Search Results

CRANIOCERVICAL INSTABILITY ATLANTOAXIAL INSTABILITY CCI and AAI are two forms of spinal instability, affecting the skull-C1 vertebrae (CCI) and C1-C2 vertebrae (AAI). They are both structural instabilities that can lead to pathological deformation of the brainstem and upper spinal cord. This page mainly focuses on CCI+AAI caused by lax ligaments failing to keep the craniocervical junction stable. SYMPTOM S Symptoms include Headache Neck pain Altered vision Diplopia Nystagmus Tinnitus Hearing loss Dysautonomia Postural orthostatic tachycardia (high heart rate upon standing) Orthostatic intolerance (Pre) syncope Dizziness Vertigo Urinary tract dysfunction, urgency, frequency GI dysfunction, constipation Weakness Spasticity Clumsiness Altered sensation, numbness (dysesthesia, paresthesias) Abnormal gait Paralysis Sleep apnea Sleep disorders Fatigue Choking, dysphagia Trouble breathing Slurred speech, dysarthria DIAGNOSIS CCI and AAI is diagnosed based on three things - 1) imaging, 2) symptoms and 3) positive neurological findings. A neurosurgeon will look at a patient's imaging and take certain measurements, such as the clivo-axial angle, which if abnormal suggests CCI or AAI. However these measurements cannot stand alone. A neurosurgeon will also go through the patients symptoms, past medical history and do a neurological exam. The symptoms and neurological findings are important to make a final diagnosis while also assessing the severity of the patient's condition. For diagnosing CCI, an upright flexion extension MR(uMRI)I is the gold standard. A digital motion xray(DMX) can be used to further confirm the diagnosis. An ordinary supine brain MRI should also be done to rule out or diagnose comorbidities such as intracranial pressure issues, chiari malformation etc. For diagnosing AAI, a rotational CT scan is considered the gold standard. An upright MRI with rotation can also be used to make a diagnosis. MEASUREMENTS CAUSES There's a number of different conditions that can cause CCI and AAI, some which are widely recognized, while others are still relatively unknown by many medical professionals. The common ones include Autoimmune connective tissue disorders such as rheumatoid arthritis and lupus Genetic conditions such as downs syndrome and osteogenesis imperfecta Conditions causing tumors in the craniocervical junction, such as Neurofibromatosis Other acquired bone conditions such as Paget's disease Severe trauma to the craniocervical junction and head Less commonly known is Hereditary connective tissue disorders, also known as hypermobility syndromes, such as Ehlers-danlos syndrome and the hypermobility spectrum disorders Trauma to the craniocervical junction(CCJ) , that would usually be considered less severe, such as whiplash Grisel syndrome, subluxation of the atlantoaxial joint (C1/2) due to inflammatory ligamentous laxity following an infectious process in the head and neck In these two conditions the instability do not stem from a malformation or degeneration of the bone, but from the ligaments in the CCJ being too lax, to properly stabilize the vertebra. In conditions such as ehlers-danlos syndrome, the laxity comes from a genetic defect of the connective tissue, while in cases of trauma, the laxity stems from the ligaments being stretched during the traumatic incident. MANAGEMENT Unfortunately there's no cure for CCI and AAI, but there is different ways to manage it. The main ones being physical therapy, symptom management, bracing, and as a last resort a spinal fusion surgery can be done. In mild to moderate cases, physical therapy, bracing and treating symptoms can be enough to manage the patient's condition. The physical therapy and symptom management will vary from patient to patient, however isometric neck exercises are one of the most common recommendations. Symptom management might include treating conditions caused or exacerbated by CCI and AAI, such as dysautonomia, medications to manage pain, nausea etc In severe cases this won't be enough to manage the condition, and spinal fusion will be recommended. SURGERY ... Sources https://link.springer.com/article/10.1007/s10143-018-01070-4 https://www.researchgate.net/publication/316895469_Occipito-atlanto-axial_Hypermobility_Clinical_Features_and_Dynamic_Analysis_of_Cranial_Settling_and_Posterior_Gliding_of_Occipital_Condyle_Part_2_Findings_in_Patients_with_Post-traumatic_Condition https://www.researchgate.net/publication/5773233_Syndrome_of_occipitoatlantoaxial_hypermobility_cranial_settling_and_Chiari_malformation_Type_I_in_patients_with_hereditary_disorders_of_connective_tissue https://www.ncbi.nlm.nih.gov/pubmed/28961036

