Grisel syndrome presenting as hemiplegia in a patient with multifocal staphylococcal sepsis

Abstract

Introduction
Grisel syndrome is a non-traumatic rotary subluxation of C1 on C2 (atlantoaxial subluxation). It is a rare condition predominantly described in paediatric population with previous history of upper respiratory infections orotolaryngeal procedures. The diagnosis is established by the association of clinical and radiologic findings4,6.
We report a case of 15-year old boy with Grisel syndrome accompanying multifocal sepsis (pericarditis, septic polyarthritis, pneumonia and pharangytis ), treated surgically – occipital cervical fusion using struts of iliac crest tri-cortical graft wired to the occiput and C3 and C4.
Objective
To describe a case of non-traumatic atlantoaxial rotatory subluxation (Grisel syndrome) and to review clinical and radiologic aspects, pathophysiology, and treatment of this rare condition, which if left untreated can lead to catastrophic outcomes. Authors are trying to demonstrate the significance of early intervention for improved outcomes, and that physicians should examine patients with multifocal sepsis thoroughly so as not to miss foci of infection and have high index of suspicion of the syndrome in patients that present with neck swelling, and develop torticollis and neurological deficit.
Methods
This is a case presentation of a 15-year-old boy who presented with an acute history of neck swelling, shortness of breath, orthopnoea, pericardial effusion, right hip and knee swelling. He later developed torticollis and left sided hemiplegia. On admission, he was septicemic. There was no history of head or neck trauma. Cervical spine radiograph showed increased atlantodental index (ADI) and prevertebral soft tissue swelling. Consent was sought from guardian and patient to use images and case notes for submission for publication.
Results
The patient was treated with analgesia, drainage of pericardial effusion and intravenous antibiotics. The right knee sepsis was noted on admission and drained on the day pericardial drain was inserted, while the right hip was missed – only to be noted 59 days later with limb length discrepancy. The C1/C2 subluxation was noted 17 days after admission. Reduction was done soon after diagnosis and maintained with a halo-thoracic immobilizer. A week later, he underwent posterior occipital cervical fusion. The hemiplegia resolved fully.
Conclusions
Multifocal staphylococcal septicemia can present with unusual foci of infection, which may be missed especially if the patient is very sick. Grisel syndrome with an associated left hemiplegia and septic arthritis of the right hip were diagnosed late in this case. Grisel syndrome patients might end up with catastrophic outcomes if left untreated or mis-diagnosed, but if prompt treatment is initiated, full functional recovery is achievable.