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TYPE OF SCANS AND TESTS SUPINE MRI An MRI is a scan that uses strong magnets and radio waves to create detailed pictures of the inside of your body — especially soft tissues like the brain, spine, muscles, and organs. It does not use X-rays or radiation, unlike a CT scan. A supine MRI is the most common type, supine means laying down. UPRIGHT MRI (uMRI) An Upright MRI is a type of MRI scan where you are sitting or standing instead of lying down. It takes images of your body while it's under natural gravity and posture — which can reveal problems that don’t show up when lying flat. Often it's done as a dynamic scan with flexion-extension positioning. Upright MRI is the preferred imaging for diagnosing CCI. Unfortunately upright MRI machines are hard to come by, often requiring you to travel to find one. You can find the locatations of uMRIs in europe here . CT SCAN A CT scan(Computed Tomography) is a scan that uses X-rays and a computer to create detailed pictures of the inside of your body. It’s like a more advanced X-ray that shows cross-section images (like slices of your body), allowing doctors to see bones, organs, blood vessels, and soft tissues more clearly. ROTATIONAL CT SCAN A Rotational CT scan is a special kind of CT scan (Computed Tomography) where imaging is done while the head or neck is rotated to the left and right, not just in a neutral (still) position. Instead of just one static picture, it gives multiple 3D images of the bones at different head positions (usually at full left rotation, full right rotation, and neutral). This is the preferred way to diagnose AAI, also called rotational instability. CONE BEAM CT (CBCT) A CBCT scan is a special type of CT scan that uses a cone-shaped X-ray beam to create 3D images, mostly of the head, neck, teeth, jaw, and sinuses. It gives high-detail images of bones and teeth with lower radiation than a regular CT. Recently this scan has been used to create dynamic (flexion/extension) imaging, as many don't have access to an upright MRI. This scan is useful in diagnosing both CCI and AAI. MR/CT VENOGRAM A venogram is a imaging test that shows how well your veins are working. It uses contrast dye to make the veins visible on scans, allowing doctors to see blockages, narrowing, or abnormal blood flow. This test is especially useful for detecting problems in deep veins that can't be seen with ultrasound alone.It can be done both as an MRI and as a CT. It's one of the scans used to diagnose jugular stenosis and transverse sinus stenosis. MR/CT ANGIOGRAM An angiogram is a imaging test used to visualize blood vessels — especially arteries — to detect problems like narrowing, blockage, bulging (aneurysm), or abnormal connections. The word "angio" means blood vessel, and "gram" means picture — so an angiogram is literally a picture of blood vessels. It can be done as either CT with contrast or MRI with or without contrast. It can be invasive, involving a catheter (thin tube) is inserted into an artery (usually in the groin, wrist, or neck) or non invasive. It's one of the scans used to diagnose jugular stenosis and transverse sinus stenosis. FLEXION-EXTENSION X-RAY A flexion-extension X-ray is a special type of X-ray imaging used to take pictures of your spine while it’s bent forward (flexion) and bent backward (extension). This is often the first scan a doctor orders when instability is suspected, however it is rarely enough to diagnose ligamentous laxity related instability such as CCI. DIGITAL MOTION X-RAY (DMX) A Digital Motion X-Ray (DMX) is a special type of real-time X-ray that shows how your bones and joints move while you're actually moving — like turning your head or bending your neck. It’s like a video X-ray, allowing doctors to watch your joints in motion to detect problems that don’t show up when you're still. It's used by some practitioners to diagnose CCI, AAI and subaxial (below c2) instability. LUMBAR PUNCTURE (LP) A lumbar puncture (LP) is a procedure that measures cerebrospinal fluid (CSF) pressure in the lower back — which indirectly reflects the pressure inside the skull.After cleaning the skin and numbing the area, a thin, hollow needle is gently inserted between two vertebrae in the lower lumbar spine (usually between L3–L4 or L4–L5). The needle passes into the subarachnoid space, where CSF flows around the spinal cord. Once the needle is in the correct space, it’s attached to a manometer — a small, clear, vertical tube that measures pressure. ICP BOLT TEST An ICP bolt (Intracranial Pressure bolt) is a small, screw-like device used to measure pressure inside the skull (intracranial pressure) directly and continuously. The ICP bolt is a type of invasive pressure monitor. It’s called a “bolt” because it’s a hollow screw that anchors a tiny pressure sensor into the skull. INVASIVE CERVICAL TRACTION (ICT) Invasive cervical traction is a medical procedure where a pulling force is applied to the cervical spine (neck) using surgically placed pins or screws in the skull or spine to help realign, decompress, or stabilize the neck bones. Unlike regular traction (which uses external devices like a neck harness), invasive traction involves inserting hardware into bone to apply more precise or stronger traction. Some doctors use ICT to help determine if a fusion surgery would help alleviate symptoms HALO VEST TRIAL A Halo Vest Trial is a temporary, non-surgical test used to see whether stabilizing the head and neck with a rigid device (a halo vest) improves a patient’s symptoms — especially in cases of Craniocervical Instability (CCI) or Atlantoaxial Instability (AAI).