TETHERED CORD SYNDROME Tethered cord syndrome is a neurological disorder caused by a tissue attachment that limits the movement of the spinal cord within the spinal column. This tethering causes the spinal cord to be stretched and damaged as the body grows and moves. SYMPTOM S - Back Pain : Often in the lower back, increasing with activity. - Leg Weakness or Numbness : Affects walking and coordination. - Foot Abnormalities : Such as high arches or curled toes. - Bladder and Bowel Problems : Incontinence or difficulty with urination and bowel movements. - Scoliosis : Abnormal curvature of the spine, which can be associated with tethered cord. - Skin Abnormalities : Such as a fatty lump, dimple, or patch of hair on the lower back. - Muscle contractions : such as clonus CAUSES Congenital (present at birth): - Spina Bifida : A birth defect where the spine and spinal cord don't form properly, often associated with tethered cord. - Lipoma : Fatty tissue that can attach to the spinal cord. - Thickened Filum Terminale : The end part of the spinal cord may be thicker than normal and cause tethering. - Diastematomyelia : A condition where the spinal cord is split into two parts, often causing tethering. Acquired: - Surgery : Scar tissue from previous spinal surgeries can cause tethering. - Injury : Trauma to the spine can result in scar tissue that leads to tethering. DIAGNOSIS - Clinical Examination : Doctors look for physical signs and symptoms. - Imaging Studies : MRI is the most common imaging test used to diagnose tethered cord syndrome, providing detailed images of the spinal cord and surrounding tissues. - Urodynamic Tests : To assess bladder function if urinary symptoms are present. TREATMENT Conservative Management: - Monitoring and physical therapy: for mild cases without severe symptoms. Surgical Intervention: - Detethering Surgery: The primary treatment, which involves surgically releasing the spinal cord from the attached tissue. This aims to restore the normal movement of the spinal cord and prevent further damage. - Postoperative Care : Includes physical therapy and regular follow-up to monitor recovery and ensure that the spinal cord remains free. POTENTIAL COMPLICATIONS - Neurological Damage : If untreated, the ongoing tension can cause permanent damage to the spinal cord. - Re-tethering : In some cases, the spinal cord may tether again after surgery, requiring further intervention. - Infection and Bleeding : Risks associated with any surgical procedure. PROGNOSIS Early diagnosis and treatment are crucial for preventing long-term complications. Many patients experience significant relief of symptoms and improved quality of life after surgery. Regular follow-up is important to monitor for potential recurrence of tethering and manage any ongoing symptoms. OCCULT TETHERED CORD Occult tethered cord syndrome (OTCS) is a condition where the spinal cord is abnormally stretched or anchored, but without the obvious, visible signs seen in classic tethered cord syndrome. The term "occult" means hidden, indicating that the tethering is not easily detected through standard imaging tests or physical examination. OTCS seems to be more prevalent in patients with CCI or ehlers-danlos syndrome, however more research is needed. CAUSES - Congenital Abnormalities : Even though the condition is hidden, it may still be caused by subtle congenital anomalies. - Trauma or Surgery : Scar tissue from previous spinal surgeries or injuries can cause occult tethering. - Degenerative Changes : Age-related changes in the spine that may not be immediately apparent. SYMPTOMS The symptoms of OTCS are similar to those of classic tethered cord syndrome but may be more subtle and harder to diagnose. DIAGNOSIS It is often a clinical diagnosis based on symptoms and physical findings, however in some cases prone lumbar MRI scans are used to identify signs of an occult tether. This is still a fairly new concept and not widely used. SOURCES Tethered Spinal Cord Syndrome – Causes, Diagnosis and Treatments Tethered Spinal Cord Syndrome | National Institute of Neurological Disorders and Stroke Tethered Cord Syndrome - Symptoms, Causes, Treatment | NORD Tethered cord syndrome | Radiology Reference Article | Radiopaedia.org Occult tethered cord syndrome: a rare, treatable condition https://thejns.org/spine/view/journals/j-neurosurg-spine/40/6/article-p758.xml?tab_body=fulltext Full article: Effect of untethering on occult tethered cord syndrome: a systematic review Diseased Filum Terminale as a Cause of Tethered Cord Syndrome in Ehlers-Danlos Syndrome: Histopathology, Biomechanics, Clinical Presentation, and Outcome of Filum Excision - ScienceDirect

