Donald Corenman, MD, DC. Signs of ligamentous damage. First of all, studies have shown that FLAIR hyperintensities (suggestive of ligamentous partial rupture or damage) have been found in a lot of asymptomatic patients (Myran et al. These cookies will be stored in your browser only with your consent. The atlanto-axial (AA) joint is the joint between the first (atlas) and second (axis) vertebrae (bones) in the neck. 2008). Thanks for your help! The patient may seek out their GP or a local neurosurgeon who will, usually, and usually rightfully so, dismiss these claims, as the patients imaging is normal and also lack neurological signs that would fit with neurovascular compromise. The problem, in the patients eyes, may be a lacking reasonable counter-argument and counter-diagnosis that would explain his or her symptoms, which then prompts the patient to seek out alternative health care. Dr. Gilete in Spain, although I often disagree with his diagnoses, tends to order beautiful dynamic CT scans and also good craniovascular scans. Neurol India. The aim of surgery is to stabilize the AA joint internally to prevent future spinal cord injury. This website uses cookies to improve your experience while you navigate through the website. DRAMMEN, NORWAY, Home The atlantoaxial complex refers to the first two bones of the neck (C1,the atlas, and C2,the axis) as well as the associated collection of ligaments that connect the bones together and the blood vessels that travel through them to the brain. Treatment is via one of two methods: If you or your veterinarian is concerned that your pet may have AA instability, please schedule a consultation with our Neurologist by calling us at our Manchester or Newington location today. A 32 year-old female patient contacted me in 2019 as she had been diagnosed (by a radiologist alone) with craniocervical and atlantoaxial instability. In such a case, however, certain important measurements (not mere CXA (norm: 150-180 degrees) or Grabb-Oakes (norm. Exam for bow hunters syndrome is done dynamically, but thats aother exam. Due to the poor practice integrity that is often associated with DMX imaging, despite these modalities indeed having some utility in certain cases, I cannot recommend having them done unless done in a serious hospital without a financial incentive (ie., without financial connections to the clinician ordering them), and without a very obvious scope of investigation that could not already be seen in MR or CT imaging. (Fixed rotatory subluxation of the atlanto-axial joint). I have seen several patients misdiagnosed and become almost paralyzed by anxiety due to an increased Grabb-Oakes measurement where the dens is just barely in tangent with the brainstem, despite zero evidence of actual compression nor signal changes in the brainstem and with normal neurological examinations without any upper motor lesion signs! In 18 patients, dynamic images showed vertical, mobile and at-least partially reducible atlantoaxial dislocation. More commonly, however, a due to asymmetrical tearing of the covering ligaments, rotational subluxation or frank luxation is seen according to the Fielding & Hawking classifications (1977): Type 1, 2, 3 and 4, wherein types one and two are the most commonly encountered ones. This may cause the patient to become afraid and to google their symptoms, which in and by itself is reasonable enough. Moderator. Surgery is often challenging because of the shape of the C1 and C2 bones, and because the vertebral arteriespass in and around these two bones on the way to the brain. Common arguments for treatment may be claims that, although the MRI and even upright MRIs are normal, their own DMX scan is positive, or that the MRI, which was deemed normal by the local hospital, in reality shows signs of ruptured ligaments and that this fits with the patients symptoms. Would this mean that upper cervical chiropractors (orthogonal, blair technique, gonstead, etc.) Atlantoaxial instability is a relatively frequent finding in individuals with Down syndrome. If your child has symptoms of AAI, the doctor will suggest an X-ray. This is not dangerous, but can cause some popping, restriction in movement, and some pain upon articulation. In cases of hyperlaxity, It is not uncommon to find subaxial cervical alterations (levels below C3 to C7 . Atlanto-axial rotatory fixation. 2019) have documented numerous symptomatic cases of jugular vein stenosis at the craniovertebral junction. Now, the I was told is clearly second-hand information, and I cannot guarantee its accuracy. Org. One is especially predisposed to this problem if the affected vertebral artery is highly dominant (much higher caliber than its contralateral counterpart) or if the contralateral artery is extremely hypoplastic, or, finally, the contralateral artery terminates as the posterior inferior cerebellar artery rather than at the basilar artery (Josy & Daily, 2015). This conformation may be associated with thickening of the interarcuate ligament (atlantoaxial band), which has been interpreted as an indicator for instability in the atlantoaxial joint [79]. Booking Flexion and extension imaging fails to demonstrate any sort of brainstem compression. The renowned scholar and neurosurgeon professor Atul