He also found that severe misalignment of these joints were often associated with Chiari malformation, basilar invagination, and various other pathologies. To compress the brainstem it must be compressed from both sides, both infront and behind. Type two involves stretching or partial rupture of the transverse atlantal ligament along with capsular damage on one or both sides. to analyze our web traffic. What muscles would need to be strengthened to prevent the ADI from opening up? After the preoperative analysis of the Magnetic Resonance Imaging (MRI) and CT scan of each patient, we perform a thin sliced preoperative CT oriented towards neuronavigation that will be carried out during surgery. This can also promote anterior dissociation of the head which will lead to an abnormally high basion-axial interval (BAI Harris measurement) of more than 12mm (Ross & Moore, 2015). We use cookies and other tools to enhance your experience on our website and Prior to surgery we perform a surgical planning of the intraoperative neuronavigation to confirm the trajectories of screws and special anatomical dispositions of structures. Luxation of the atlantoaxial joints, ie., luxation that surpasses what is seen in Cock Robin syndrome, can also occur with traumatic and gross ligamentous rupture. If not, does the patient actually have any significant symptom induction with rotation? 2014 Aug;4(3):197-210. doi: 10.1055/s-0034-1376371. Traditional cases of atlantoaxial instability and craniocervical instability require obvious imaging findings with strong clinical correlation, and, when its criteria are met, are certainly treated (operated) in any skilled and compatible neurosurgical ward. Necessary cookies are absolutely essential for the website to function properly. What I prefer to do is to first draw lines that show the actual rotational alignment of the C2 and C1 when looking left and right. medullary) symptoms when looking down, and will tend to improve when pulling the head up and back. Booking Generally, however, in ligamentous laxity, some bowing and lateral hypermobility (evident by lateral flexion overhangs) will almost definitely not result in frank luxations down the line nor do they tend to elicit symptoms from the actual atlantoaxial facet joints. When these muscles get tight (due to profound weakness), due to poor posture and movement patterns, or, as well, in many cases due to head or neck trauma, restricted joint movement will occur and popping and cracking, even loud clunks can occur. (2019) documented another case where a patient with RA developed odontoid fracture and subsequent anterolateral subluxation of the atlantoaxial joint. Additionally, spinal instability in the form of spondylolisthesis 1. Diagnostic markers for occult craniovascular congestion. But we must see adequate imaging as well as adequate clinical fulfillment of diagnostic criteria to render these diagnoses; it is not enough to feel neck clunking, upper cervical pain, weakness in the neck or wobbleheaded. Once the diagnosis of atlantoaxial instabilityis made, one should consult the neurologist, neurosurgeon, and a geneticist if the patient is a child. Pearls and Other Issues The atlantoaxial segment consists of the atlas (C1) and axis (C2) and forms a complex transitional structure bridging the occiput and cervical spine. At Dr Gilete we are experts in Ehlers Danlos surgery, craniocervical instability EDS,neuro and spine disorders related to EDS and whiplash. Wake up and walking begins on the second day after surgery. Many of these patients who have been misdiagnosed with AAI or CCI may feel neck wobbliness, heaviheaded, neck weakness, and clicking or clunking in the neck upon movement, often along with upper neck pain. Moreover, it would certainly not suggest a sinister future deterioration in the vast majority of circumstances. 2000). We were referred to a specialist vet (swift in Wetherby) who thinks it is AAI but unless she regains use of her legs they cannot operate The problem begins when certain nonsensical articles about CCI and AAI, that do not properly explain relevant clinical correlation nor imaging requirements, but rather, just lists a set of associated symptoms, finds favor in the patient. Some have proposed 2mm of translational difference, but this is completely unreliable in my opinion and exprience. This, of course, must be evaluated on a case-to-case basis. <9mm), which overestimate the pathologies and are much misunderstood due to unrealistic consensus of what is normal) will clearly be abnormal, such as the Harris measurement (BAI), basion dens interval (BDI), or Powers ratio. 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 the patients neck often completely locks up due to facetal luxations, then atlantoaxial fixation may certainly be a viable option for treatment, especially if conservative stabiization fails (capsular and alar ligamentous prolotherapy, postural corrections, strengthening of the suboccipital, longus capitis and levator scapulae muscles). 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. 333 Earle Ovington Blvd, Suite 106. Journal of Neuro-Ophthalmology 2013;33:330337doi: 10.1097/WNO.0b013e318299c292, Alkhotani A. Cerebrospinal Fluid Rhinorrhea Secondary to Idiopathic Intracranial Hypertension. Mild to moderate cases tend to respond well to appropriate conservative therapy (not general therapy), cf., once again, my atlas joint article from 2017 linked several times earlier. How is possible for them to have results when there is no symptomatic AAI/CCI? 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. We'll assume you're ok with this, but you can opt-out if you wish. Both positional (ie., upright. 10 things you should know about Cervical Disc Replacement. I recommend sticking to clinics that have good reputations and good imaging protocols. Specialist imaging research to help diagnosis. A patient with positional brainstem compression due to TAL rupture, for example, will develop neurological (ie. Thus, the patients in the rotary subluxation group are expected to present with severe and sudden neck pain as well as rigidity to the extent of being unable to move the neck. What does this mean? 3. The other side of the AAI/CCI coin is the risk for facetal luxation; a less sinister-, but still a problem that warrants surgical treatment. If the brainstem compression is not positional, ie., it is seen even on neutral imaging, then the symptoms would be expected to be constant. In previous epidemiologic studies, the prevalence of atlantoaxial instability in persons with Down syndrome was found to be between 9% and 31%. Kjetil has also published several peer-reviewed studies on musculoskeletal and neurological topics. It will rarely cause frank luxation, however where the facets dislocate and lock laterally. This means routine X-rays are not helpful. Pain medications and anti-inflammatories are typically also prescribed. 