OTHER CONDITIONS COMING SOON

Toppen af siden CCI/AAI Tethered cord IJVS + IH RESEARCH ARTICLES Neurological and spinal manifestations of the Ehlers–Danlos syndromes CCI/AAI Cranio-cervical Instability in Patients with Hypermobility Connective Disorders Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization Occipito-cervical fusion in patients with occipito-atlanto-axial joints instability: A preliminary report of outcome and examination of influence factors for outcome Occipito-atlanto-axial Hypermobility : Clinical Features and Dynamic Analysis of Cranial Settling and Posterior Gliding of Occipital Condyle. Part 2 : Findings in Patients with Post-traumatic Condition Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue CSF Craniocervical Instability Colloquium 2013 Occipito-atlanto-axial Hypermobility : Clinical Features and Dynamic Analysis of Cranial Settling and Posterior Gliding of Occipital Condyle. Part 1 : Findings in Patients with Hereditary Disorders of Connective Tissue and Ehlers-Danlos Syndrome Surgical Outcomes and Complications after Occipito-Cervical Fusion Using the Screw-Rod System in Craniocervical Instability Diagnosis of Atlantoaxial Instability Requires Clinical Suspicion to Drive the Radiological Investigation Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review Tethered cord Tethered Cord Syndrome : Preliminary Report of Clinical Features and Morphometric Analysis on Association of Chiari Malformation Type The Tethered Cord Syndrome and Its Occult Form Tethered cord syndrome in adults Variation in Outcome in Tethered Cord Syndrome Detethering of a congenital tethered cord in adult patients: An outcome analysis IJVS + IH Manifestations of Pediatric Intracranial Hypertension From the Intracranial Hypertension Registry MRI features of idiopathic intracranial hypertension Feasibility and usefulness of ultrasonography in idiopathic intracranial hypertension or secondary intracranial hypertension Headache in Idiopathic Intracranial Hypertension: Findings From the Idiopathic Intracranial Hypertension Treatment Trial Idiopathic Intracranial Hypertension: A Venous Disease? Understanding jugular venous outflow disturbance Internal jugular vein stenosis - the neurosurgery dictionary Styloidogenic Jugular Venous Compression Syndrome: Diagnosis and Treatment: Case Report Cerebral venous etiology of intracranial hypertension and differentiation from idiopathic intracranial hypertension Intracranial hypertension and cranial sinus stenosis

HEALTHCARE ABROAD HEALTHCARE ABROAD Before you go Research your destination’s healthcare system Find out if it’s public, private, or mixed. Learn how emergency care works — what’s the local emergency number? (e.g., 112 in the EU, 999 in the UK, 911 in the US, 000 in Australia.) Check your health insurance coverage Contact your provider to confirm if international care is included. Ask about medical evacuation (air transport) — important for serious conditions. If not covered, consider travel health insurance or expat insurance. Gather your medical information Bring a english language summary of your medical history, including: Diagnoses and allergies Current medications (generic names) Emergency contacts and physician info A symptom list is not a necessity, but can be very helpful Keep both digital and paper copies. Get necessary vaccinations or documentation Check if you need any vaccinations, new or updated. Some countries require proof of vaccinations (e.g., COVID-19). Know where to go for care Save the names and addresses of nearby hospitals, clinics, and pharmacies. Check if there’s an English-speaking medical center or embassy-recommended doctor. Check if the specialist you're seeing can assist you in case of emergency or sudden worsening of symptoms. While You’re Abroad Carry your insurance card and medical ID If you're in Europe you need to bring your European health insurance card Consider wearing a medical alert bracelet for serious allergies or conditions. For ongoing conditions Bring extra medication (with prescriptions). Keep meds in original labeled containers for customs. Know local equivalents if refills are needed. Language barriers Learn a few key health phrases in the local language (e.g., “I need a doctor”). Use a translation app for symptoms and medications. Many countries have expat medical interpreters. If you need urgent care Contact the doctor you're there to see, if theres sudden worsening of your symptoms. Go to an emergency department (ER/A&E) for