Advance Osteopathy
Grisel’s syndrome

Grisel’s syndrome is a rare but potentially serious condition characterized by non-traumatic atlantoaxial rotatory subluxation (AARS), involving abnormal rotation or displacement between the first cervical vertebra (atlas, C1) and the second cervical vertebra (axis, C2). Unlike traumatic cervical instability, Grisel’s syndrome develops secondary to inflammation or infection in the head and neck region, often following upper respiratory tract infections or otolaryngological procedures.2

Grisel Syndrom

Although predominantly reported in children, adult cases have also been documented.911 Early recognition is essential because delayed diagnosis may lead to chronic deformity, neurological complications, spinal cord compression, or permanent disability.18

Etiology and Pathophysiology

Grisel’s syndrome is most commonly associated with inflammatory conditions affecting the upper respiratory tract, pharynx, tonsils, adenoids, nasopharynx, and surrounding cervical tissues.26 Common triggers include:

  • Tonsillitis and pharyngitis7
  • Otitis media and upper respiratory infections6
  • Mycoplasma pneumoniae infection4
  • Mumps infection12
  • Kawasaki disease10
  • Adenotonsillectomy and other ENT surgeries114
  • Nasopharyngeal surgery9

The most widely accepted mechanism is the “two-hit hypothesis.” First, children naturally possess greater ligamentous laxity at the atlantoaxial joint. Second, inflammatory mediators spread from the pharyngeal region through the pharyngovertebral venous plexus, causing hyperemia, edema, and weakening of the transverse and alar ligaments stabilizing C1 and C2.27

This combination of ligamentous laxity and inflammation may result in atlantoaxial rotatory subluxation without any traumatic event.5

Risk Factors

Several factors increase susceptibility to Grisel’s syndrome:

  • Childhood age group, particularly under 12 years6
  • Recent ENT surgery1
  • Upper respiratory tract infections7
  • Inflammatory disorders such as Kawasaki disease10
  • Congenital ligamentous laxity
  • Down syndrome, which is associated with atlantoaxial instability3

Clinical Symptoms

The hallmark symptom of Grisel’s syndrome is acute torticollis, often developing several days after infection or surgery.13

Common symptoms include:

  • Painful neck stiffness
  • Restricted cervical motion
  • Head tilted to one side and rotated to the opposite side (“Cock-Robin” posture)20
  • Occipital or upper cervical pain
  • Muscle spasm of the sternocleidomastoid muscle
  • Difficulty turning the head
  • Fever or recent history of infection

More severe cases may present with:

  • Neurological deficits
  • Upper extremity weakness
  • Sensory disturbances
  • Spinal cord compression
  • Respiratory compromise in extreme cases18

Children frequently present with persistent torticollis that fails to improve despite standard treatment for muscle strain.8

Diagnosis

Diagnosis requires a combination of clinical suspicion, physical examination, and imaging studies.

Clinical Assessment

Clinicians should suspect Grisel’s syndrome when painful torticollis develops following:

  • Tonsillitis
  • Pharyngitis
  • Otitis media
  • Adenoidectomy
  • Tonsillectomy
  • Other head and neck procedures16
Physical Examination
  • Abnormal head posture
  • Marked restriction of cervical rotation
  • Painful neck movement
  • Muscle guarding and spasm
  • Neurological examination to assess spinal cord involvement
Imaging Studies

Computed tomography (CT) is considered the gold standard for confirming atlantoaxial rotatory subluxation.8 CT accurately demonstrates rotational displacement and allows classification of the severity.

Magnetic resonance imaging (MRI) is useful for evaluating:

  • Ligament injury
  • Inflammatory changes
  • Spinal cord compression
  • Retropharyngeal infection15

Plain radiographs may identify gross instability but are generally less sensitive than CT imaging.2

Differential Diagnosis

Conditions that may mimic Grisel’s syndrome include:

  • Congenital muscular torticollis
  • Cervical muscle strain
  • Cervical disc pathology
  • Traumatic atlantoaxial instability
  • Retropharyngeal abscess
  • Meningitis
  • Cervical tumors
  • Juvenile idiopathic arthritis

Medical Treatment

Treatment depends on the severity and duration of symptoms.

Conservative Management

Most early cases respond well to conservative treatment.615

  • Appropriate antibiotic therapy for underlying infection
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Muscle relaxants
  • Soft cervical collar
  • Bed rest and activity modification

Early diagnosis is associated with excellent outcomes and lower risk of chronic instability.20

Cervical Traction

Patients with persistent subluxation may require cervical traction to restore normal alignment.8 Traction is typically performed under specialist supervision and followed by immobilization.

Surgical Treatment

Surgery may be necessary when:

  • Conservative treatment fails
  • Subluxation becomes chronic
  • Neurological deficits develop
  • Severe instability is present18

Surgical options include:

  • Posterior cervical fusion
  • Internal fixation
  • Reduction of atlantoaxial displacement

Osteopathic Treatment Considerations

Osteopathic assessment may be valuable during the rehabilitation phase after the atlantoaxial instability has been medically stabilized and cleared by the treating physician. Osteopathic treatment is not intended to reduce an active atlantoaxial subluxation and should never replace emergency medical management.

