# Diagnosis and Management of Upper Cervical Instability: A brief primer for primary care clinicians **DISLAIMER: This is not medical advice. This is for informational purposes only. If you think you might have UCI, please discuss this information with your doctor.** A PDF version of this guide can be downloaded [HERE](https://drive.google.com/file/d/1rt5DTc3M3khkELimwAA1hz4pF2CCOKEx/view?usp=drive_link) --- Upper Cervical Instability (UCI) is a frequently missed diagnosis that can cause debilitating neurological symptoms. This is a brief guide designed to walk primary care physicians through the diagnosis and management of this underserved disorder.  This guide is not comprehensive and if you have a local physical therapist or physician who is knowledgeable about UCI I recommend referring your patient out to those with clinical experience in this condition. However, clinicians familiar with UCI are limited and not always accessible. The purpose of this guide is to improve patient access to UCI diagnosis and treatment.  # About the author My name is Dr. Katie Brown. I am a family physician with expertise in the management of Myalgic Encephalomyelitis / Chronic Fatigue Syndrome (ME/CFS). I have been mentored by Peter Rowe, MD and Wendy Wagner, PT who have expertise in UCI and who have published papers on the subject. Wendy was a co-author on the expert consensus paper which is heavily referenced here. While I hold no claim to be an expert in UCI, I am not aware of any guide for primary care doctors that contains this basic information and so I offer up this information as an imperfect educational resource to help patients with UCI access treatment. # Sources  Presentation and Physical Therapy Management of Upper Cervical Instability in Patients with Symptomatic Generalized Joint Hypermobility: International Expert Consensus Recommendations by Russek at al. Craniocervical Instability in Patients with Ehlers-Danlos Syndromes: Outcomes Analysis Following Occipito-Cervical Fusion by Henderson et al. Chapter 12 of the Clinical Care Guide to Managing ME/CFS, Long COVID, & IACCs by the Bateman Horne Center.  # Presentation—When to suspect UCI Upper Cervical Instability (UCI) is most commonly seen in people with generalized joint hypermobility but may also be caused by cervical trauma in people without hypermobility. Symptoms of UCI are published in table 2 of the Expert Consensus Paper by Russek et. al. It is transcribed in a modified form here.  - A sensation that the head is too heavy or "bobble-headed," requiring support or bracing to reduce symptoms - Fear or apprehension about moving the neck or riding in a car - A lump in the throat, trouble swallowing, choking, or voice changes - Consistent clicking or clunking of the neck with movement - Ringing in the ears or dizziness - Headaches at the base of the skull - Shoulder pain in areas where a coat hanger would rest - Neck tension and muscle spasms - Brain fog - Worsening symptoms or lack of improvement after neck massage or other neck pain treatments - Snoring or sleep apnea - Seizure-like activity without seizures detected on EEG; often diagnosed as "non-epileptic seizures" or "pseudo-seizures" - Sudden collapse (“drop attacks”) unrelated to POTS or low blood pressure* - Symptoms of autonomic nervous system dysfunction that do not respond to treatment, such as persistent anxiety, irritable bowel syndrome, heat intolerance, or lightheadedness - A feeling of instability, like being on a boat - Poor coordination - A pulling sensation in the face, head, teeth, or tongue due to muscle contractions rather than pain* (I have not observed this yet, so if anyone has experienced it and can describe it, I would love to hear from you.) - Tingling or numbness in the face - Vision changes, double vision, or difficulty with coordinated eye movements (convergence) - Involuntary muscle contractions leading to abnormal movements or postures - Numbness, tingling, or pain in the limbs that fluctuates in location or quality - Sudden changes in alertness, episodic unresponsiveness, or episodic amnesia # Physical Exam—Objective evidence of UCI - Loss of cervical lordosis and tension of the muscles in the neck and upper back are common - Cranial nerve deficits including amblyopia, anisocoria, unilateral facial numbness, or an absent gag reflex - Positive Hoffman reflex - Hyperreflexia  - Clonus, hypertonia, dystonia, myoclonic jerking - Horse, weak or gravely voice which may worsen over the course of the exam as the patient becomes more tired - Ataxia - Balance dysfunction (positive Romberg sign or inabiulity to perform a tendem gait) - Dysdiadochokinesia # Comorbidities—Commonly associated with UCI - Hypermobile joints, recurrent subluxations, elevated Beigton score - Orthostatic intolerance, including POTS which can be diagnosed with a simple 10 minute stand test - Tethered cord—may present with lower extremity signs/symptoms and can be exacerbated by wearing a cervical collar # Conservative Management **POSTURE** Keep head and neck in a neutral position as much as possible. Avoid looking down at your phone or other activities that put your neck in flexion. for extended periods of time. Similarly, avoid extension and rotation as much as possible.  