serious illness or injury. For less urgent issues, private clinics often have shorter wait times. Contact your insurer as soon as possible — they can coordinate and guarantee payment. GENERAL TRAVEL TIPS Pack Strategically Bring medical documents: Include prescriptions, doctor’s notes, and emergency information. Pack extra medication & supplies: Don’t rely on local availability—bring more than you expect to need. Mobility aids: If using a wheelchair, cane, or other aids, check airline and transport policies. Bring any tools or spare parts in case of repairs. Bring adapters to charge equipment Book Accessible Accommodations Call, don’t just book online: Accessibility standards vary; a “wheelchair accessible” room may still have a step at the door. Request specifics: Ask about door widths, grab bars, roll-in showers, elevator access, if they provide aids such as a showerchair etc Consider location: Stay near accessible public transport or the hospital you're going to, to reduce commuting stress. Transportation Considerations Accessible public transit: Check if buses, trains, and metro systems offer ramps, lifts, or priority seating. Wheelchair taxis or car rentals: Reserve these in advance if needed. Parking permits: If you have a disability parking placard, check if it’s recognized in the country you're visiting. Prepare for Emergencies Emergency contact list: Include both local (embassy, emergency services) and home contacts. Insurance for pre existing conditions: Get travel insurance that covers your specific medical and accessibility needs. Plan for lost/damaged equipment: Know where to get repairs or replacements if needed. Bring a toolkit for your wheelchair/walker. Get an English language medical alert band/tag, or have your companion carry a document with vital medical infomation, in case of emergency PRODUCT RECOMMENDATIONS Portable fan - to help with temperature dysregulation A good neck pillow - to provide support and be more comfortable during travel Neck brace (if cleared by doctor) - to protect the neck and minimize symptoms Mask - to protect yourself from virus, infection and mast cell triggers Foot hammock - for comfort and to minimize blood pooling Noise cancelling headphones and sunglasses - if you’re sensitive to stimuli A medical alert bracelet - to provide important information in case of an emergency Compression socks - to minimize bloodpooling and help dysautonomia symptoms

RESOURCES GENERAL MEchanical Basis BRAIN FOUNDATION AU CCI MEpedia DR.GILETE - WHAT IS CCI IIH/PC INTRACRANIAL HYPERTENSION RESEARCH FOUNDATION IIH UK CSF LEAK CSF LEAK ASSOCIATION SPINAL CSF LEAK FOUNDATION CHIARI CHIARI BRIDGES CONQUER CHIARI CHIARI AND SYRINGOMYELIA FOUNDATION EDS & HSD THE EDS/HSD TOOLKIT THE EHLERS-DANLOS SOCIETY MIND BODY EDS EHLERS DANLOS UK HYPERMOBILITY SYNDROMES ASSOCIATION

AIRTRAVEL Flying can be a grueling process when you're sick and disabled. We've collected some tips and tricks to making the process easier Before You Fly Contact the airline in advance When booking (or at least 48 hours before travel), tell them about your accessibility needs — for example: Mobility assistance (wheelchair, electric cart, or escort) Priority boarding Seating preferences (need to recline seat, aisle seat for easy transfer) Assistance with carrying luggage or medical devices Most airlines have a “special assistance” or “accessibility” section on their website or a dedicated phone line. For electric wheelchair users. Inform the airline about the battery and dimensions of your chair ahead of time. You might need a certificate for the battery which your wheelchair manufacturer can provide Request airport assistance Many airports offer meet-and-assist services from check-in to the gate — and even through customs on arrival. They can provide wheelchairs, escorts, etc Prepare your medical or mobility equipment Label your wheelchair or other medical devices clearly with your name and contact info. Protect your wheelchair. Wrap/protect any breakable and scratchable parts, take off any loose parts, like the seat cushion, and bring it with you into the cabin. Bring a doctor’s note or documentation for any medications, implants, or devices. Check with your pharmacy beforehand, some medications require a "medication passport" Keep medication in their original container. Pack essential medications in your carry-on, not checked baggage. Pack extra medications in case you need to extend your stay Check if the airline allows you to check in an extra bag of medical equipment free of charge. Be prepared You might need a fit to fly note. This is especially important