What are the clinical features of Grisel's syndrome?

Clinical Features of Grisel's Syndrome

Grisel's syndrome is a nontraumatic atlantoaxial rotatory subluxation that characteristically presents with painful torticollis following head and neck infections or otolaryngological procedures, predominantly affecting children. 1, 2

Cardinal Clinical Features

Classic Triad of Presentation

• Torticollis (painful, fixed head tilt) is the hallmark presenting sign, typically appearing suddenly after the inciting event 1, 2
• Cervical pain accompanies the abnormal head position and is often severe enough to limit neck movement 1, 3
• Recent history of head/neck infection or ENT surgery within days to weeks before symptom onset is nearly universal 2

Temporal Relationship and Triggers

• Head and neck infections account for 59.7% of cases, including pharyngitis, tonsillitis, adenoiditis, retropharyngeal abscess, and cervical lymphadenitis 2, 4
• Otolaryngological procedures (particularly adenoidectomy and tonsillectomy) account for 35.7% of cases, with symptoms typically emerging in the immediate postoperative period 2
• Mean delay in diagnosis is 33 days, highlighting the importance of maintaining high clinical suspicion 2

Demographic and Clinical Characteristics

Age and Sex Distribution

• Predominantly affects children, though rare cases occur in adults (as late as 78 years old in one reported case) 2, 3
• No sex predilection exists, with nearly equal distribution between males (49.7%) and females (50.2%) 2

Physical Examination Findings

• Fixed head rotation with the chin rotated away from the side of subluxation, creating the characteristic "cock-robin" position 2
• Resistance to passive neck movement due to pain and mechanical limitation from the subluxation 1, 5
• Palpable cervical muscle spasm and tenderness over the upper cervical spine 1
• Neurological impairment occurs in 8% of cases, ranging from radiculopathy to myelopathy in severe presentations 2

Pathophysiological Mechanism

• Inflammation-induced laxity of cervical ligaments (particularly the transverse and alar ligaments) is the key pathologic mechanism, as demonstrated by MRI showing inflammation surrounding cervical spinal ligaments that resolves concurrently with subluxation reduction 4
• Vascular hyperemia in the perivertebral venous plexus connecting the pharynx to the atlantoaxial joint may facilitate spread of inflammation, leading to ligamentous weakening 4, 5

Clinical Presentation Variants

Typical Presentation (Most Common)

• Acute onset of painful torticollis within days to 2 weeks following ENT infection or surgery 1, 2
• Symptomatic patient with obvious discomfort and restricted neck mobility 2

Atypical Presentation (Rare)

• Delayed presentation occurring weeks to months after the inciting event 3
• Asymptomatic subluxation discovered incidentally on imaging, as reported in a 78-year-old patient three months after skull base infection treatment 3

Severity Grading and Complications

• Fielding-Hawkins classification (Types I-IV) is used to grade the degree of rotatory subluxation based on CT imaging 2
• High-grade instability (Types III-IV) carries increased risk of neurological compromise and typically requires surgical intervention 2
• Neurological sequelae develop in 3.6% of cases when diagnosis is delayed, with minor limitation of neck movement being the most frequent long-term complication 2

Critical Diagnostic Pitfalls

• Do not dismiss persistent torticollis in a child following ENT procedures or upper respiratory infections as simple muscle spasm—maintain high suspicion for Grisel's syndrome 1, 2
• Asymptomatic presentations can occur, particularly in delayed cases, emphasizing the need for appropriate follow-up imaging in patients with skull base or deep neck infections even when clinically improved 3
• Early diagnosis is crucial: the 33-day average delay in diagnosis contributes to the 12% rate of surgical intervention and 3.6% rate of permanent sequelae 2