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Grisel syndrome in pediatric rheumatic diseases as a rarely recognized complication: case-based review
Lutfiye Koru1, Begumhan Baysal2, Sabriye Gulcin Bozbeyoglu2
1Department of Pediatric Rheumatology, Faculty of Medicine, Istanbul Medeniyet University, Istanbul, Turkey.
Insights
Grisel syndrome (GS), a rare neck instability, can occur without infection or surgery. Systemic inflammation alone may cause GS, highlighting the need for early diagnosis in children with inflammatory conditions and neck symptoms.
Area of Science:
- Rheumatology
- Pediatrics
- Neurology
Background:
- Grisel syndrome (GS) is a rare, non-traumatic atlantoaxial rotatory subluxation primarily affecting children.
- GS can lead to severe neurological deficits, including quadriplegia and respiratory arrest.
- Typically, GS follows upper respiratory infections or head/neck surgery, though associations with systemic inflammatory diseases exist.
Purpose of the Study:
- To report two cases of GS developing without infectious or surgical triggers.
- To highlight the role of systemic inflammation alone in causing atlantoaxial instability.
- To review literature on GS associated with rheumatologic diseases.
Main Methods:
- Case report of two pediatric patients with GS.
- Literature review of GS in the context of rheumatologic diseases.
Main Results:
- Two cases of GS were identified: one associated with juvenile idiopathic arthritis (JIA) and another with familial Mediterranean fever (FMF).
- Neither case had preceding infectious or surgical triggers.
- Literature review confirmed limited but existing reports of GS in rheumatologic conditions.
Conclusions:
- Systemic inflammation, as seen in JIA and FMF, can independently cause atlantoaxial instability leading to GS.
- Clinicians should consider GS in pediatric patients with systemic inflammatory diseases presenting with neck pain or torticollis, even without infection or surgery.
- Early recognition and management of GS are vital to prevent neurological sequelae.
Abstract:
Grisel syndrome (GS) is a rare, non-traumatic atlantoaxial rotatory subluxation that predominantly affects children and can lead to severe neurological complications such as quadriplegia, radiculopathy, and respiratory arrest. It typically occurs following upper respiratory tract infections or head and neck surgery. Although GS associated with systemic inflammatory diseases such as Kawasaki disease (KD) and acute rheumatic fever (ARF) has been reported, these cases usually involve an underlying infectious trigger. In contrast, we report two cases of GS that developed without any infectious or surgical triggers: one associated with juvenile idiopathic arthritis (JIA) and the other with familial Mediterranean fever (FMF). These cases highlight that systemic inflammation alone may be sufficient to induce atlantoaxial instability, emphasizing the need for clinicians to consider GS in patients with systemic inflammatory conditions presenting with neck pain or torticollis, even in the absence of infection or surgical history. Early recognition and prompt management are crucial for preventing serious neurological sequelae. Due to the limited number of GS cases reported in association with rheumatologic diseases, this report also includes a review of the literature to provide a broader perspective on this rare but serious complication.
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