Over the recent decade of clinical practice, I have increasingly served clients with Ehlers-Danlos Syndrome (EDS), a heterogeneous group of hereditary connective tissue disorders. Their clinical features include multi-joint hypermobility, skin extensibility, tissue fragility, and chronic pain resulting in their related physical, nutritional, and psychological challenges.
Our practice has special interests and expertise in managing EDS clients with altered sensorimotor function, affected by neurological and spinal manifestations from cervical instability (i.e. Cranio-Cervical Instability (CCI), Arnold-Chiari Malformation, Upper Cervical Ligament Laxity). Commonly associated symptoms among clients with CCI include neck pain/stiffness, heaviness of controlling head or feeling of bobble headedness, blurry vision, dizziness, headache, sleeping disorder, hearing impairment, speech impairment, swallowing and imbalance, and dysautonomia.
These clients present with severe medical complexity making it very difficult for clinicians to determine whether orthopedic conservative care approach vs. surgical approach is appropriate to pursue. As long as the overall nature of client’s condition and stability are not progressively worsening, it is very reasonable to recommend clients with EDS to participate in physical therapy and individualized exercise programs to improve pain and function. Optimally, it helps clients to develop self-coping strategies to work towards staying active and maintaining healthy lifestyle. In general, these individualized programs address joint positioning sense, postural control, low-impact strength, endurance, balance, cardiovascular training, and functional tasks with proper body mechanics.
In order to serve clients with CCI who are seeking conservative management or post-surgical recovery, our practice extensively utilizes progressive sensorimotor retraining program and refines strategy continuously as needed. Sensorimotor retraining program consists of step-by-step exercise implementation into a progressive interaction of all relevant coordinated systems (somatosensory, proprioceptive, visual, vestibular, and central integration). This approach helps to control proper head and neck orientation (relocation of upper cervical position and facilitation of proper neck muscle endurance) in space and maintain equilibrium (postural stability/balance/coordination).
I have observed many clients with CCI complaining of pain, stiffness, fatigue, poor cervical endurance, and muscle spasm around neck as well as upper and mid-back. These common symptoms are considered as perpetuating factors of overall stability issues and adversely affect the feedback of the proprioceptors in the cervical spine. The altered sensory inputs from cervical spine contribute to development of abnormal control of head-neck positional awareness, oculomotor disturbance (visual tracking), altered cervical joint positional sensation, poor neck stability, headache/dizziness provoked by neck or head movements, and postural imbalance.
As a general rule, it is important to address all aspects of sensorimotor impairments progressively as clinicians continue to monitor irritability, severity, stability of client’s overall condition over the course of the management. Throughout the implementation of sensorimotor retraining plan, physical therapy should also include the treatment of musculoskeletal impairments of pain/stiffness/spasms in the neck and the improvement of postural correction using manual therapy/dry needling/breathing/relaxation techniques.
The flow of sensorimotor retraining strategy is recommended: 1. Altered head-neck awareness and oculomotor disturbances are addressed prior to the introduction of training neck movement control. 2. Gaining proper range of motion and reposition of upper cervical repositioning are necessary before training with movement control exercises. 3. The imbalance of postural stability is addressed at the last stage to minimize any compensatory movement patterns. 4. Finally, there is an integration of joint position sense, oculomotor control (eye exercises), neck-scapular-trunk muscle endurance, and movement sense that are integrated into the balance/coordination exercise program.
It is quite challenging to make a smooth transition of implementing above treatment strategies without understanding client’s nature of conditions (progression vs. regression) and listening them in person. By this way, we can make proper clinical decisions and fully utilize a holistic approach driven by clients being more centered in the management of CCI. We are devoted for your recovery of function successfully and guide you into staying healthy lifestyle.