Grisel's syndrome is a non-traumatic

subluxation A subluxation is an incomplete or partial dislocation of a joint or organ. According to the World Health Organization, a subluxation is a "significant structural displacement" and is therefore visible on static imaging studies, such as X-rays. ...

of the

atlanto-axial joint The atlanto-axial joint is a joint in the upper part of the neck between the atlas bone and the axis bone, which are the first and second cervical vertebrae. It is a pivot joint, that can start from C2 To C7. Structure The atlanto-axial joint ...

caused by

inflammation Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', '' ...

of the adjacent tissues. This is a rare disease that usually affects children. Progressive throat and neck pain and neck stiffness can be followed by

neurologic Neurology (from , "string, nerve" and the suffix -logia, "study of") is the branch of medicine dealing with the diagnosis and treatment of all categories of conditions and disease involving the nervous system, which comprises the brain, the s ...

symptoms such as pain or numbness radiating to arms ( radiculopathies). In extreme cases, the condition can lead to quadriplegia and even death from acute respiratory failure. The condition often follows soft tissue inflammation in the neck such as in cases of upper respiratory tract infections, peritonsillar or

retropharyngeal abscess Retropharyngeal abscess (RPA) is an abscess located in the tissues in the back of the throat behind the posterior pharyngeal wall (the retropharyngeal space). Because RPAs typically occur in deep tissue, they are difficult to diagnose by physical ...

es. Post-operative inflammation after certain procedures such as

adenoidectomy Adenoidectomy is the surgical removal of the adenoid for reasons which include impaired breathing through the nose, chronic infections, or recurrent earaches. The effectiveness of removing the adenoids in children to improve recurrent nasal sympt ...

can also lead to this condition in susceptible individuals such as those with Down syndrome.

Pathophysiology

Pathophysiology Pathophysiology (or physiopathology) is a branch of study, at the intersection of pathology and physiology, concerning disordered physiological processes that cause, result from, or are otherwise associated with a disease or injury. Pathology is ...

of this disease consists of relaxation of the transverse ligament of the atlanto-axial joint.

Diagnosis

Diagnosis can be established using plain film

x-rays An X-ray (also known in many languages as Röntgen radiation) is a form of high-energy electromagnetic radiation with a wavelength shorter than those of ultraviolet rays and longer than those of gamma rays. Roughly, X-rays have a wavelength ran ...

as well as

CT scan A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or ...

of the neck/cervical spine. Children with Down syndrome have inherently lax ligaments making them susceptible to this condition. In select cases, these children may require pre-operative imaging to assess the risk for complications after procedures such as

Treatment

Treatment includes anti-inflammatory medications and immobilization of the neck in addition to treatment of the offending infectious cause (if any) with appropriate

antibiotics An antibiotic is a type of antimicrobial substance active against bacteria. It is the most important type of antibacterial agent for fighting pathogenic bacteria, bacterial infections, and antibiotic medications are widely used in the therapy ...

. Early treatment is crucial to prevent long-term

sequelae A sequela (, ; usually used in the plural, sequelae ) is a pathological condition resulting from a disease, injury, therapy, or other trauma. Derived from the Latin word meaning "sequel", it is used in the medical field to mean a complication or c ...

. Surgical fusion may be required for residual instability of the joint.

References

* Grisel P. ''Enucléation de l'atlas et torticollis naso-pharyngien'' Presse Med 1930;38:50–4. * Mathern GW, Batzdorf U. ''Grisel's syndrome: Cervical spine clinical, pathologic, and neurologic manifestations''. Clin Orthop Relat Res. 1989 Jul;(244):131-46. * C Bocciolini, D Dall'Olio, E Cunsolo, PP Cavazzuti, and P Laudadio, ''Grisel's syndrome: a rare complication following adenoidectomy'', Acta Otorhinolaryngol Ital. 2005 August; 25(4): 245–249.

External links

{{Medical resources , DiseasesDB = 32750 , ICD10 = , ICD9 = {{ICD9, 723.5 , ICDO = , OMIM = , MedlinePlus = , eMedicineSubj = orthoped , eMedicineTopic = 503 , MeshID = Musculoskeletal disorders Syndromes