Presentation
Nuchal spasm was present on the left side. No neurological impairment was observed. [ncbi.nlm.nih.gov]
We therefore present the case of a patient with Grisel's syndrome as a sequela of an acute mastoiditis complicated by a Bezold's abscess. [elsevier.es]
As the symptoms did not improve, she was presented in our clinic. Our radiographs revealed a subluxated atlantoaxial joint. Results. [insights.ovid.com]
Grisel syndrome has a variable presentation, time between inciting event and symptom onset is variable, and laboratory investigations may be normal. So diagnosis is difficult. [radiopaedia.org]
Because lymph nodes are present in this plexus, septic exudates may be freely transferred from the pharynx to the C1-C2 articulation. [consultant360.com]
Entire Body System
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Fever
A previously healthy 2-year-old boy was hospitalized after 2 weeks of persistent fever (temperature to a maximum of 38.9C [102F]) and a 2-day history of neck stiffness. There was no history of cough, rhinorrhea, or dysphagia. [pediatricsconsultantlive.com]
A previously healthy 2-year-old boy was hospitalized after 2 weeks of persistent fever (temperature to a maximum of 38.9 C [102 F]) and a 2-day history of neck stiffness. There was no history of cough, rhinorrhea, or dysphagia. [consultant360.com]
Boy • Torticollis, halitosis, cough, odynophagia for 1/52 • No fever • PE: – posture in right-sided head rotation – Subtle flexion – Ipsilateral SCM spasm – Nuchal pain – Hypertrophied tonsils, no abcess – Normal Neuro exam • Lab: N crp and WBC 5. • CT [de.slideshare.net]
Her recent clinical history revealed cough and fever from the day before the neck stiffness' onset, which spontaneously regressed in 2-3 days; no trauma was reported. [pediatricneurosciences.com]
Case Report The patient was a 3-year-old boy with no relevant history except for a dubious allergy to amoxicillin, who presented right otalgia with otorrhea and fever (38.6 C) of 3 days duration despite treatment with oral clarithromycin and topical ciprofloxacin [elsevier.es]
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Sepsis
We opted for admission to the paediatric ICU due to suspicion of sepsis. [elsevier.es]
Respiratoric
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Cough
There was no history of cough, rhinorrhea, or dysphagia. The oropharynx could not be examined because of neck stiffness. The patient had bilateral anterior cervical lymphadenopathy. [pediatricsconsultantlive.com]
Boy • Torticollis, halitosis, cough, odynophagia for 1/52 • No fever • PE: – posture in right-sided head rotation – Subtle flexion – Ipsilateral SCM spasm – Nuchal pain – Hypertrophied tonsils, no abcess – Normal Neuro exam • Lab: N crp and WBC 5. • CT [de.slideshare.net]
Her recent clinical history revealed cough and fever from the day before the neck stiffness' onset, which spontaneously regressed in 2-3 days; no trauma was reported. [pediatricneurosciences.com]
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Tonsillar Hypertrophy
His oropharynx was not erythematous; the uvula was in the midline and there was no tonsillar hypertrophy. The patient was reluctant to move his neck in any direction, but especially side-to-side. There were no other notable physical findings. [pediatricsconsultantlive.com]
On admission she presented, at physical examination, with right torticollis, sore throat, tonsillar hypertrophy and hyperemia; neurological examination reported no deficits. [pediatricneurosciences.com]
Gastrointestinal
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Odynophagia
Boy • Torticollis, halitosis, cough, odynophagia for 1/52 • No fever • PE: – posture in right-sided head rotation – Subtle flexion – Ipsilateral SCM spasm – Nuchal pain – Hypertrophied tonsils, no abcess – Normal Neuro exam • Lab: N crp and WBC 5. • CT [de.slideshare.net]
Jaw & Teeth
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Halitosis
Boy • Torticollis, halitosis, cough, odynophagia for 1/52 • No fever • PE: – posture in right-sided head rotation – Subtle flexion – Ipsilateral SCM spasm – Nuchal pain – Hypertrophied tonsils, no abcess – Normal Neuro exam • Lab: N crp and WBC 5. • CT [de.slideshare.net]
Musculoskeletal
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Neck Pain
Progressive throat and neck pain and neck stiffness can be followed by neurologic symptoms such as pain or numbness radiating to arms (radiculopathies). [youtube.com]
On the day before readmission, the patient continued to show signs of fatigue and neck pain. His parents noted that while riding in their car, the patient held his neck with both hands-especially when the ride became bumpy. [pediatricsconsultantlive.com]
• KarKos et al : – Systemic review 71 articles – 96 Pt with C1 C2 subluxation – All no hx. of trauma – 48% had recent URTI – 40% post neck procedures – Clinical picture: torticollis,neck pain maily in nape – Signs: – Palpable deviation spinous process [de.slideshare.net]