if you appear sick, as it might cause airline staff to refuse you boarding without a fit to fly note. Know your rights In the U.S., the Air Carrier Access Act (ACAA) protects travelers with disabilities. In the E.U., Regulation (EC) No 1107/2006 gives similar rights. These ensure free assistance, no discrimination, and support for medical devices. At the Airport Arrive early Give yourself extra time for check-in, security, and boarding — 2–3 hours before departure is ideal. Locate where you need to meet up with airport assistance, if you booked this. Often airports has a meeting point for this Ask for accessible security screening At TSA or security checkpoints, you can: Request a private screening. Stay seated if standing is difficult. Bring a companion or personal assistant through screening. You can notify TSA Cares in advance (U.S. program): call 72 hours before travel for help. Use accessible facilities Look for accessible restrooms, quiet rooms, and charging points for mobility devices. Most airports list these on their websites or maps. During Boarding and the Flight Use pre-boarding You have the right to board early to get settled and store mobility aids safely. Ask for help reaching your seat or transferring from an aisle chair. Communicate your needs Let flight attendants know if you need: Help stowing bags Special meal arrangements Assistance moving to the restroom Before landing, remind flight attendants that you need your equipment brought to the gate. Medical needs You can carry medical devices (CPAP, insulin, oxygen concentrator) — just notify the airline ahead of time. If you need to keep medication cold, bring a small insulated bag or ask for ice from the crew. On Arrival Request assistance at your destination An airport representative can meet you at the gate and help with: Immigration and baggage claim Transfers to connecting flights or ground transportation Double-check mobility equipment Make sure your wheelchair or devices are returned intact. If anything is damaged, report it immediately to the airline before leaving the baggage area. Bonus Tips Wear a Hidden Disabilities Sunflower lanyard if you have an invisible condition — many airports recognize this and can offer discreet support. Download airport accessibility maps or apps. Keep all medical documentation and assistance confirmations in one easy-to-access folder. Don’t hesitate to advocate for yourself — you have the right to dignified, accessible travel. Wear a mask and bring disinfectant wipes to minimize risk of catching something

WEBINARS AND LECTURES Five-Year Follow Up of Craniocervical Fusions - Dr. Fraser Henderson SUBJECTS: CCI, AAI, FUSION, FOLLOW UP, INSTABILITY, TREATMENT INFO ON VIDEO All Videos Sign in All Videos Play Video Share Whole Channel This Video Facebook Twitter Pinterest Tumblr Copy Link Link Copied Search videos Search video... All Categories All Categories Entertainment Nonprofits & Activism Now Playing SPINAL CSF LEAKS: UPDATES IN DIAGNOSIS AND MANAGEMENT. HOW CAN YOUR NEURORADIOLOGIST HELP YOU? 02:05:10 Play Video Now Playing "Cerebrospinal Fluid Pathologies" - Petra M. Klinge, MD, PhD 19:05 Play Video (2019 Think Tank) Cervical Medullary Syndrome & Cranial Instability - Dr. Fraser Henderson SUBJECTS: CCI, AAI, CONSENSUS, CRITERIA, RADIOLOGICAL FINDINGS, TREATMENT INFO ON VIDEO Craniocervical Instability Lecture - Dr.Vicenç Gilete SUBJECTS: CCI, AAI, SYMPTOMS, CRITERIA, RADIOLOGICAL FINDINGS, TREATMENT INFO ON VIDEO Chiari I Malformation, EDS, and Craniocervical Instability - Dr. Paolo Bolognese SUBJECT INFO ON VIDEO Imaging of Patient with Occult Tethered Cord Syndrome - Amanda Baker SUBJECT INFO ON VIDEO The Occult Tethered Cord Syndrome -Dr. Petra M. Klinge SUBJECT INFO ON VIDEO Tethered Cord Syndrome and Ehlers Danlos Syndrome - Dr. Sunil Patel SUBJECT INFO ON VIDEO Tethered Cord Syndrome - Dr. Holly Gilmer SUBJECT INFO ON VIDEO Intracranial Venous Stenting for Idiopathic Intracranial Hypertension - Dr. Kenneth C. Liu SUBJECT INFO ON VIDEO Idiopathic Intracranial Hypertension (IIH): Advances in Treatment - Dr. Imran Chaudry SUBJECT INFO ON VIDEO

