Grisel's syndrome involves a subluxation of the atlantoaxial joint and sometimes lead to a partial dislocation of the atlantooccipital joint. It usually affects children between 6 and 12 years of age, but may also occur in adults
2,11,14). Grisel's syndrome was first described in 1930. However, it is a rare disease with an apparently low index of suspicion
13). History taking and clinical signs and symptoms are important clues in the diagnosis of Grisel's syndrome
14).
Patients generally seek treatment for progressive throat and neck pain, and pain on attempted motion. One of the outstanding symptoms of Grisel's syndrome is a spontaneously arising, which is the symptomatic manifestation of unilateral or bilateral atlantoaxial subluxation
14). Neurological complications occur in approximately 15% of these cases and can range from radiculopathy to myelopathy, and may even result in death
4). A subluxation may not yet be evident at the onset of the disease. Early recognition of the infectious disease and a prompt attempt of appropriate conservative treatment are essential in the prevention of progression from such infectious disease to Grisel's syndrome
11). Several theories have been proposed to explain the etiology and pathogenesis of atlantoaxial subluxation in Grisel's syndrome. There is general agreement today that the disorder may occur in association with any condition or surgical procedure that results in hyperemia and pathologic laxity of the transverse ligament and of the atlantoaxial joint
1,14). Therefore, the prospective pathophysiology of subluxation of the atlantoaxial joint may be inflammatory ligamentous laxity caused by an infection. It is largely associated with an overt or occult head and neck infection, especially, an otolaryngologic infection or cervical lymphadenitis
2). Furthermore, Grisel's syndrome has been reported postoperatively after tonsillectomy, adenoidectomy, repair of choanal atresia, surgery for the otitis media and surgery for a neck disease
9). It is well known that patients with clefts are prone to upperairway infections, even more so after surgery
10). It is also postulated that infection may spread directly into the small atlantoaxial joints and thus into the surrounding ligaments. This infection may increase the laxity of the ligaments of the atlantoaxial joint. This in turn may explain the gap between the dens and the front arch of atlas
8). Several authors support these assumptions, but pathogens of Grisel's syndrome are rarely identified. Paping et al. found Fusobacterium necrophorum, an anaerobic bacterium, in the blood and ear cultures of a child with Grisel's syndrome after the child had been inflicted with bilateral fulminant otitis media
12). Clark et al. described a case of infection-related atlantoaxial subluxation without torticollis, from which a retropharyngeal beta-hemolytic streptococcal abscess was found
3). Those pathogens described by another study included: Pseudomonas aeruginosa (1 case), Staphylococcus aureus (1 case) or Methicillin-resistant Staphylococcus epidermidis (1 case) (isolated variably from the neck and blood cultures), and Epstein-Barr virus (of infectious mononucleosis). However, there was no case associated with
Mycobacterium tuberculosis.
Any one treatment method for Grisel's syndrome would not be universally accepted. Several authors would consider a conservative treatment for type I (immobilization, antibiotics, rest and analgesia) and type II (includingcervical traction or muscle relaxants) while they may utilize a more invasive approach for types III and IV (halo immobilization, arthrodesis and C1-C2 cervical fusion). Nevertheless, these various therapeutic approaches may be applicable in each case, and patients should be individually managed. A proper management of Grisel's syndrome should be based on three aspects: (1) a specific treatment of the infectious disease, (2) correction of the bone deformity and (3) prevention of neurological damage. Furthermore, a neurosurgical consultation is essential for all cases, even mild ones
7). This case had no anterior displacement and had less than 3mm of atlantoaxial space, which corresponds to Type 1 subluxation in the Fielding's Classification. Concurring to the recommendations for the management of type 1 subluxation of the Fielding's Classification, she was prescribed with a soft collar to wear for cervical immobilization, antibiotics, and botulinum toxin as a supplementary muscle relaxant. In an attempt to correct severe subluxation with a kyphoscoliotic curvature, anterior cervical discectomy and fusion (ACDF) were performed at the C4-5 level in this patient. Also, she was put on an oral medication for a long time. Her symptoms of torticollis and pain improved. Furthermore, radiographs and MR images showed an improved atlantoaxial alignment and decreased posterior epidural abscess after administration of oral drugs or after surgery.