Sit with the head supported such as in a recliner, gaming chair, or high backed desk chair.  Keep the head balanced on the neck. Keep excellent posture with the whole spine in alignment.  Avoid dependent head positioning (positions in which the head would fall off if it wasn't attached) Avoid jarring head movements. This includes trampolines, roller coasters, high velocity chiropractic manipulation, etc. **IMMOBILIZATION** Consider the use of a hard to soft cervical collar. Using a collar to immobilize the joints can be useful to reduce neuro-inflammation, avoid flares during high risk activities (such as riding in a car where the vibrations and unexpected acceleration can cause an exacerbation of symptoms), act as a prompt to keep the neck in neutral, and help rule in or rule out UCI depending upon patient response to neck immobilization. An [Aspen Vista cervical collar](https://www.amazon.com/Aspen-Cervical-Provides-Recovery-Adjustable/dp/B00E0MGSX2) can be purchased off Amazon and fits most people. Some patients feel worse in the collar either due to uncomfortable pressure to the cervical spine or a poor fit causes worsening of UCI symptoms. Pressure on the jaw can also exacerbate TMJ. Consider fitting by an orthotist if necessary.  A soft collar, such as by [Velpeau](https://www.amazon.com/Velpeau-Brace-Foam-Cervical-Collar/dp/B07FVW4DY7?ref_=ast_sto_dp&th=1), is often better tolerated and can still provide some amount of cervical support.  A cervical collar can cause cervical muscle deconditioning if worn more than 4 hours per day. Deconditioning will worsen instability and so should be avoided if at all possible. Cervical isometric exercises can be used to strengthen the unstable joint and prevent deconditioning if wearing a c-collar for an extended period of time.  If in a flare, a patient can try wearing a cervical collar continuously (day and night) or as close to continuously as possible for 1-2 weeks. This should be enough time to allow the neuroinflammation to improve.  **STRENGTHENING** The only way to "fix" UCI without surgery is to strengthen the cervical postural muscles so that they can stabilize the unstable joint.  Cervical isometric exercises, when done consistently, can accomplish this They are best paired with physical therapy for posture improvement and pelvic/core strengthening. I recommend the Ground Control Program for physical therapy appropriate for hypermobile individuals with UCI.  See attached handout on cervical isometrics. Strongly emphasize the importance of doing them gently and without moving the head. If the patient flares, do them even more gently. Start slow. # Physical Therapy Like ME/CFS itself, upper cervical instability is not widely recognized in mainstream or alternative medicine. If you suspect you may have upper cervical instability, the following physical therapists specialize in diagnosis, treatment, and, if necessary, referral to a knowledgeable surgeon: - Susan Chalela from Charleson, NC - Wendy Wagner from Chicago, IL - Patricia Stott from Aurora, CO - Heather Purdin from Portland, OR # Imaging Severe neurological symptoms such as seizure-like activity or episodic paralysis with apnea warrant urgent referral to a neurosurgeon who specializes in UCI. Referral can also be considered in patients with debilitating symptoms that do not improve with conservative management. I am most familiar with Dr. Fraiser Henderson Sr. who practices in Maryland and whose clinic is dedicated to people with hypermobility. For referral to his clinic I obtain an upright flexion/extension MRI (must be done in a dedicated upright MRI machine) and a rotational CT scan of the cervical spine. I recommend checking with the surgeon who you will be referring your patient to.  There are three specific measurements to request the radiologist provide on their report. Each measurement should be done in flexion, extension and neutral.  ## 1. Clivo-Axial Angle Line parallel to clivus and dens. The more acute that angle becomes, the more stretched the brainstem and spinal cord or there is more pressure on it from the dens. The more acute the angle the higher the risk of brainstem stretching or compression.  [>]144 NORMAL 135-144 MODERATE RISK <135 ABNORMAL ## 2. Grabb-Oaks Measurement This is the line measured from the tip of the clivus to the base of the 2nd cervical vertebrae. If it is longer then the soft tissue has to stretch longer.  <7 NORMAL 7-8 MODERATE RISK [>]9 ABNORMAL ## 3. Harris Measurement The line drawn perpendicular from the Grabb-Oaks line to the dura. If too long then there is too much motion. When the difference >4 mm between flexion and extension this is another suggestion that the patient is at higher risk of instability.  <10 NORMAL 10-11 ABNORMAL [>]11 SUGGESTIVE OF INSTABILITY