Presentation
The most common presentation of RA in the cervical spine is atlantoaxial subluxation (AAS). [austinpublishinggroup.com]
All patients presented in casualty with suspected cervical spine injury and concussive head injury were subjected to X-rays of the cervical spine. [ijoonline.com]
This article presents such a case treated non-surgically, with discussion of clinical presentation, diagnosis, pathogenesis, and treatment. The differential diagnosis of non-traumatic atlantoaxial subluxation is presented for review. [ncbi.nlm.nih.gov]
Entire Body System
- Pain
After two years of follow–up, the patient was without any cervical pain, had no instability in flexion–extension radiographs and also had a C1–2 fusion seen on CT scan. [austinpublishinggroup.com]
A 36-year-old man with an 8-year history of AS presented with progressive neck pain, low back pain, hand numbness, and limited mobility of the neck. [ncbi.nlm.nih.gov]
- Weakness
The patient was admitted to our hospital with gait disturbance, progressive motor weakness in both upper extremities and sensory disturbance in all four extremities. [ncbi.nlm.nih.gov]
Sometimes, Marfan syndrome causes the bones and ligaments to become weak and soft, increasing the risk for bone dislocations. [study.com]
Disturbances Gait disturbances are caused by muscle weakness resulting in abnormal walking. [epainassist.com]
- Falling
The bones are susceptible to fracture from high-energy impact such as falls or car accidents, especially in the elderly. [neurosurgery.wustl.edu]
Case 64M, s/p fall down stairs while intoxicated 3 days ago. No LOC. P/w persistent neck pain & right shoulder pain. PMH: EtOH abuse Neuro: AA&Ox3, FC, MAE 5/5, SILT 3. CT C Spine 4. [slideshare.net]
Fall or whiplash injury may cause injury or fracture of Odontoid Process, body of atlas or axis vertebra. [epainassist.com]
- Surgical Procedure
In the past 30 years, developments in surgical procedures, instruments, and understanding of the related anatomical structures and biomechanical relationships have improved the therapeutic effects of surgery. [healio.com]
The most common surgical procedure for anterior C1-C2 subluxation is posterior fusion by internal fixation of C1-C2. References Barr W. Neurologic complications of rheumatoid arthritis: identifying clinical features of nerve involvement. [dizziness-and-balance.com]
It primarily affects the pediatric population with 90% of patients being under 21 years of age. [3] It is seen most frequently following upper respiratory tract infections and surgical procedures of the head and neck. [1] Its management involves reducing [asianjns.org]
These methods have the advantage in that the midline surgical procedures as necessary can be carried out without affecting the fixation. [jpgmonline.com]
- Swelling
Fifteen milligrams of dexamethasone was given once a day for 3 days to reduce the swelling and pain. We performed a cutaneous traction for the subluxation and carefully watched the hyoid bone and the patient's respiration. [ncbi.nlm.nih.gov]
[…] occurs from cord compression between the odontoid and posterior arch of C1 Imaging findings Widening of the predentate space Disruption in the smooth curve of the imaginary line connecting the spinolaminar white lines of the vertebral bodies Soft tissue swelling [learningradiology.com]
In addition, a pannus (localized mass/swelling of rheumatoid synovial tissue) can also form at this location, causing even more spinal cord compression. [uscspine.com]
Dystonia, torticollis and gait abnormality may correct following complete recovery and reduction of swelling of spinal cord. Chronic pain. Injury Associated With Spinal Cord Damage Prognosis often is not good. [epainassist.com]
Gastrointestinal
- Overeating
Type A (n=33) There is a forward translation of C1 over C2 causing an increase in the ADI with a reduction of the PADI. [ijoonline.com]
The expected distance between anterior arch of C1 and the dens in the fully flexed position should be In a vertical subluxation, the dens is often above the McGregor line by over 8 mm in men and 9.7 mm in women. [radiopaedia.org]
Visit for more related articles at Journal of Spine Abstract It is known that cervical spine involvement occurs in over half of patients with rheumatoid arthritis (RA). This involvement is over 70% in Tunisia. [omicsonline.org]
Note anterior displacement of the atlas over the axis. Also note amputation of the axis, with a separate ossicle located superoanteriorly and representing the dislocated os odontoideum. [arthritisresearch.us]
- Nausea
[…] dysarthria dysphagia facial pain/pareasthesia feeling of the head 'falling off' when going over a bump in a car needing to hold head when getting up out of bed neck pain headache hypoaesthesia of both hands and/or feet limb muscle wasting and weakness nausea [back-in-business-physiotherapy.com]
