Ann Rehabil Med.  2017 Jun;41(3):511-515. 10.5535/arm.2017.41.3.511.

A Case of Grisel Syndrome Showing No Underlying Laxity of the Atlanto-axial Joint

• Ahn AR
• Park YH
• Park EJ
• Yim SY

• KMID: 2389470
• DOI: http://doi.org/10.5535/arm.2017.41.3.511

Abstract

Grisel syndrome is a rare, non-traumatic atlanto-axial subluxation associated with an inflammatory or infectious process in the upper neck. According to the two-hit hypothesis, which is widely accepted for the pathogenesis of Grisel syndrome, preexisting ligamentous laxity of the atlanto-axial joint is regarded as the first hit. An inflammatory or infectious process of the atlanto-axial joint acts as the second hit, resulting in non-traumatic atlanto-axial subluxation. We report on a 6-year-old girl with atlanto-axial subluxation following retropharyngeal and cervical lymphadenitis. She was diagnosed with Grisel syndrome, for which an initial computed tomography did not show any preexisting ligamentous laxity of the atlanto-axial joint. A literature review found only 4 case reports on Grisel syndrome with an initially normal atlanto-axial joint. The present case offers some evidence that a single hit, such as inflammatory changes in the atlanto-axial joint, might cause Grisel syndrome, even without underlying ligamentous laxity.

Keyword

Atlanto-axial joint; Torticollis; Joint instability

MeSH Terms

Atlanto-Axial Joint*
Child
Female
Humans
Joint Instability
Ligaments
Lymphadenitis
Neck
Torticollis

Figure

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  Fig. 1 Serial neck computed tomography (CT). (A) The initial CT on admission shows a normal alignment of the atlanto-axial joint with symmetric lateral atlanto-dens interval and without rotation of the atlanto-axial joint. (B) CT taken after the onset of torticollis shows rotation of the atlanto-axial joint (blue curved arrow) with a lateral shift of the dens toward the right (yellow arrow). (C) CT on the 28th hospital day shows a normal alignment of the atlanto-axial joint. C1, the atlas; C2, the axis; blue line, a line connecting bilateral transverse foramina of the atlas and the axis; yellow dotted line, the lateral atlanto-dens interval.

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  Fig. 2 A 6-year-old girl with Grisel syndrome showing abnormal head posture.

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  Fig. 3 Radiographic studies showing inflammatory changes of the soft tissue. (A) Computed tomography with contrast shows a diffuse, low attenuating lesion in the retropharyngeal space, indicating a fluid collection due to inflammation (white arrow). (B) Axial gadolinium-enhanced T1-weighted magnetic resonance imaging (MRI) shows a fluid collection in the retropharyngeal space (white arrow). (C) Sagittal gadolinium-enhanced T1-weighted MRI shows abnormal enhancement of ligaments of the atlanto-axial joint (black arrow) and posterior longitudinal ligament at the level of the C2 vertebra (black arrow head), suggesting inflammatory changes of the soft tissue. (D) Sagittal T2-weighted MRI shows a fluid collection in the atlanto-axial joint space (white arrow head).

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