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ABOUT This page is made by a patient, for patients. When i was diagnosed i scoured the internet for information about CCI and it's comorbidities. Unfortunately i didn't find much, and what i found was either anecdotal or hard to understand. I was missing an information hub that was backed by evidence and reputable sources, yet easy to understand despite the cognitive difficulties my conditions gave me. So once i started to get better, i decided to create one myself and hopefully give back to a community who's helped me survive the journey. That's what brought us to this place. I hope this page can be everything i needed, to someone else.

SLIDESHOWS CCI/AAI Neurosurgical management of Hereditary Hypermobility Connective Tissue Disorders Cervical instabilities in the EDS population Recognition of Craniocervical Instability in complex Chiari patients Retethering in Ehlers-danlos syndrome Urological manifistations of Tethered cord syndrome Tethered cord syndrome in Ehlers-Danlos syndrome Tethered cord IJVS + IH

Our research database contains a large amount of studies and scientific papers relating to brain and spinal conditions. All documents are peer reviewed and published in reputable journals. In cases where it hasn't been possible to verify all information, or verify that it has been peer reviewed, the file will be marked with a ❋. These are predominantly slideshows/powerpoint presentation from webinars hosted by specialists.

CEREBROSPINAL FLUID LEAK A cerebrospinal fluid (CSF) leak occurs when the cerebrospinal fluid, which cushions and protects the brain and spinal cord, escapes through a hole or tear in the dura mater, the tough outer membrane surrounding the brain and spinal cord. This fluid can leak out through the nose, ears, or spinal area, depending on where the tear is located. A CSF leak can cause the pressure within the spinal canal to drop, leading to an array of symptoms SYMPTOM S - Headaches: Often described as positional headaches, which worsen when standing or sitting up and improve when lying down. Often accompanied by neck stiffness and pain - Clear Fluid Drainage : Clear, watery fluid may drain from the nose (rhinorrhea) or ears (otorrhea). - Nausea and Vomiting : Associated with headaches and intracranial pressure changes. - Hearing disturbances : Hearing loss and ringing in ears (tinnitus) - Visual Disturbances : Blurred vision, double vision and sensitivity to light (photophobia) - Imbalance : Dizziness and a sense of imbalance might occur CAUSES - Trauma : Head or spinal injuries can cause tears in the dura mater. - Surgery : Neurosurgery or spinal surgery can sometimes inadvertently create a tear. - Spontaneous Leaks : These can occur without any obvious cause, often due to weak spots in the dura mater. - Increased Intracranial Pressure : Conditions that increase pressure inside the skull can sometimes lead to CSF leaks. DIAGNOSIS - Clinical Examination : Doctors look for clear fluid drainage and other signs of CSF leak. - Imaging Studies: MRI, CT or Myelogaphy scans can help identify the site of the leak. - CSF Tests: Fluid samples can be tested for beta-2 transferrin, a protein unique to CSF. TREATMENT 1. Conservative Measures: - Bed Rest: Encouraging the patient to lie flat to reduce pressure on the dura and allow it to heal. - Hydration: Increasing fluid intake to maintain CSF pressure. - Caffeine: Sometimes used to help reduce headaches. - Avoid Straining : Patients are advised to avoid activities that increase intracranial pressure, like heavy lifting or straining. 2. Medical Interventions: - Epidural Blood Patch : A procedure where a small amount of the patient’s blood is injected into the epidural space near the leak to form a clot and seal the tear. - Fibrin sealant: A special sealant made from human plasma is injected into the spinal canal to seal the leak. - Surgery: In cases where conservative measures fail, surgical repair may be necessary to close the tear in the dura mater. POTENTIAL COMPLICATIONS Potential Complications: - Meningitis: Infection due to bacteria entering the brain or spinal cord through the tear. - Chronic Headaches : Persistent headaches due to ongoing CSF leakage. - Neurological Symptoms : If the leak affects brain or spinal cord function. PROGNOSIS A CSF leak can significantly impact quality of life, but with proper diagnosis and treatment, many patients recover fully. SOURCES Mayo Clinic – CSF Leak https://www.hopkinsmedicine.org/health/conditions-and-diseases/cerebrospinal-fluid-csf-leak https://www.ncbi.nlm.nih.gov/books/NBK538157/ https://my.clevelandclinic.org/health/diseases/16854-cerebrospinal-fluid-csf-leak

