Description

Spontaneous, nontraumatic rotatory subluxation of the atlantoaxial joint following peripharyngeal inflammation or otorhino-laryngologic surgical procedures (e.g. tonsillectomy, adenoidectomy). Occurs most commonly in the pediatric population.

Pathogenesis of Grisel syndrome is still not quite clear, but it is probably a ‘double hit’ phenomenon. Firstly those with a susceptibility for having a lax C1/C2 joint i.e. children or those with ligament disorder or Down syndrome. The ‘second hit’ results from spasms caused by inflammatory mediators carried to cervical muscles by the pharyngeovertebral venous plexus.

Eponymously named after Pierre Grisel (1869–1959), a French paediatric surgeon who described the syndrome characterized by dislocation of the C1–C2 joint in patients with pharyngitis and torticollis in 1930


History of Grisel Syndrome

Spontaneous, nontraumatic rotatory subluxation of the atlantoaxial joint following peripharyngeal inflammation or otorhino-laryngologic surgical procedures such as tonsillectomy, adenoidectomy and mastoidectomy.

In 1830, Sir Charles Bell reported a case of death from atlanto-axial subluxation which resulted in spinal cord compression in a patient with probable syphilitic ulceration of the pharynx.

A patient, who had a deep ulcer in the back part of the throat, was seized with symptoms like those of apoplexy. These symptoms continued for two hours. At this time the patient’s head fell suddenly forward, and he instantly expired. On dissection, it was found that the ulcer had destroyed the transverse ligament, which holds the process of the dentata in its place. In consequence of the failure of this support, the process was thrown back, so as to compress the spinal marrow. The parts are preserved in my collection.

Bell, Case LXIV 1830

1930 Pierre Grisel

Fig 3. Observation II. Radiographie de profil des articulations sous-occipitales

Fig 3 bis. Observation II. Schéma de la Fig 3. On voit en (i) l’angle de la surface articulaire droite de l’axis, complètement abandonnée par la masse laterale correspondante reportée en arrière, les tubercules antérieurs et postérieurs de l’atlas, (a) et (a’), et sa masse latérale gauche (m) qui a été chassée par la compression et la traction en bas et an avant au devant du corps de l’axis. La partie de l’atlas non cachée par la base du crane est seule teintée en gris

Grisel P, Figure 3 and 3a. 1930: 52

Fig 3. Observation II. Profile x-ray of the suboccipital joints

Fig 3a. Observation II. Diagram of Fig 3. We see in (i) the angle of the right articular surface of the axis, completely abandoned by the corresponding lateral mass carried back, the anterior and posterior tubercles of the atlas, (a) and (a’), and its left lateral mass (m) which has been driven out by compression and traction down and forward in front of the body of the axis. The part of the atlas not hidden by the base of the skull is only tinted in grey

Grisel P, Figure 3 and 3a. 1930: 52


Associated Persons

Alternative names
  • Altlantoaxial rotation
  • Grisel’s syndrome
  • La maladie de Grisel
  • Nontraumatic rotatory subluxation of the atlantoaxial joint; Nontraumatic atlantoaxial subluxation

Controversies

References

Historical references

Eponymous term review


eponymictionary CTA

eponymictionary

the names behind the name

Physician in training. German translator and lover of medical history.

Emergency physician MA (Oxon) MBChB (Edin) FACEM FFSEM with a passion for rugby; medical history; medical education; and asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | Twitter |

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