On the day before readmission, the patient continued to show signs of fatigue and neck pain. His parents noted that while riding in their car, the patient held his neck with both hands—especially when the ride became bumpy. [consultant360.com]
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Fracture
Atlantoaxial instability in acute odontoid fractures is associated with nonunion and mortality. Spine J. 2015 May 1. 15 (5):910-7. [Medline]. Zikou AK, Alamanos Y, Argyropoulou MI, Tsifetaki N, Tsampoulas C, Voulgari PV, et al. [medscape.com]
Neurologic
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Irritability
He was irritable, however, and lay in bed with his head tilted to the left. His oropharynx was not erythematous; the uvula was in the midline and there was no tonsillar hypertrophy. [pediatricsconsultantlive.com]
Pathphysiology • Unknown • Grisel emphasize the role of muscle spasm • Tedesco et al – Cervical lympadenitis induce irritative spasm of suboccipital and parvertebral muscles. • Most accepted theory is by Parke et al. – Studied vascularization of c spine [de.slideshare.net]
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Quadriplegia
In extreme cases, the condition can lead to quadriplegia and even death from acute respiratory failure. [youtube.com]
These last 2 types are more severe and have been associated with spinal cord compression, quadriplegia and even death. They require cervical traction, immobilisation and bed rest. [elsevier.es]
Workup
CT of the neck is the gold standard for diagnosis and further workup. Several theories have been proposed to explain the pathogenesis of this inflammatory subluxation, but its etiology and exact pathogenesis continues to be debated. [ncbi.nlm.nih.gov]
Microbiology
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Staphylococcus Aureus
The most common etiologies 2 are as follows: upper respiratory tract infections tonsillectomy/adenotonsillectomy otitis media other ENT infections/surgery The causative organisms are usually 3 : Staphylococcus aureus Group B. [radiopaedia.org]
Treatment
Treatment and prognosis In milder cases and with early diagnosis, treatment with broad spectrum antibiotics and soft collar is sufficient. Delayed diagnosis / increasing severity needs treatment with traction brace. [radiopaedia.org]
The importance and effectiveness of conservative treatment in Grisel's syndrome. [pediatricneurosciences.com]
Antibiotic treatment appears to be sufficient in these cases, although intensive monitoring is required to identify a possible progression of symptoms. [elsevier.es]
Treatment includes anti-inflammatory medications and immobilization of the neck in addition to treatment of the offending infectious cause (if any) with appropriate antibiotics. Early treatment is crucial to prevent long-term sequelae. [youtube.com]
At least in this case, our treatment led to shorter recovery and avoidance of halo fixation. Our new therapeutic approach to patients with Grisel's syndrome might lead to a shorter recovery. [insights.ovid.com]
Prognosis
Treatment and prognosis In milder cases and with early diagnosis, treatment with broad spectrum antibiotics and soft collar is sufficient. Delayed diagnosis / increasing severity needs treatment with traction brace. [radiopaedia.org]
Fielding et al. 10 proposed a grading scale to guide its prognosis and therapeutic management. In this scale, type I (the most frequent) is a rotating fixation (not a true subluxation), maintaining stability of the transverse ligament. [elsevier.es]
Etiology
Etiology The syndrome may result from any inflammatory process of the head and neck. [radiopaedia.org]
These imaging studies support the two-hit hypothesis of the etiology behind Grisel syndrome. [ncbi.nlm.nih.gov]
Primarily seen in children aged 5-12 years (though possible in adults), its etiology and pathoanatomy are not totally understood. [medscape.com]
Epidemiology
Atlantoaxial instability in individuals with Down syndrome: epidemiologic, radiographic, and clinical studies. Pediatrics. 1987 Oct. 80(4):555-60. [Medline]. Evaniew N, Yarascavitch B, Madden K, Ghert M, Drew B, Bhandari M, et al. [medscape.com]
Pathophysiology
Pathophysiology of this disease consists of relaxation of the transverse ligament of the atlanto-axial joint. Diagnosis can be established using plain film x-rays as well as CT scan of the neck/cervical spine. [youtube.com]
Although first described in 1830, the exact pathophysiology of Grisel syndrome remains unclear. [ncbi.nlm.nih.gov]
Prevention
Early treatment is crucial to prevent long-term sequelae. Surgical fusion may be required for residual instability of the joint. [youtube.com]
The resulting synovial and vascular engorgements may damage the transverse and facet capsular ligaments, leading to laxity and eventual subluxation. 10,11 Immobilization of the neck relieves the subluxation and prevents potential spinal cord compression [consultant360.com]