EAGLE SYNDROME Eagle Syndrome is a condition where an elongated styloid process or calcified stylohyoid ligament in the neck causes compression or irritation of nearby nerves or blood vessels, leading to a range of head, neck, face, or vascular symptoms. ANATOMY What’s the Styloid Process? A thin, pointed bone located just below your ear. Normally around 2.5–3 cm long. In Eagle Syndrome, it’s often >3 cm and can press on: Cranial nerves (like glossopharyngeal, trigeminal, or vagus) Internal carotid artery Internal jugular vein TYPES There are two types of eagles syndrome Classic Eagle Syndrome : Nerve compression (often CN IX) Vascular Eagle Syndrome : Compression of artery or vein Polygon data were generated by Database Center for Life Science(DBCLS)[2] . CC-BY-SA-2.1-jp SYMPTOM S Classic (nerve-related): Throat or ear pain Pain with swallowing, turning the head, or yawning Sensation of a foreign object in the throat Jaw or facial pain Voice changes Vascular (vein or artery compression): Head pressure (especially when lying down or turning head) Dizziness or lightheadedness Pulsatile tinnitus (hearing your heartbeat in your ear) Visual disturbances Stroke-like symptoms (rare but serious) Often linked with internal jugular vein compression → increased intracranial pressure leading to a condition called Intracranial hypertension Symptoms can fluctuate depending on head position — turning or tilting can worsen or relieve compression. PATHOPHYSIOLOGY Anatomy The styloid process is a slender bone extending from the temporal bone of the skull, normally about 2–3 cm long. When elongated (often > 3 cm) or abnormally angled, it can impinge on nearby neurovascular structures. The stylohyoid ligament, connecting the styloid process to the hyoid bone, can also become ossified, acting like an extra bony bar in the neck. Eagle Syndrome typically manifests in two overlapping forms, each with distinct mechanisms. Vascular (Stylocarotid or Stylojugular Type) The elongated styloid process or calcified ligament compresses blood vessels, particularly: The internal carotid artery (ICA) The external carotid artery (ECA) The internal jugular vein (IJV) (between the styloid process and the C1 transverse process) Compression can lead to: Arterial irritation or dissection → transient ischemic attacks (rare) Venous outflow obstruction → increased intracranial pressure, headache, visual symptoms, tinnitus, cognitive fog This venous subtype is sometimes called “Styloidogenic Jugular Venous Compression Syndrome.” Mechanism : Mechanical narrowing → turbulent or reduced blood flow → venous hypertension → secondary intracranial hypertension symptoms. Neurological (Classic Eagle Syndrome) The styloid process or ossified ligament compresses or irritates nearby cranial nerves, particularly: Glossopharyngeal nerve (CN IX) – throat and tongue pain Vagus nerve (CN X) – voice changes, autonomic symptoms Trigeminal nerve branches – facial pain or jaw discomfort Facial nerve (CN VII) – sometimes causes ear pain Mechanism: Mechanical irritation or compression → local inflammation and neuropathic pain → referred pain to the throat, ear, or jaw. Positional Effects Turning or tilting the head can worsen compression — particularly of the internal jugular vein — explaining positional headaches or pulsatile tinnitus. Some patients develop dynamic compression seen only when the neck is rotated or extended on imaging (CT or MR venography). DIAGNOSIS Usually involves imaging to measure the length and position of the styloid process and look for vascular or nerve compression CT scan with 3D reconstruction is the gold standard (best to measure styloid) CT Venography or Angiography (CTV/CTA) – shows vascular compression Ultrasound or Doppler – for blood flow evaluation Dynamic rotational venography - shows how neck position changes venous outflow. MRV (Magnetic Resonance Venography) and cone-beam CT - provides less radiation TREATMENT Conservative management Management of pain and symptoms with medication Steroid or lidocaine injections around the styloid for diagnostic or temporary relief Physical therapy or posture correction in milder cases This approaches may relieve pain but don’t fix the structural problem (elongated styloid or compressed vein/artery). Surgical Treatment – Styloidectomy This is the definitive treatment for most symptomatic cases. Removal or shortening of the elongated styloid process May be combined with removal of calcified stylohyoid ligament Two Surgical Approaches: Intraoral: Through the mouth (tonsillar area). No external scar, but less visibility External : Small incision in the neck. Better visibility, safer for vascular cases, leaves scar Recovery time: 2–6 weeks Usually done under general anesthesia Low complication rate with experienced surgeons If Jugular Vein or Artery Is Compressed In vascular Eagle Syndrome, you may also need: C1 bone shave: C1 vertebra is also compressing the jugular vein Venous stenting : Vein remains narrowed even after bone removal Carotid artery decompression: If the elongated styloid presses on the carotid artery (rare) Complications The