JUGULAR VEIN STENOSIS Jugular vein compression is when one or both internal jugular veins (IJVs) — the major veins that drain blood from your brain — become partially or fully blocked due to external pressure or narrowing. This can lead to reduced blood flow out of the brain, causing symptoms related to intracranial pressure, drainage issues, or brainstem congestion. ANATOMY AND FUNCTION You have two internal jugular veins — one on each side of your neck — and they are the main drainage pathways for venous (used) blood from the brain back to the heart. If one or both veins are compressed: Blood may back up in the head Brain pressure may increase Collateral veins may form but may not drain efficiently SYMPTOM S The symptoms of jugular vein compression are typically related to impaired venous drainage from the brain, which can cause increased intracranial pressure and reduced blood flow. Symptoms may include: Headache : A common symptom due to increased intracranial pressure. Headaches may be persistent, throbbing, and worse in the morning or when changing position (such as lying down) Tinnitus: Ringing or pulsatile sounds in the ears, often caused by increased pressure in the venous system or impaired venous drainage from the brain Visual Impairment : Blurred vision, transient visual loss, or even blind spots may occur due to pressure on the optic nerves or from papilledema. Neurological Symptoms: Symptoms such as dizziness, nausea, or vomiting due to increased intracranial pressure. In severe cases, neurological deficits like motor weakness, seizures, or cognitive changes may develop. Swelling of the Neck : In some cases, neck or facial swelling may occur due to impaired venous drainage. Cognitive Impairment : Patients with jugular vein stenosis may experience difficulty concentrating, memory problems, or a general feeling of "brain fog." Seizures: In severe cases of venous congestion or increased intracranial pressure, seizures may occur due to reduced cerebral blood flow. CAUSES Bone compression : The C1 (atlas) or styloid process can press on the vein Vascular compression: Nearby arteries or abnormal veins may push against the jugular Scar tissue or fibrosis: From past surgeries or infections Tumors or masses: Can press on the vein externally Neck alignment/instability : May narrow the vein when the head is turned Often occurs in people with: Ehlers-Danlos Syndrome (EDS) Craniocervical Instability (CCI) and Atlantoaxial Instability(AAI) Eagle Syndrome (elongated styloid bone compresses jugular vein) DIAGNOSIS Diagnosing transverse sinus stenosis typically involves a combination of clinical examination, imaging, and sometimes diagnostic procedures: Imaging : Magnetic Resonance Imaging (MRI) with Magnetic Resonance Venography (MRV): This is the gold standard for diagnosing jugular vein stenosis. MRV allows detailed imaging of the venous structures and can identify areas of narrowing or occlusion in the jugular vein. CT Venography (CTV): This test can also be used to visualize jugular veins, particularly if MRV is not available or contraindicated. Conventional Venography: This invasive test, which involves injecting contrast into the venous system and taking X-ray images, is rarely used today but may be helpful in certain situations. Lumbar Puncture (Spinal Tap) : A lumbar puncture may be performed to measure opening pressure in the cerebrospinal fluid (CSF). Elevated opening pressure can indicate increased intracranial pressure, which might be secondary to impaired venous drainage from the brain. Fundoscopic Eye Exam: The ophthalmologist may observe papilledema (swelling of the optic disc), which is indicative of increased intracranial pressure and can be seen in patients with transverse sinus stenosis. Ultrasound : In certain cases, Doppler ultrasound of the neck veins may help assess venous flow and detect stenosis or clot formation. TREATMENT The treatment for jugular vein stenosis depends on the severity of the condition, the underlying cause, and the presence of complications such as increased intracranial pressure or venous thrombosis. Common treatments include: Anticoagulation: If the stenosis is due to thrombosis, anticoagulant therapy (e.g., heparin or warfarin) is typically used to prevent further clotting and manage the risk of embolism. Management of Idiopathic Intracranial Hypertension (IIH): If transverse sinus stenosis is associated with IIH (pseudotumor cerebri), treatment may include: Acetazolamide (a carbonic anhydrase inhibitor) to reduce cerebrospinal fluid (CSF) production and lower intracranial pressure. Weight loss for patients who are overweight or obese, as it has been shown to reduce intracranial pressure in IIH. Diuretics like furosemide to reduce fluid buildup. In severe cases, optic nerve sheath fenestration (a surgical procedure to relieve pressure on the optic nerve) or a lumbar peritoneal shunt may be needed. Surgical Intervention: Surgical interventions may be required to address external compression, e.g., styloidectomy, C1 shave) if anatomical structures are compressing the vein or to insert a stent in the jugular vein. Surgical fusion for CCI and AAI may sometimes help, if the compression is due to instability PROGNOSIS The prognosis for jugular vein compression is generally good if the cause is clearly identified and treated appropriately, especially in cases of mechanical compression (e.g., styloid or C1). However, outcomes can vary, especially if there are comorbid conditions like CCI and AAI, EDS, or IIH SOURCES https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-019-1344-0 https://pmc.ncbi.nlm.nih.gov/articles/PMC6726807/ https://www.sciencedirect.com/science/article/pii/S0967586825002760 https://pmc.ncbi.nlm.nih.gov/articles/PMC6489808/

