BSA
    
  • SJS–TEN overlap syndrome: 10–30% of BSA
    
  • TEN: >30% of BSA, can affect up to 100% BSA
    
• More common in older patients and immunocompromised patients
  
• Mortality rate is about 30%, usually due to secondary sepsis from
  Staphylococcus aureus
  and
  Pseudomonas aeruginosa
  
• Synonym(s):
  
  • Lyell syndrome
    
  • Fixed drug necrolysis
    
  • Epidermolysis necroticans combustiformis
    
  • Epidermolysis bullosa