Grisel Syndrome in Adult Patients.

BRIEF COMMUNICATIONS

Report of Two Cases and Review of the Literature

Karam Youssef, Shedid Daniel
Can. J. Neurol. Sci. 2009; 36: 109-113

Grisel’s syndrome involves the subluxation of the atlanto-

axial joint from inflammatory ligamentous laxity following an
infectious process in the head or neck1. It rarely occurs in adults.
In children, the treatment is first medical with the need for
immobilization and sometimes internal fixation depending on
the stability of C1-C2. In this paper we report two cases of Grisel
syndrome in adults.

CASE REPORT
Case 1
A 50-year-old female intravenous drug user presented with
neck pain and electrical sensations in the upper and lower limbs
on neck flexion (Lhermitte’s sign). There was no history of head
and neck trauma but she reported tonsilopharyngitis one to two
weeks prior to consultation. Her physical examination was
normal except for a positive Lhermitte’s sign. Magnetic
resonance imaging (MRI) of the cervical spine revealed a mass
infiltrating the posterior subligamentous region of the anterior
arch of C1, the odontoid and the body of C2 and compressing the
bulbo-medullary junction (Figure 1). and an increase of the
atlanto-dental interval (Figure 2). The CT-scan did not show any
bony destruction or a rotatory subluxation (Figure 3). The patient
received antibiotics with wide spectrum coverage and was
stabilized in a Halo vest after closed reduction. The radiological
follow-up revealed a decrease in the prevertebral collection as
well as a decrease in the spinal cord compression (Figure 4
and 5).
After three months the patient fell and presented to the
emergency room with loosening of the Halo. She was
neurologically intact but the flexion cervical x-ray revealed a C1- Figure 1: Sagital T1 contrast enhanced MRI Grisel’s syndrome.
C2 instability (Figure 6). The patient underwent a C1/C2 fusion,
with C1 lateral mass screw and a pars screw (Figure 7). At her
last follow-up (five months), the patient was doing very well and
was pain-free.
completed with plain films (Figure 10), CT-scan and MRI of the
cervical spine. There was no bone decalcification nor rotatory
Case 2
subluxation. The patient was treated with antibiotics and
A 53-year-old alcoholic male was admitted to the service of stabilized clinically before operation. He then underwent a
gastroenterology for alteration of vigilance and gastro-intestinal C1/C2 fusion, with C1 lateral mass screw and a pars screw
bleeding. No neurological deficit was found. Blood workup
showed elevated hepatic enzymes, electrolyte disturbances,
anemia and leukocytosis. He also had positive hemoculture for
gram negative rods but no septic focus was found. A meningitis
was ruled out. Before doing a lumbar puncture, a brain MRI was From the Division of Neurosurgery, Spine Surgery Unit, Centre Hospitalier de
done to rule out an abcess. This showed a peri-odontoidal l’Université de Montréal. Notre-Dame Hospital. Montréal, Québec, Canada.

infiltration with an increased atlanto-dental interval and

RECEIVED MARCH 7, 2008. FINAL REVISIONS SUBMITTED SEPTEMBER 5, 2008.
Correspondence to: Youssef Karam, Montréal University, 1690 Sherbrooke St. East,
retropharyngeal abcess (Figures 8 and 9). The investigation was Montréal, Québec, H2L 1M5, Canada.

THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES 109

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Figure 2: Sagittal T2 showing the increased ADI. Figure 4: Sagital T2WI before treatment. We see the peri-odontoidal
infiltration with compression of the bulbo-medullary junction.

Figure 3: CT-scan. There is no bone decalcification nor rotatory Figure 5: Sagital T2WI after two months of treatment. Resolution of the
subluxation. peri-odontoidal infiltration with decrease of the compression.

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Figure 6: Increased Atlanto dental interval (ADI) in the flexion x-ray. Figure 8: Sagital brain MRI showing the peri-odontoidal infiltration
C1-C2 instability. and the increased ADI.

Figure 7: Surgical fixation.We see the reduction of the ADI interval. Figure 9: Sagital T1WI with Gado that shows the retropharyngeal
abcess with the increased ADI.

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Figure 10: Lateral plain X-ray showing the increased ADI. Figure 11: Surgical fixation and reduction of the ADI.