TRANSVERSE SINUS STENOSIS Transverse sinus stenosis refers to the narrowing (stenosis) of the transverse sinus, a large venous channel in the brain responsible for draining blood from the brain toward the internal jugular vein. The transverse sinuses are part of the dural venous sinuses, which are specialized blood vessels within the dura mater (the outermost membrane surrounding the brain) that collect venous blood from the brain and direct it toward the jugular veins. When there is stenosis (narrowing) of the transverse sinus, it can obstruct the normal flow of blood from the brain, potentially leading to increased intracranial pressure and other neurological symptoms. This condition is also referred to as transverse sinus thrombosis if the stenosis is caused by a clot. ANATOMY The transverse sinus is located on each side of the brain and runs along the back of the skull. It collects venous blood from the brain's internal structures, such as the cerebellum, brainstem, and cerebral hemispheres, before draining it into the internal jugular vein. The sigmoid sinus is a continuation of the transverse sinus, leading directly into the internal jugular vein, and from there, blood is returned to the heart. SYMPTOM S The symptoms of transverse sinus stenosis are typically related to impaired venous drainage from the brain, which can cause increased intracranial pressure and reduced blood flow. Symptoms may include: Headache : A common symptom due to increased intracranial pressure. Headaches may be persistent, throbbing, and worse in the morning or when changing position (such as lying down) Tinnitus: Ringing or pulsatile sounds in the ears, often caused by increased pressure in the venous system or impaired venous drainage from the brain Visual Impairment : Blurred vision, transient visual loss, or even blind spots may occur due to pressure on the optic nerves or from papilledema. Neurological Symptoms: Symptoms such as dizziness, nausea, or vomiting due to increased intracranial pressure. In severe cases, neurological deficits like motor weakness, seizures, or cognitive changes may develop. Swelling of the Neck : In some cases, neck or facial swelling may occur due to impaired venous drainage. Cognitive Impairment : Patients with transverse sinus stenosis may experience difficulty concentrating, memory problems, or a general feeling of "brain fog." Seizures: In severe cases of venous congestion or increased intracranial pressure, seizures may occur due to reduced cerebral blood flow. CAUSES Transverse sinus stenosis can result from various conditions, including: Venous Thrombosis Transverse sinus thrombosis is a condition in which a blood clot forms within the transverse sinus, leading to stenosis or complete blockage. This can occur due to various factors, such as: Hypercoagulable states (conditions that increase blood clotting, like certain genetic disorders, cancer, or pregnancy). Infections (e.g., sinusitis or otitis media). Trauma or surgery in the head or neck region, particularly if there is damage to the venous system. Oral contraceptives, pregnancy, or hormone replacement therapy (all of which can increase the risk of clotting). External Compression: Tumors, cysts, or enlarged lymph nodes around the neck and base of the skull can compress the transverse sinus, leading to narrowing and reduced venous outflow. Atherosclerosis: Although less common in veins, atherosclerotic plaques can form in the venous system and contribute to stenosis of the transverse sinus. Idiopathic Intracranial Hypertension (IIH): Also known as pseudotumor cerebri, IIH is a condition characterized by elevated intracranial pressure without an identifiable cause. The high pressure can lead to narrowing or stenosis of the venous sinuses, including the transverse sinus. Congenital Anomalies: Some individuals may have an inherent narrowing of the transverse sinus due to structural or developmental abnormalities in the venous