Musculoskeletal
- Arthritis
The subsequent bony erosion and increased synovial effusion represent arthritis that is more advanced. [ajnr.org]
Etiology congenital os odontoideum Down syndrome (20%) Morquio syndrome spondyloepiphyseal dysplasia osteogenesis imperfecta Marfan disease neurofibromatosis type 1 (NF1) arthritides rheumatoid arthritis psoriatic arthritis Reiter syndrome (reactive arthritis [radiopaedia.org]
( 28255666 ) Han M.H....Chun H.J. 2017 11 Risk Factors for the Development and Progression of Atlantoaxial Subluxation in Surgically Treated Rheumatoid Arthritis Patients, Considering the Time Interval between Rheumatoid Arthritis Diagnosis and Surgery [malacards.org]
Keywords: Rheumatoid arthritis; Atlantoaxial subluxation; Cranial settling; Basilar impression; Vertical subluxation. [austinpublishinggroup.com]
- Neck Pain
A 36-year-old man with an 8-year history of AS presented with progressive neck pain, low back pain, hand numbness, and limited mobility of the neck. [ncbi.nlm.nih.gov]
During the physical examination, her neck was in the neutral position, and she had neck pain on motion and restricted neck mobility with no neurological deficits. [jocr.co.in]
- Fracture
Fracture of the odontoid process: Treatment with anterior screw fixation. Spine (Phila Pa 1976) 1989;14:1065-70. 17. Julien TD, Frankel B, Traynelis VC, Ryken TC. Evidence based analysis of odontoid fracture management. [ijoonline.com]
Work-up revealed an anterior C1 arch and Type II C2 dens fracture with atlantoaxial subluxation (in addition to multiple cervical spinous process fractures). Examination Awake and alert but confused. No focal motor or sensory deficits. [spineuniverse.com]
They have few, if any, complications, such as dysphagia, hypoxia, cardiorespiratory collapse, laceration of the pharynx, mandibular fracture, thyroid cartilage fracture, and facial fracture. [ncbi.nlm.nih.gov]
- Joint Subluxation
(Fixed rotatory subluxation of the atlanto-axial joint). J Bone Joint Surg Am 1977;59:37-44. 2. Subach BR, McLaughlin MR, Albright AL, Pollack IF. Current management of pediatric atlantoaxial rotatory subluxation. [jocr.co.in]
(Fixed rotatory subluxation of the atlanto-axial joint). J Bone Joint Surg Am. 1977;59(1):37-44. Herzka A, Sponseller PD, Pyeritz RE. Atlantoaxial rotatory subluxation in patients with Marfan syndrome. A report of three cases. [practicalneurology.com]
Psychiatrical
- Suggestibility
Computed tomography did not reveal abnormality of the odontoid process or the presence of a high-density area that could suggest calcification in or near the cystic mass. [ncbi.nlm.nih.gov]
It is thought that this technique reduces atlantoaxial subluxation caused by forward flexion of an unstable cervical spine. [4] Aspinall [5] suggests that if upper cervical instability is suspected, the Sharp-Purser test should be performed first before [physio-pedia.com]
Neurologic
- Quadriplegia
To demonstrate two rare cases of atlantoaxial subluxation associated with ossification of the posterior longitudinal ligament of the cervical spine, in which spastic quadriplegia developed. [ncbi.nlm.nih.gov]
[…] is the most significant complication of rheumatoid arthritis (RA) in the cervical spine [1], occuring in 29-70% of RA patients. [2] Cervical spinal cord compromise due to atlantoaxial subluxation can have serious neurological consequences, including quadriplegia [physio-pedia.com]
Patient may suffer paralysis of all 4 extremities ( quadriplegia ). [epainassist.com]
- Ataxia
A 52-year-old male with a 6-year history of RA had a sudden onset of visual field defect and mild right cerebellar ataxia. [ncbi.nlm.nih.gov]
According to Sawin and Menezes series in persons with osteogenesis imperfecta (1997), symptoms and signs included headache (76%), lower cranial nerve dysfunction (68%), hyperreflexia (56%), quadriparesis (48%), ataxia (32%), nystagmus (28%), and scoliosis [dizziness-and-balance.com]
Syringomyelia and neurological deficits, including hyperreflexia, clonus, abnormal gait, ataxia, and tetraparesis have been described as potential sequelae of atlanto-axial dislocation in Down syndrome (Semine et al. 1978). [arthritisresearch.us]
These may include mechanism of injury - whiplash, other unguarded impacts, and severe loading can all be yellow flags altered sensation and movement of the tongue eg frequent biting of the tongue with potentially little pain ataxia tinnitus diplopia dizziness [back-in-business-physiotherapy.com]
- Monoplegia
We describe a patient who had, along with this rotatory subluxation, a distant cord contusion (with normal imaging of the subaxial vertebral column) resulting in left upper limb monoplegia. [neurologyindia.com]