styloid process lies close to several cranial nerves — these can be stretched, irritated, or injured during surgery. Temporary soreness or tightness when swallowing is common. Rarely, pharyngeal scarring or dysphagia can persist, especially after intraoral approaches. Persistent or recurrent pain (if part of the styloid remains or scar tissue forms) Incomplete symptom relief (especially if venous compression is from multiple causes, e.g., C1 vertebra or ligament) Scar tissue or fibrosis causing tightness in neck muscles In rare cases, symptoms may recur if the styloid regrows or calcification continues. Picture showing measurement made on an elongated styloid PRE-SURGERY CONSIDERATIONS In vascular Eagle Syndrome, the elongated styloid process or calcified ligament can compress major blood vessels, most often the internal jugular vein (IJV) or, less commonly, the internal carotid artery (ICA). Before any surgical removal (styloidectomy), it’s crucial to determine exactly where and how the compression occurs. This matters because: Multiple structures can contribute to the narrowing. Sometimes it’s not just the styloid, the C1 vertebra (atlas) can also press against the vein. If only the styloid is removed and the C1 bone continues to pinch the vein, the symptoms such as head pressure, tinnitus, or dizziness may persist or return. Targeted surgery prevents incomplete relief. By performing pre-operative venography or dynamic imaging, surgeons can decide if the patient also needs a C1 bone shave to free up the jugular vein, or in some cases, a venous stent after surgery if the vein remains narrowed. It reduces surgical risk. Knowing which vessels are involved helps surgeons plan a safer surgical route and avoid complications related to nearby nerves and arteries. It distinguishes nerve-related from vascular symptoms. Classic (nerve-type) Eagle’s and vascular Eagle’s can overlap in symptoms, and detailed vascular evaluation helps confirm which mechanisms are at play so treatment can be tailored correctly. In short, proper vascular imaging before surgery ensures the real cause of compression is identified, the right structures are addressed, and unnecessary or incomplete surgeries are avoided. PROGNOSIS The prognosis for Eagle Syndrome is generally good, especially when appropriate treatment is provided. Most patients experience partial to full relief of symptoms after treatment — particularly after surgical intervention. SOURCES https://my.clevelandclinic.org/health/diseases/eagle-syndrome https://www.ncbi.nlm.nih.gov/books/NBK430789/ https://eso-stroke.org/blog-eagle-syndrom-a-rare-and-underdiagnosed-condition/ https://emedicine.medscape.com/article/1447247-overview?form=fpf

CONNECTIVE TISSUE DISORDERS Connective tissue disorders are a group of diseases that affect the parts of the body that connect, support, or surround other tissues and organs. These tissues include collagen and elastin, which provide strength and flexibility to skin, joints, blood vessels, and internal organs .Connective tissue is found throughout the body and includes: Tendons (connect muscles to bones) Ligaments (connect bones to each other) Cartilage Fat tissue Bone Skin Blood vessel walls TYPES They can be inherited (genetic) or acquired (autoimmune). Inherited (Genetic) Disorders These are caused by mutations in genes responsible for collagen or other connective tissue proteins: Marfan syndrome Ehlers-Danlos syndrome (EDS) & Hypermobility spectrum disorder (HSD) Osteogenesis imperfecta Stickler syndrome Autoimmune (Acquired) Disorders The body’s immune system mistakenly attacks its own connective tissue: Systemic lupus erythematosus (SLE) Rheumatoid arthritis (RA) Scleroderma Sjogren's syndrome Mixed connective tissue disease (MCTD) EDS & HSD Ehlers-Danlos syndrome (EDS) is a group of inherited disorders that affect the connective tissues, which provide support in skin, joints, blood vessels, and other organs and tissues. The defects in connective tissue result in symptoms that vary in severity and type depending on the specific form of EDS. Hypermobility Spectrum Disorder(HSD) is a connective tissue disorder that affects the joints, causing them to move beyond the normal range — also known as hypermobility — along with pain and other symptoms. What is Hypermobility Spectrum Disorder (HSD)? HSD refers to a group of conditions where people have joint hypermobility plus symptoms (like pain, instability, or fatigue), but don’t meet the full criteria for hypermobile Ehlers-Danlos syndrome (hEDS). This page will focus mainly on these two conditions, as they are the most closely linked to CCI & Co. TYPES OF EDS There are several types of EDS, with the most common being: Hypermobile EDS (hEDS): Characterized by joint hypermobility, frequent dislocations, and chronic pain. Classical EDS (cEDS): Features include highly elastic (stretchy) and fragile skin, as well as joint