Description

• Torticollis is a spectrum of disorders characterized by head tilt with rotation or involuntary movement.
• Pediatric disorders include:
  • Congenital muscular torticollis (CMT): 80% of all infants presenting with torticollis are found to have CMT. CMT is seen at birth or early infancy and results from unilateral fibrosis and shortening of the sternocleidomastoid (SCM) muscle.
  • Acquired torticollis
• Adult disorders include:
  • Acquired torticollis (also known as wryneck), which is usually self-limited
  • Spasmodic torticollis, also known as cervical dystonia, is caused by recurrent involuntary muscular contractions.
• Other forms (oculogyric, gastroesophageal reflux, arthritis-related, scoliosis-related, and hysterical torticollis) are not discussed.
• System(s) affected: Musculoskeletal; Nervous.
• Synonym(s): Acute wryneck; Idiopathic generalized torticollis; SCM torticollis; Neonatal torticollis; Idiopathic cervical dystonia; Focal dystonia; Nuchal dystonia.

Epidemiology

• ~90% of cases occur in individuals aged 31–60.
• Predominant age: CMT: Newborn and infants; pediatric acquired torticollis, <10 years; adult acquired torticollis, 30–60 years; spasmodic torticollis, 30–50 years (mean age 40–43) (1)
• Predominant sex: Spasmodic torticollis, Female > Male (1.6:1); congenital muscular, Male > Female (3:2) (2)

Incidence
Congenital: Up to 1/250 births (2); spasmodic, estimated at 1/100,000; overall incidence for torticollis is 24/1 million persons.

Prevalence
All focal dystonias combined: 295/1 million persons; no reliable data for pediatric and adult acquired torticollis

Risk Factors

• CMT: Intrauterine crowding, breech position, ischemia, birth injury
• Pediatric acquired torticollis: Soft-tissue inflammation or infection, neurologic conditions, visual disturbances, trauma
• Adult acquired torticollis: Stress, unusual positioning (particularly when sleeping), exposure to cold drafts of air, medications, trauma, infection
• Spasmodic: Family history of dystonia, soft-tissue inflammation or infection, neurologic conditions, visual disturbances, trauma

Genetics
Some forms may have genetic basis, such as spasmodic torticollis (1).

Etiology

• CMT:
  • Intrauterine malpositioning may lead to trauma of the SCM and fibrosis.
  • Birth trauma, such as a clavicular fracture
• Pediatric acquired torticollis: Pain, spasm, decreased range of motion without trauma
• Adult acquired torticollis:
  • Emotional stress, postural factors (e.g., work, sleep, lying while reading or watching TV, prolonged unusual positioning of neck), or exposure to cold; many cases are idiopathic
  • Medication reactions (e.g., amphetamines, haloperidol, chlorpromazine, ketamine)
• Spasmodic torticollis:
  • Muscular damage from inflammatory or infectious diseases
  • Cervical spine injuries and spondylosis
  • Ocular disorders
  • Organic CNS disorders
  • Psychogenic
  • Tumors
  • Vestibular dysfunction

Pediatric Considerations
Congenital: Associated with birth injury that, without treatment, becomes a fibrous cord and may be associated with persistent craniofacial deformities.

Commonly Associated Conditions

• >80% of infants with CMT also present with craniofacial asymmetry, deformational plagiocephaly of varying degrees, and developmental hip dysplasia.
• Congenital and pediatric acquired torticollis: Consider Klippel-Feil syndrome (congenital fusion of cervical vertebrae).
• Pediatric and adult acquired torticollis: Spinal abnormalities
• Spasmodic torticollis: Treatment of psychiatric disorders