Goel was the first person, to the best of my knowledge, to acknowledge and document the notion of horizontal misalignment of the craniocervical facet joints and that this would often be present despite a completely normal-looking mid-sagittal slice (where most craniovertebral junction measurements are done). Myran R, Kvistad KA, Nygaard OP, Andresen H, Folvik M, Zwart JA. Medullopathy (signal changes, cord damage) will not occur by mere deflection, which is also evident by the blatant lack of upper motor neuron findings in these alleged brainstem compression patients. She started researching on certain online forums, in which she was advised to look into AAI and CCI. I recommend first measuring the degree of rotation between the C1 and C2 by drawing a line from the bifid process to the middle of the anterior aspect of the vertebra, and then another line from the posterior to the anterior tubercles of the C1. There is a growing trend, however, within the (or, at least, certain) alternative medical communities, where patients with normal or virtually normal imaging, and with the absence of clinical triggers that would suggest atlantoaxial or craniocervical instability, still end up diagnosed with these relatively sinister diagnoses. Dashti SR, Nakaji P, Hu YC, Frei DF, Abla AA, Yao T, et al. Conveniently, she was sent out to a colleague for very expensive nonsense therapy (again, regardless of lacking serious findings that would require surgery) and sent tens of thousands of euros on stemcell and prolotherapy procedures in a desperate attempt to avoid the inevitable wheelchair. 15 Piscataqua DriveNewington (Portsmouth), NH, 03801 603-431-3600, 8 Maple Street, Suite 2 Meredith, NH 03253 603-279-1117, 2023 All rights reserved | Sitemap | Legal | Law Firm Essentials by PaperStreet Web Design, Caudal Cervical Spondylomyelopathy (Wobblers). Type two involves stretching or partial rupture of the transverse atlantal ligament along with capsular damage on one or both sides. Spine (Phila Pa 1976). Magnetic resonance imaging assessment of the alar ligaments in whiplash injuries: a case-control study. This Out of these, the cookies that are categorized as necessary are stored on your browser as they are essential for the working of basic functionalities of the website. This is a major component in the workup for TOS CVH). The ligaments supporting these joints are quite strong, but if they become Ultimately, the reader must discern for themselves. The BDI was 6mm and the BAI was 8mm, which are all farily normal. He specializes in the treatment of chronic pain and has developed several distinctive protocols both with regards to diagnosis and conservative rehabilitation of difficult conditions. Atlantoaxial (AAI) and craniocervical instability (CCI) are two potentially sinister diagnoses that cause damage to the segmental neurovascular structures due to overmobility of the upper cervical spine. AAI and CCI are diagnoses that mainly cause the risk for either brainstem damage or injury to the arteries that supply the brain with blood, and this can cause paralysis or stroke if left untreated in cases where there is legitimate evidence for pathology. 2011 Apr;15(1):41-47. The BDI indicates vertical-, and the BAI horizontal structural integrity. This is important to understand, because maximal rotation will induce, and neutral position will stop the symptoms in patients with legitimate vascular conflict in AAI. BDI, ie. It is, as we say, in tangent with the dens and tectoral ventrally alone. More information about surgical treatment. Specialist imaging research to help diagnosis. Styloidectomy and Venous Stenting for Treatment of Styloid-Induced Internal Jugular Vein Stenosis: A Case Report and Literature Review. Supine cervical MRI including T2-w sagittal-oblique sequences at 2mm slice thickness (disc and foraminal health is best evaluated on a supine MRI). The atlantoaxial segment consists of the atlas (C1) and axis (C2) and forms a complex transitional structure bridging the occiput and cervical spine. The brainstems were completely void of evidence for compression in both cases, and there was no evidence of signal changes (consistent with brainstem damage) on MRI. Out of these, the cookies that are categorized as necessary are stored on your browser as they are essential for the working of basic functionalities of the website. English. Burry et al (1978) documented a rare case of lateral luxation in a patient with rheumatoid arthritis, in which the supporting facet had eroded away. If this was the case, ie., if the brainstem and medulla was being stretched, then the patient would highly likely get neurological symptoms that improve with extension and worsen with flexion (as patients with legitimate tethered cord syndrome do), and would certainly have a positive Slump test, a test which stretches the spinal cord. This is easily seen on imaging, especially on CT, as the alignment of the joint will be unequivocally abnormal to the extent that would not be achievable without tremendous ligamentous injury. This, however, is very rarely the case with this patient group in my experience. Both measurements tend to worsen with neck extension. Request Appointment. Why would you jump to the worst possible explanation, and