2009 Sep;11(3):326-9. doi: 10.3171/2009.4.SPINE08689. In people with Down syndrome, the ligaments (connections between muscles) are lax or floppy. 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. This webpage is intended to provide health information so that you can be better informed. Epub 2014 May 22. Powers ratio will be abnormal in cases of both BI and craniocervical dissociation (Ross & Moore, 2015). collected, please refer to our Privacy Policy. https://doi.org/10.13104/jksmrm.2011.15.1.41. Atlanto-axial instability is a potentially dangerous condition where the ligament between the atlas (C1`) and axis (C2) vertebrae at the top of your neck is partially torn. This, seriously augmented by poor hinge neck postures (Larsen 2018). Because this article is, in essence, just another opinion piece, let us then focus on logical reasoning and objective arguments. In some circumstances, gradual degenerative basilar invagination can also occur due to gradual and progressive degenerative horizontal misalignment of the atlantoaxial joints (Goel 2014), due to certain diseases such as rheumatoid arthritis, but it is usually caused by head and neck trauma. Strong evidence of clinical correlation must be present from a clinician that is familiar with the signs and triggers in upper cervical instability-cases. Common findings: Ovalization of the orbitae, dilated optic nerve sheaths, pituitary concavity, Chiari malformation, tight brain appearance, jugular vein compression with or without white-vessel signs, dilation or narrowing of the lateral and possibly third ventricles, periventricular ependymal T2 FLAIR hyperintensities), Neck MRI (general evaluation of the neck integrity), CT angiogram of the head neck and subclavian arteries with the arms raised (contrast infusion via femoral vein. PMID: 32623537; PMCID: PMC8121728. This, as significant irritation of the brachial plexus can also cause autonomic coaffection (Larsen et al 2021) and thus derange the function of the phrenic nerves, which in turn control the diaphragm. The findings may be quite subtle and are easy to miss outside of dynamic exams. Global Spine J. J Korean Soc Magn Reson Med. My symptoms are mostly sitting or standing but better laying down, wont doing the CT angiogram then become useless if I do it laying down (my symptoms are dysautonomia-like when standing). The surgical treatment for Atlantoaxial instability, when it manifests alone without occipitocervical instability, it mainly consists of a posterior fusion of the first cervical vertebra (C1 or Atlas) and the second cervical vertebra (C2 or Axis). A 32 year-old female patient contacted me in 2019 as she had been diagnosed (by a radiologist alone) with craniocervical and atlantoaxial instability. 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. The patient had headache, dizziness, fatigue, pain in the arms and chest and often felt difficulty breathing. Unfortunately, she was not compliant to the treatment that I prescribed (TOS, TOS CVH) other than the treatment for AAI, which she was convinced that was her problem. Fielding JW, Hawkins RJ. The report claimed that there were signs of ligamentous rupture and bidirectional subluxation upon rotation in the atlantoaxial joints. Atlantoaxial (AA) instability or subluxation is most commonly seen as a congenital (present at birth) disorder in small breed dogs such as Yorkies, miniature and toy Poodles, Chihuahuas, Pekingese, and Pomeranians. Either way, if positive, move on to confirm narrowing of the jugular passage between the styloid process and C1 transverse process on a CT scan. Advanced Surgical Neuro-oncology Fellowship, Complex and Minimally Invasive Spine Deformity Fellowship, Endovascular Surgical Neuroradiology Fellowship, Neurosurgical Spine Innovation Fellowship, Neurosurgical Peripheral Nerve and Spine Fellowship. Congenital, inflammatory, traumatic, The utmost majority of these patients have have normal supine imaging, and many of them also normal or nearly normal upright imaging. Some top offenders may suggest full craniocervical fusion, ie. 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. Epub 2020 Jul 4. DOI: 10.3171/2015.1.FOCUS14791. I dont recommend MRA. The atlanto-axial (AA) joint is the joint between the first (atlas) and second (axis) vertebrae (bones) in the neck. Necessary cookies are absolutely essential for the website to function properly. That is why they are much less affected by actual neck position than legitimate CCI AAI patients are, and certainly do not become symptom free in neutral positions. I told her clearly that her brainstem was normal and that she did not have any positional induction of 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). to get a better impression of its actual thickness. Myran R, Kvistad KA, Nygaard OP, Andresen H, Folvik M, Zwart JA. TOS increases perfusion rates to the brain, to which the brain is very sensitive and may dysfunction depending on how high the pressures are (Larsen et al 2020), often resulting in severe fatigue, dizziness, headaches and especially occipital headaches/pain (these are hypertensive headaches, not an atlas problem). 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. The bones are susceptible to fracture from high-energy impact such as falls or car accidents, especially in the elderly. Compression of the glossopharyngeal nerve will frequently cause pharyngeal pain (back of the throat pain) whereas vagal compression may lead to dry coughing, lump in the throat feeling, ear itching and various strange things when unilateral, but has been associated with more problematic issues when bilateral such as gastroparesis (Waldock et al. DMX I dont recommend getting a DMX. Atlantoaxial and craniocervical instability are both real and potentially sinister diagnoses that require treatment. For TOS CVH the patient will generally feel better when stress is reduced along with taking beta blockers (confer with your doctor). For occipial neuralgia, an ultrasound guided nerve block will cure these symptoms for three hours and thus confirm the diagnosis. Because it doesnt work most of the time, and doesnt cause any lasting results.