A case of adult-onset Grisel's syndrome

Br J Neurosurg. 2022 Apr 8:1-3. doi: 10.1080/02688697.2022.2061420. Online ahead of print.

Abstract

Aim: Grisel's syndrome is a non-traumatic subluxation of the atlanto-axial joint that occurs after infection or inflammation in the otolaryngological area, primarily in children.Method: This report describes the clinical characteristics of an extremely rare case of adult-onset Grisel's syndrome.Result: A 77-year-old woman presented with neck and bilateral shoulder pain and stiffness. Her temperature was 37.6 °C. Blood testing revealed a mildly elevated inflammatory response, although blood cultures were negative. Computed tomography (CT) showed atlanto-axial subluxation and joint destruction. T2-weighted magnetic resonance imaging (MRI) displayed high signals in the soft tissues in the anterior space of the atlas and axis, posterior wall of the pharynx, and interspinous ligament, indicating spinal cord compression at the C1 level. Differential diagnoses of inflammation and diseases causing atlanto-axial subluxation included rheumatoid arthritis, amyloidosis, pyogenic spondylitis due to posterior pharyngeal abscess, and crowned dens syndrome. After the systematic elimination of each condition, we considered Grisel's syndrome and began non-surgical treatment with intravenous antibiotics and a Philadelphia neck collar. Her inflammatory response and neck pain gradually decreased. Six months later, there was no progression of instability. She was able to walk unaided and live normally with the use of a neck collar as needed.Conclusion: Grisel's syndrome occurs predominantly in children, but can also afflict adults. Since early diagnosis and treatment can improve symptoms in some cases and prevent progressive atlanto-axial instability, prompt evaluation of the atlanto-axial joint using CT or MRI is advised in patients with neck pain and limited range of motion.

Keywords: Grisel’s syndrome; antibiotic therapy; atlanto-axial subluxation; inflammation; upper cervical spine.