especially when lacking apt evidence? Facetal locking with rigid torticollis (Cock Robin syndrome) or similar, in cases where there is no neurological compromise, is less dangerous. At Dr Gilete we are experts in Ehlers Danlos surgery, craniocervical instability EDS,neuro and spine disorders related to EDS and whiplash. A common but severely ignorant misunderstanding that some clinicians make (the patient cannot be blamed for thinking like this, but the clinician should set it straight), is the notion that mild to moderate ligamentous instabilities makes the neck (or the whole body for that matter) tense up to protect against the ligamentous instability, even though there are minimal or no clear MRI findings to support this notion, and that this somehow causes all of the patients symptoms. Atlantoaxial instability (AAI) is the term for increased motion at the joint between the 1st and 2nd cervical vertebrae (the atlas and the axis). Then the patient can make an informed decision about whether or not they want to invest in experimental therapy. Compare the two to obtain the degree of rotation. For example, if there is a C4-5 anterolisthesis with resultant chronic radiculopathy, C4-5 ADCF would often be utilized as operative treatment. Lateral cervical x-ray and flexion-extension views can give us complementary information in regards to atlantoaxial instability, although it does not seem indicated as the first choice method of diagnosis. 2021 Jun;44(3):1553-1568. doi: 10.1007/s10143-020-01345-9. My poor baby has become completely lame and incontinent in the last 48 hours. Most dogs with AA instability will develop clinical signs within the first 2 years of life, often after a seemingly mild traumatic event. Having a strong neck and good posture helps a lot as well (details on what this entails can be read in my article on atlas instability). Some rare cases have also demonstrated rotary compression of the vertebral artery in the lower neck due to arthritis or disc bulges that fills up the transverse foraminae (Ujifuku et al. 2009), but this is extremely rare. She was also said to have ventral brainstem compression, which particularly scared her due to her difficulties with respiration. Last Update [site_last_modified date_format=Y-m-d H:i:s]. Because of its role in movement, it is, unfortunately, commonly injured. The dorsal lamina of the atlas shifts caudally and ventrally against the spinous process of the axis. This, with or without accompanied neurological symptoms, be it vascular or neurological. This madness must stop. Suboccipital symptoms that occur only with cracking, if the MRI shows arthritis or joint effusion, especially if the neck locks in rotary fixation, then this could be a case of legitimate AAI or CCI. How is possible for them to have results when there is no symptomatic AAI/CCI? Global Spine J. Patient resources for the Down Syndrome Program. Our surgeons provide a full range of treatments including non-surgical options as well as surgical repair. Faris AA, Poser CM, Wilmore DW, et al.. Radiologic visualization of neck vessels in healthy men. I have not receiving anything that comes close of what they produce. In patients with Ehler Danlos syndrome, instability is present frequently in several segments, generally C0-C1-C2 (from occipital to axis). We offer diagnostic and treatment options for common and complex medical conditions. For example, although the medical literature (almost exclusively biased reports written by people considered experts on the topics (I am also biased on the topic; all experts are) may suggest a clivo-axial angle lower than 150 degrees as abnormal, this is still a measurement used to associate concrete craniocervical angles with medullary compression. Look for signs of retinal hypertension (subtle copper wiring, AV nicking, tortuosity of the arterioles, generalized vasospasm or papilledema. If its caused by rotation (rare), manipulation may temporarily improve jugular outlet passage, but it will not last. 10 things you should know about Cervical Disc Replacement. But this measurement in and by itself, when it is 9 or 10 or even higher, but there is no brainstem compression not even in flexion-extension imaging this cannot be interpreted as a surgical indicator. In reality, in legitimate cases of atlantoaxial or craniocervical instability, the instability may cause a potentially dangerous neurovascular conflict, as mentioned initially, where the brainstem or vertebral arteries can get damaged. Ujifuku K, Hayashi K, Tsunoda K, Kitagawa N, Hayashi T, Suyama K, Nagata I. Positional vertebral artery compression and vertebrobasilar insufficiency due to a herniated cervical disc. Save my name, email, and website in this browser for the next time I comment. However, if there is obvious compromise of a ligament but there is no evidence of sinister hypermobility or structural displacement (eg., very high ADI), the ligamentous should be further examined with high-resolution T2 FLAIR imaging with low slice thickness (supine imaging!) Lack of signal change in the cord, and especially when it is not being compressed from both sides, is not a case of brainstem compression, Mild to moderate ligamentous compromise in cases where all measurements are normal or nearly normal, and