Grisel's syndrome

Overview

Grisel's syndrome is a rare condition characterized by non-traumatic subluxation (partial dislocation) of the atlantoaxial joint, which is the joint between the first two cervical vertebrae (C1 and C2) in the neck. This syndrome most commonly affects children and often occurs following an infection in the head or neck region, or after ENT (ear, nose, and throat) surgical procedures. The condition results in painful torticollis (twisted neck) and restricted neck movement, and requires prompt diagnosis and management to prevent neurological complications.

Causes

Grisel's syndrome is typically caused by inflammation of the tissues surrounding the cervical spine, which leads to ligamentous laxity and instability of the atlantoaxial joint. Common underlying causes include:

• Upper respiratory tract infections: Such as pharyngitis, tonsillitis, or otitis media

• Postoperative inflammation: Following surgeries like adenoidectomy, tonsillectomy, or mastoidectomy

• Lymphadenitis: Inflammation of lymph nodes in the neck

• Other infections: Such as retropharyngeal abscess

The condition is more common in children due to their increased ligamentous laxity and more horizontal orientation of cervical facets.

Symptoms

The primary symptom of Grisel’s syndrome is acute torticollis, accompanied by other signs of neck dysfunction and systemic illness. Common symptoms include:

• Torticollis: Head tilted to one side with limited ability to rotate the neck

• Neck pain and stiffness

• Muscle spasms in the neck

• Fever: Especially if an underlying infection is present

• Difficulty swallowing (dysphagia)

• Neurological symptoms: In severe cases, compression of the spinal cord can lead to weakness or sensory changes

The onset is often sudden and may occur a few days after the initial infection or surgical procedure.

Diagnosis

Diagnosis of Grisel's syndrome is based on clinical findings and imaging studies. Key diagnostic steps include:

• Medical history: Recent upper respiratory infection or ENT surgery

• Physical examination: Identifies torticollis and pain on neck movement

• X-rays of the cervical spine: May show misalignment of the atlantoaxial joint

• CT scan: Provides detailed visualization of the C1–C2 joint and confirms subluxation

• MRI: Useful for assessing soft tissue inflammation and potential spinal cord compression

Prompt imaging is crucial to avoid delayed diagnosis and potential neurological deterioration.

Treatment

Treatment of Grisel’s syndrome depends on the severity of the subluxation and presence of neurological symptoms. Early intervention typically leads to excellent outcomes.

Conservative Treatment:

• Antibiotics: To treat underlying infection if present

• Anti-inflammatory medications: To reduce pain and inflammation

• Neck immobilization: With a cervical collar or soft brace to allow ligament healing

• Bed rest and physical therapy: To gradually restore neck mobility

Surgical Treatment:

• Required in rare cases with persistent subluxation or neurological deficits

• May involve spinal fusion or stabilization of the cervical vertebrae

Prognosis

The prognosis of Grisel’s syndrome is generally favorable with early diagnosis and conservative treatment. Most children recover fully without long-term complications. However, if diagnosis is delayed or if the condition progresses, there is a risk of permanent neurological damage due to spinal cord compression. Regular follow-up and imaging are essential to ensure complete resolution and prevent recurrence. Education of healthcare providers about this rare but serious condition is key to improving outcomes.