During the acute stage, direct cervical manipulation, high-velocity low-amplitude (HVLA) techniques, and forceful rotational procedures are contraindicated because of the risk of worsening instability or neurological injury.

Appropriate Osteopathic Approaches After Stabilization
  • Gentle myofascial release of cervical and thoracic soft tissues
  • Balanced ligamentous tension techniques
  • Indirect functional techniques
  • Rib cage mobility restoration
  • Thoracic spine mobility improvement
  • Diaphragmatic release and respiratory mechanics optimization
  • Lymphatic drainage techniques to support recovery

These approaches may help reduce muscular guarding, improve cervical mobility, normalize posture, and enhance overall biomechanical function once the cervical spine is confirmed stable.2

Rehabilitation and Home Care

Recovery continues after the inflammatory process resolves and cervical stability is restored.

Postural Education
  • Avoid prolonged forward-head posture
  • Maintain neutral neck alignment
  • Use ergonomically appropriate workstations
Gradual Mobility Exercises

Gentle physician-approved range-of-motion exercises can help restore normal cervical movement while avoiding excessive rotation.

Activity Modification
  • Avoid contact sports during recovery
  • Avoid sudden neck movements
  • Gradually return to normal activities
Monitoring for Recurrence

Patients should seek prompt medical attention if symptoms recur, including:

  • New torticollis
  • Neck pain
  • Neurological symptoms
  • Progressive movement restriction

Prognosis

The prognosis of Grisel’s syndrome is generally excellent when recognized and treated early.620 Delayed diagnosis increases the risk of persistent deformity, chronic instability, and neurological complications requiring surgical intervention.18

Healthcare providers should maintain a high index of suspicion in children or adults presenting with painful torticollis following recent head and neck infection or otolaryngological surgery.1

References

1. Al-Driweesh T, Altheyab F, Alenezi M, Alanazy S, Aldrees T. Grisel's syndrome post otolaryngology procedures: A systematic review. Int J Pediatr Otorhinolaryngol. 2020;137:110225.

2. Wetzel FT, La Rocca H. Grisel's syndrome. Clin Orthop Relat Res. 1989;(240):141-152.

3. Shayan-Moghadam R, Sharafi M, Violas P, Nabian MH, Mehrpour SR. Grisel's syndrome and Down syndrome: a case report. Int J Burns Trauma. 2023;13(2):94-98.

4. Falsaperla R, Piattelli G, Marino S, Marino SD, Fontana A, Pavone P. Grisel's syndrome caused by Mycoplasma pneumoniae infection: a case report and review of the literature. Childs Nerv Syst. 2019;35(3):523-527.

5. Guleryuz A, Bagdatoglu C, Duce MN, Talas DU, Celikbas H, Köksel T. Grisel's syndrome. J Clin Neurosci. 2002;9(1):81-84.

6. Karkos PD, Benton J, Leong SC, Mushi E, Sivaji N, Assimakopoulos DA. Grisel's syndrome in otolaryngology: a systematic review. Int J Pediatr Otorhinolaryngol. 2007;71(12):1823-1827.

7. Khodabandeh M, Shakiba S, Alizadeh S, Eshaghi H. Grisel's syndrome associated with tonsillitis. IDCases. 2019;15:e00470.

8. Iaccarino C, Francesca O, Piero S, Monica R, Armando R, de Bonis P, et al. Grisel's Syndrome: Non-traumatic Atlantoaxial Rotatory Subluxation-Report of Five Cases and Review of the Literature. Acta Neurochir Suppl. 2019;125:279-288.

9. Chua AJK, Tan BWS, Tan TY, Heah HHW. Grisel's Syndrome in an Adult After Endoscopic Nasopharyngectomy. Laryngoscope Investig Otolaryngol. 2019;4(5):504-507.

10. Liu X, Zhou K, Hua Y, Wu M, Liu L, Shao S, Wang C. Grisel's syndrome in Kawasaki disease. Orphanet J Rare Dis. 2020;15(1):246.

11. Nakai A, Uehara M, Miyaoka Y, Oba H, Ikegami S, Takizawa T, et al. A case of adult-onset Grisel's syndrome. Br J Neurosurg. 2024;38(6):1319-1321.

12. Shen Y, Yang L, Liu X, Xie Y, Dai X, Wang C. Grisel's syndrome associated with mumps: A case report. Front Pediatr. 2022;10:916538.

13. Ortiz GL, Pratts I, Ramos E. Grisel's syndrome: an unusual cause of torticollis. J Pediatr Rehabil Med. 2013;6(3):175-180.

14. Park JA, Yi HJ, Kang JW, Kim JH. Grisel's syndrome developed after adenotonsillectomy. Br J Hosp Med (Lond). 2019;80(9):ii.

15. Al-Hussain OH, Al-Hussain G. Diagnostic Approach and Treatment Options for Pediatric Cases of Grisel's Syndrome Post Otolaryngology Procedure: A Systematic Review. Cureus. 2024;16(1):e51739.

18. Mathern GW, Batzdorf U. Grisel's syndrome. Cervical spine clinical, pathologic, and neurologic manifestations. Clin Orthop Relat Res. 1989;(244):131-146.

20. Galer C, Holbrook E, Treves J, Leopold D. Grisel's syndrome: a case report and review of the literature. Int J Pediatr Otorhinolaryngol. 2005;69(12):1689-1692.

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