there is no neurovascular compression, is generally NOT a surgical indication nor an indication for aggressive treatment. A patient with positional brainstem compression due to TAL rupture, for example, will develop neurological (ie. The report claimed that there were signs of ligamentous rupture and bidirectional subluxation upon rotation in the atlantoaxial joints. to get a better impression of its actual thickness. Most cases of mild to moderate unilateral compression, sometimes even intermittent occlusion, is asymptomatic due to contribution from the contralateral VA (Faris et al. 14 Postoperative care advices following cervical disc herniation surgery, 4 Predictive factors of the results in Cervical Herniated Disc surgery. We also use third-party cookies that help us analyze and understand how you use this website. This increased mobility causes headache and cervical pain as well as signs of compression of adjacent neural elements that form cervicomedullary syndrome. The deep neck flexors should not engage as this lessens the compression. We also use third-party cookies that help us analyze and understand how you use this website. Regardless, be it rooted in benevolent or malevolent intention, this does not change the fact that pursuing the diagnosis and especially its related treatment (conservative or surgical strategies) are extremely expensive and potentially dangerous as well. We can consider that there is atlantoaxial instability or atlantoaxial subluxation (AA subluxation) in cases where there is principally incompetence of the ligamentous elements of the atlantoaxial (C1-C2) joint, which allow a significant increase in the mobility of this area thus considered pathological mobility. The atlantoaxial joint is normally stabilized by a projection off the axis called the dens, which fits into the atlas, as well as several ligaments between the two bones. There are two causes for the instability, trauma and birth abnormalities. Traumatic instability occurs after forceful flexion of the head, En este folleto, aprender sobre la IAA y cmo afecta a las personas con sndrome de Down. Some have proposed 2mm of translational difference, but this is completely unreliable in my opinion and exprience. Uniondale, NY 11553. Grabb-Oakes interval is another measurement that is often misunderstood. Patients with severe ligamentous compromise and a risk for actual dangerous secondary potentially pathologies, must have instability so aggressive that it can cause damage to the brainstem or adjacent cerebro-arterial supply. Type three involves anterior subluxation of the entire atlas due to combined full rupture of the TAL and partial rupture of the capsules and other structures. PMID: 33064218. Albeit still a surgically treated problem. Hopefully, this is the result of ignorance combined with poor clinical workup skills (incompetence) and not mere greed and malevolence. It means that the instability is, or will probably, shortly, become bad enough to carry the potential to damage nerves or blood vessels. However, can we say the same if there is major guesswork involved in the rendering of the diagnosis? Upright MRI has very low quality and because of this, there is a lot of guesswork involved in its interpretation. Journal of Neuro-Ophthalmology 2013;33:330337doi: 10.1097/WNO.0b013e318299c292, Alkhotani A. Cerebrospinal Fluid Rhinorrhea Secondary to Idiopathic Intracranial Hypertension. 1963;13(5):386396. Li M, Gao X, Rajah GB, Liang J, Chen J, Yan F, et al. Sometimes flexion-extension and rotational imaging is necessary. The term AAI can also be used in cases of transverse ligament rupture, in which the odontoid process (the axis of the C2) may, especially if there is also damage to the tectorial membrane, dislocate dorsally and compress the brainstem. About Some top offenders may suggest full craniocervical fusion, ie. The CXA was 138 degrees and the Grabb-Oakes measurement was 8,3mm. Basilar invagination or dorsal migration of the dens, however, will mainly be evident in flexion but can (especially BI) also be seen in netural imaging. Washington University neurosurgeons have extensive experience treating problems in this area and are recognized nationally as experts in providing innovative treatments for this unique and complex area of the neck. A caveat here may be if the the translational value is very high, as this would be a reasonable indication of foreseeable joint damage, but there is no consensus in the literature with regards to how much that is. We examined 404 patients with this chromosome disorder and observed their atlanto-dens intervals and spinal canal widths to be significantly different from children without Down syndrome. My experience has been that these approaches do not work, and certainly do not cause long term results. ), induction of symptoms (all or nearly all of your symptoms, not some neck pain) with maximal rotation, nor during flexion or extension. Secondly, and perhaps more importantly, the extent of facetal overap must be measured. She had been out from work for one year at the point of consultation, but her doctors could not find anything wrong with her. Brainstem compression, when symptomatic, will usually cause quadriparesis along with phrenic nerve palsy. Copyright 