system. Fibromuscular Dysplasia: A rare condition that causes abnormal growth in the walls of blood vessels, including veins, which can lead to stenosis in the transverse sinus. DIAGNOSIS Diagnosing transverse sinus stenosis typically involves a combination of clinical examination, imaging, and sometimes diagnostic procedures: Imaging : Magnetic Resonance Imaging (MRI) with Magnetic Resonance Venography (MRV): This is the gold standard for diagnosing transverse sinus stenosis. MRV allows detailed imaging of the venous structures and can identify areas of narrowing or occlusion in the transverse sinus. CT Venography (CTV): This test can also be used to visualize venous sinuses, particularly if MRV is not available or contraindicated. Conventional Venography: This invasive test, which involves injecting contrast into the venous system and taking X-ray images, is rarely used today but may be helpful in certain situations. Lumbar Puncture (Spinal Tap) : A lumbar puncture may be performed to measure opening pressure in the cerebrospinal fluid (CSF). Elevated opening pressure can indicate increased intracranial pressure, which might be secondary to impaired venous drainage from the brain. Fundoscopic Eye Exam: The ophthalmologist may observe papilledema (swelling of the optic disc), which is indicative of increased intracranial pressure and can be seen in patients with transverse sinus stenosis. Ultrasound : In certain cases, Doppler ultrasound of the neck veins may help assess venous flow and detect stenosis or clot formation. TREATMENT The treatment for transverse sinus stenosis depends on the severity of the condition, the underlying cause, and the presence of complications such as increased intracranial pressure or venous thrombosis. Common treatments include: Anticoagulation: If the stenosis is due to venous thrombosis, anticoagulant therapy (e.g., heparin or warfarin) is typically used to prevent further clotting and manage the risk of embolism. Management of Idiopathic Intracranial Hypertension (IIH): If transverse sinus stenosis is associated with IIH (pseudotumor cerebri), treatment may include: Acetazolamide (a carbonic anhydrase inhibitor) to reduce cerebrospinal fluid (CSF) production and lower intracranial pressure. Weight loss for patients who are overweight or obese, as it has been shown to reduce intracranial pressure in IIH. Diuretics like furosemide to reduce fluid buildup. In severe cases, optic nerve sheath fenestration (a surgical procedure to relieve pressure on the optic nerve) or a lumbar peritoneal shunt may be needed. Endovascular Procedures: In cases of severe stenosis or thrombosis, endovascular procedures like angioplasty (dilating the narrowed sinus) or the placement of a stent to keep the vein open may be considered. Surgical Intervention: Surgical interventions may be required to address external compression, such as tumor removal, or to insert a stent in the stenosed transverse sinus STENTING Transverse sinus stenting is a medical procedure used to treat venous sinus stenosis — a narrowing of the transverse sinus. It’s most commonly done in patients with Idiopathic Intracranial Hypertension (IIH)/Pseudotumor cerebri when high pressure in the brain is linked to this narrowing. It’s usually considered when: A person has Idiopathic Intracranial Hypertension (IIH). There is evidence of transverse sinus stenosis on imaging (MRI/MRV, CT venogram). Other treatments (like medication or weight loss) haven’t worked. A pressure gradient (> 4–10 mmHg) is found across the stenosis during venous manometry (a catheter-based pressure test). How the Procedure Works Venous access is typically gained through the femoral vein (in the groin). A catheter is threaded up through the venous system into the transverse sinus. A stent (a mesh-like tube) is placed in the narrowed area to hold it open. This improves blood outflow and can reduce intracranial pressure. PROGNOSIS The prognosis for transverse sinus stenosis depends on the underlying cause and the promptness of treatment. If the condition is diagnosed early and treated effectively, especially in cases of venous thrombosis or IIH, the outcome can be favorable. However, untreated stenosis may lead to chronic increased intracranial pressure, permanent vision loss (due to papilledema), and in severe cases, stroke or cognitive decline. SOURCES https://radiopaedia.org/articles/transverse-sinus-stenosis https://www.sciencedirect.com/science/article/pii/S2772687824001053 https://pubmed.ncbi.nlm.nih.gov/38983575/ https://pmc.ncbi.nlm.nih.gov/articles/PMC11410061/ https://pubmed.ncbi.nlm.nih.gov/21799038/