- Paresis
This lesion, untreated, may result in damage to the spinal cord, paresis or sudden death. Occipital headache, present in 13 of 22 patients, was often aggravated by working with the head in forward flexion. Paresthesias were present in six patients. [ncbi.nlm.nih.gov]
Workup
Congenital scoliosis Osteogenesis imperfecta Neurofibromatosis Larsen syndrome Spondyloepiphyseal dysplasia congenita Chondrodysplasia punctata Metatropic dysplasia (a rare syndrome that can present with AAI) Kniest syndrome (also can present with AAI; a workup [openanesthesia.org]
However, given the high frequency of hypothyroidism in individuals with Down syndrome, a full thyroid workup may be indicated. [emedicine.medscape.com]
Treatment
Nonsurgical treatment most often resolves the symptoms; however, in some cases surgical treatment is necessary to repair the subluxation. [ncbi.nlm.nih.gov]
Suggest Treatment Indicate how you would treat this patient by completing the following brief survey. Your response will be added to our survey results below. Selected Treatment We elected a posterior occiput-cervical fixation (O-C3). [spineuniverse.com]
Early treatment implies a non-surgical approach and a good outcome. Conservative treatment is the recommended first step for this condition. [jmedicalcasereports.com]
Prognosis
Good prognosis has been reported; however, a return to high-level sports activities is thought to be a contraindication. [ncbi.nlm.nih.gov]
The delay between injury and management affects the prognosis. Early treatment implies a non-surgical approach and a better outcome. Conservative treatment is the first step with this condition. [jmedicalcasereports.com]
[…] lateral tilt; - it can occur spontaneously or after trauma; - complete C1-C2 dislocation is a known complication of football spearing; - pts complain of neck pain, occipital neuralgia, and occassionally symptoms of vertebrobasilar artery insufficiency; - prognosis [wheelessonline.com]
Etiology
Etiology congenital os odontoideum Down syndrome (20%) Morquio syndrome spondyloepiphyseal dysplasia osteogenesis imperfecta Marfan disease neurofibromatosis type 1 (NF1) arthritides rheumatoid arthritis psoriatic arthritis Reiter syndrome (reactive arthritis [radiopaedia.org]
A review of the available literature concerning etiology and treatment of la maladie de Grisel is presented. [ncbi.nlm.nih.gov]
The etiology of this process is speculative. [healio.com]
Epidemiology
The epidemiology of rheumatoid arthritis. Rheum Dis Clin North Am 2001;27(2):269-81. Majithia V, Geraci SA. Rheumatoid Arthritis: Diagnosis and Management. Am J Med 2007;120(11):936-9. Caglayan O, Sukru Aydog Y. [acta.tums.ac.ir]
Epidemiology: Anatomy 50% rotation of the cervical spine occurs at C1-C2. [posna.org]
Pediatrics. 1995 ; 96 : 151 –154 Atlantoaxial Instability in Individuals With Down Syndrome: Epidemiologic, Radiographic and Clinical Studies. Pueschel SM, Scola FH. [pedsinreview.aappublications.org]
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. [emedicine.medscape.com]
Pathophysiology
Pathophysiology of atlantoaxial subluxation revolves around the periodontoid vascular plexus that drains the posterosuperior pharyngeal region. Infectious and inflammatory emboli cause synovial engorgement, weakening paraspinal ligaments. [ncbi.nlm.nih.gov]
CrossRef Google Scholar × Volume 126, Issue 5 May 2012, pp. 529-531 Objectives: To discuss the pathophysiology of atlanto-axial subluxation as a rare complication of tonsillectomy, and to discuss the important radiological findings for diagnosis and treatment [cambridge.org]
Introduction C1-C2 rotatory instability (fixed rotation of C1 on C2) caused by subluxation or facet dislocation common cause of childhood torticollis spectrum of disease that ranges from mild subluxation to fixed facet dislocation Pathophysiology common [orthobullets.com]
AARF is more commonly caused by infection or trauma, 2 in association with a predisposing medical condition such as inflammatory or ligament laxity, 2-4 or head and neck surgery. 5 The pathophysiology is not well defined, but there appears to be a predisposition [practicalneurology.com]
Prevention
Of these 66 cases, 3 cases in which destruction of C1 lateral mass, C1-C2 joints and posterior elements prevented C1-C2 fixation were subjected to occipito cervical fixation and were not included in the study. [ijoonline.com]
An awareness of this complication among paediatricians, otolaryngologists and emergency physicians, and a high index of suspicion in any patient presenting with torticollis following ENT surgery is essential in preventing significant neurological morbidity [ncbi.nlm.nih.gov]