2007-2023. Neurosurgery. Both tests should evaluate the movements of the occipitoatlantoid and atlantoaxial joints. Another patient was told by a well-known pain physician in the US that she had brainstem compression and required several expensive prolotherapy procedures. Moreover, I have heard numerous similar stories from other patients. Patients with normal structural alignment and more or less normal or completely normal radiological imaging, without clinical correlation, end up diagnosed with CCI or AAI due to a slightly low (non-sinister) CXA, say 135 degrees, and some signal changes in the alar ligaments on T2 FLAIR imaging or slight increase in the atlantodental interval (ADI) despite normal thickness of the transverse atlantal ligament (TAL). Larger breeds can also be affected, and any dog or cat is at risk of a very similar acquired injury if they sustain trauma, such as being hit by a car. Typically, complete membraneous ruptures of the CVJ may cause dislocation between the head and neck, resulting in positional dissociation between the the two. A critical view on the overdiagnosis of AAI/CCI. The patient will hinge back at their neck while simultaneously flexing the cranium. Patients with craniovenous outlet obstruction due to JOS may induce their symptoms with a Queckenstedts test, that is in essence a manual compression test of the internal jugular veins. What cervical artificial disc should I choose? Clunking, clicking and pain in the upper neck. For example, I have seen patients with 45 degrees of rotation (which is higher than normal) between the C1-2 that had completely normal overlap due to large facets, and I have seen patients with 30 degrees of rotation (which is usually completely normal) with poor overlap and AAI, due to small facetal surfaces. Therefore, when I hear about patients being operated on with no other abnormality than a CXA of 140 degrees, my opinion is that this is reckless butchery. Diagnostic imaging: Spine, 3rd edition. The exam should be done lying down, without a neck pillow. The atlas can sublux anteriorly, posteriorly, laterally, or vertically. This category only includes cookies that ensures basic functionalities and security features of the website. Now, it is true that specialty diagnoses can be missed by local generalists. Moreover, genuine cases of brainstem compression causes paralysis and other upper motor neuron signs, and will present with syringobulbia or compressive bulbopathy. This site complies with the HONcode standard for trustworthy health information: verify here. We'll assume you're ok with this, but you can opt-out if you wish. 1978 Dec;37(6):525-8. doi: 10.1136/ard.37.6.525. fusion from the head, all the way down to the T1 or T2 vertebrae, even though there may be zero evidence for major neurovascular conflict. 1977;59 (1): 37-44. The ligaments holding the bones together can also be injured in trauma, or weakened in certain inflammatory conditions such as rheumatoid arthritis or Downsyndrome. nr. Whats interesting, regardless, is that one year after we had the first consultation she underwent another uMRI (due to lack of improvement of symptoms), which showed completely resolution of the atlantoaxial subluxations, which were now overlapping at about 30%; 300% improvement (remember: >20% is normal). DOI: https://doi.org/10.35975/apic.v24i1.1230. This is Bow hunters syndrome, and may be caused by legitimate atlantoaxial instability. PMID: 24475346; PMCID: PMC3899735. Epub 2014 May 22. Gweon HM, Chung TS, Suh SH. This, once again emphasized if the patient also does not induce any sinister symptoms in the positions where the alleged instability occurs. 2020). Traumatic ligamentous ruptures or gradual deterioration of joint stability may cause basilar invagination, which is a degenerative process causing the odontoid process to graduall migrate into the head via the foramen magnum. 2014). It is advisable to obtain just a lateral view first. This category only includes cookies that ensures basic functionalities and security features of the website. Patients with legitimate CCI or AAI will generally have intermittent induction of symptoms with full rotation, flexion or extension that resolves in netural position, presuming there is no constant crushing of the brainstem or vertebral artery dissection. Surgical reduction and fixation would be the only appropriate treatment. 1963). Acute or chronic spinal cord compression causing clinical signs consistent with an upper cervical myelopathy can result from this instability [2]. First, need I mention the notion that there is tremendous money in this patient group, and that if treatment goes wrong, becuase they have already burned their bridges with their GPs, no one will listen nor care? If you have an atlanto-dens interval (ADI) of 5mm or greater, you have instability by definition. the basion-dens interval, is the distance between the tip of the clivus and tip of the C2. Surgery to address problems in this area can be risky. Most imaging is tends to be normal, except certain craniovascular workups, especially a CTV of the head, TOS workups, and doppler of the carotid and vertebral arteries (not positive for hypoperfusion, but hyperperfusion).

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