hypermobility. Vascular EDS (vEDS): The most severe type, affecting blood vessels and organs, making them prone to rupture. HEDS is the most common of all types, along with HSD. SYMPTOMS Joint Hypermobility: Unusually flexible joints that extend beyond the normal range of motion. Skin Elasticity: Soft, velvety skin that is highly elastic and fragile, prone to bruising and scarring. Chronic Pain: Due to joint and muscle issues. Frequent Dislocations and Subluxations: Partial dislocations of the joints. Easy Bruising: Blood vessels can be fragile, leading to frequent bruising. Slow Wound Healing: Scars that may stretch or become wide. Cardiovascular Issues: In some types, especially vEDS, there is a risk of blood vessel or organ rupture. Muscle Weakness: Generalized weakness and fatigue. Digestive Issues: Such as gastrointestinal reflux, constipation, or hernias. DIAGNOSIS Clinical Examination: Assessment of symptoms, including skin texture, joint mobility, and family history. Genetic Testing: To identify specific mutations associated with various types of EDS. There's no known gene for hEDS and HSD but reserch is ungoing Skin Biopsy: Examining a sample of skin tissue to study collagen structure and composition. TREATMENT While there is no cure for EDS & HSD, treatment focuses on managing symptoms and preventing complications: Physical Therapy: To strengthen muscles and stabilize joints. Pain Management: Medications, physical therapy, and sometimes surgery. Protective Measure: Using braces or supports to protect joints, and avoiding activities that put stress on the joints. Mobility aids: using aids to manage pain and mobility issues Wound Care: Special care for skin injuries to promote healing and minimize scarring. Regular Monitoring: Particularly for types that involve cardiovascular risks, such as vEDS, regular check-ups with a cardiologist may be necessary. Surgical Interventions: May be required for severe joint issues or vascular complications, though surgeries can be more complicated due to tissue fragility. PROGNOSIS The prognosis for EDS & HSD varies widely depending on the type and severity of the condition. Many individuals with EDS live a normal lifespan with proper management of symptoms. However, those with vascular EDS (vEDS) may face serious, life-threatening complications and require more intensive monitoring and care. Regular follow-up with healthcare providers and a multidisciplinary approach to care can help manage the condition effectively. For more info, please check out our resources here SOURCES https://www.mayoclinic.org/diseases-conditions/ehlers-danlos-syndrome/symptoms-causes/syc-20362125 https://www.ehlers-danlos.org/what-is-eds/ https://www.ehlers-danlos.org/what-is-eds/information-on-eds/hypermobile-eds-and-hypermobility-spectrum-disorders/ https://www.ncbi.nlm.nih.gov/books/NBK549814/ https://my.clevelandclinic.org/health/diseases/17813-ehlers-danlos-syndrome

GLOSSARY CONDITION

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SUSPECTED DIAGNOSIS? CCI & AAI WHERE TO START Knowing where to start when you suspect you, or a loved one, has CCI/AAI can be challenging due to the lack of knowledge of the condition in mainstream medicine. Many have been told by their doctors that nothing is wrong with their neck after a standard MRI or X-ray, although this is rarely enough to make a diagnosis. The first step you should take is finding a practitioner. You can find a list of well established CCI knowledgeable practitioners here . When you have chosen one, reach out to them to ask what imaging they prefer, this varies. Most practitioners offer this imaging as a service, or is able to refer you to a place that does. You can also find our list of upright MRI machines here . Once you have this info, you should consider what sort of consultation you'd like. Some practitioners offer scan reviews, some offer telehealth/remote consultations, and all offer in person consultation. While seeing a doctor in person is always recommended, it's not always accessible due to finances or health limitation. In that situation you might choose to get the relevant scans at the facility closest to you, and get a remote consult or simply a scan review. Keep in mind that most practitioners won't make an official diagnosis or recommendations without an in-person consult, however they can give you an idea of what might be going on, which can be helpful in letting you decide the next step. DIAGNOSED - WHAT'S NEXT Once you're diagnosed or has a suspected diagnosis it's time to decide what management route you want to go. There are both traditional and more alternative options available. You can read a short description of the treatment options here . If your case is severe, especially if there's signs of neurological/brainstem involvement, you might be recommended surgery, while in mild to moderate cases, conservative management is recommended.