(Figure 11) and a transoral evacuation of the retropharyngeal Anatomical findings that support this theory: the peri-odontoidal
abcess. After surgery the patient completed antibiotic treatment vascular plexus drains in the postero-superior pharyngeal
and he was doing well at two months follow-up. region1,3,4.
This condition is more common in children because the upper
DISCUSSION spine is more mobile than in adults and they have a hypertrophic
status of the peripharyngeal lymphoid tissue which is located in
Grisel syndrome was first described in 1830 by Sir Charles the area drained by the pharyngovertebral plexus2.
Bell2 and until now there is controversy regarding the Clinical manifestations include: neck stiffness, torticollis,
pathogenesis, the diagnosis, and treatment of this syndrome. neck pain on attempted motion and dysphagia. Neurological
Grisel reported three cases in 1930 and defined this syndrome as complications occur in 15% of cases and include radiculopathy,
an atlanto-axial instability from inflammatory ligamentous laxity quadriplegia and death from cord compression1. Clinical signs
in the head and neck1. It is a rare disease and usually affects include: 1) Sudeck’s sign where spinous process of C2 is
children, but infrequent adult cases do occur. Several entities palpable away from the midline at the site opposite to the
were reported in the literature to be associated with this dislocation and 2) Cock-Robin sign which is torticollis with the
syndrome: pharyngitis, nasopharyngitis, adenotonsillitis, chin turned away from the subluxated side. The head is tilted
tonsillar abcsess, parotitis, cervical abscess, otitis media1, towards the affected side and flexed anteriorly making it difficult
nasopharyngeal operations (velopharyngoplasty)2, and after to open the mouth1.
adentonsillectomy with use of monopolar suction cautery3. Imaging is very important in the diagnosis and follow-up of
Several theories have been proposed to explain its pathogenesis: these patients4: An open-mouth view can provide good
1) by Witteck; metastatic inflammatory effusion cause visualization regarding rotational deformity. Antero-posterior
ligamentous stretching and subluxation, 2) by Grisel; view can reveal asymmetry between the facet joints. Lateral
subluxation occurs on the basis of muscular spasm, and 3) the view reveals an increased atlanto-dental interval and dynamic
most accepted mechanism by most authors today; hyperemia views show the instability. A CT-scan with 3D reconstruction
following infection or trauma that leads to decalcification of the offers detailed illustration of the bony cervical spine and
anterior arch of C1 and laxity of the transverse ligament.

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subluxation. Magnetic resonance imaging is helpful for REFERENCES

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very helpful and should be done early if there is a suspicion of ActaNeurochir (ien). 2003 Jan;145(1):69-72.
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H. Grisel syndrome after velopharyngoplasty. Scand J Plast
Fielding has described a working classification of atlantoaxial Reconstr Surg Hand Surg. 2004;38(1):53-7.
subluxation into four distinct types6. In type I, the atlas is rotated 3. Martinez-Lage JF, Martinez Perez M, Fernandez Cornejo V, Poza
on the odontoid and there is no anterior displacement of the atlas. M. Atlanto-axial rotatory subluxation in children: early
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3 to 5 mm of anterior atlas displacement. In type III, there is 4. Yu KK, White DR, Weissler MC, Pillsburry HC. Nontraumatic
rotation of the atlas with anterior displacement greater than 5 atlantoaxial subluxation (Grisel syndrome): a rare complication.
mm. In type IV, there is rotation and posterior displacement of Laryngoscope. 2003 Jun;113(6):1047-9.
the atlas. Types III and IV are the least common overall but the 5. Meek MF, Hermens RAEC, Robinson PH. La maladie de Grisel:
most likely to develop spinal cord compression. This spontaneous atlantoaxial subluxation. Cleft Palate Craniofac J.
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a respiratory tract or a pharyngeal infection5. The treatment is
first of all medical (antibiotics if an infection is established).
Other treatment modalities (immobilization) depend on the
stability of C1/C2. If the transverse ligament is intact, a cervical
immobilization with a hard cervical collar for six to eight weeks
is recommended. If the transverse ligament is deficient, the neck
should be stabilized by cervical skeletal traction with Gardner-
Wells or immobilized with Minerva cast or halo vest. If the
patient has subluxation lasting longer than three months, primary
C1/C2 fusion is recommended1.
Our two adult patients presented with Grisel’s syndrome. The
first after a tonsilopharyngitis and the second after a
retropharyngeal abcess. The first presented with Lhermitte’s sign
and a moderate nuchal rigidity and the second with nuchal
rigidity in the context of sepsis and retropharyngeal abcess. In
the two cases we had an instability of C1-C2 with deficiency of
the transverse ligament, revealed by the increase of the atlanto-
dental interval. The medical treatment alone failed in the first
case. The internal fixation reduces the atlanto-dental interval in
the two cases and the patients are stabilized.
According to our experience and in the light of the literature :
1) Grisel’s syndrome is a rare disease that we should rule out in
a patient presenting with neck pain with or without neurological
deficit after upper respiratory tract infections.
2) Recognition of Grisel’s syndrome and early intervention is a
critical factor for a successful outcome.
3) Surgical treatment depends on the grade of the disease, the
stability of C1-C2, and the response to medical therapy
(antibiotics).

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