Finally the book I’ve been waiting for has arrived fresh from the UK! Can’t wait to devour this from start to finish! ❤️
Finally the book I’ve been waiting for has arrived fresh from the UK! Can’t wait to devour this from start to finish! ❤️
When I was still a physiotherapy student almost ten years ago, I don’t remember being taught much about hypermobility. At most we were shown a Powerpoint slide about the Beighton scoring system to screen for hypermobility, but much was unknown about the correlation between hypermobility and the other medical conditions associated with it that can affect physical health. As physiotherapists, improving the physical health of those who come to see us through patient education, manual therapy and exercise prescription is definitely one of our key responsibilities.
Fast forward to 2019, more than 50% of my caseload in clinical practice now consists of those with Hypermobility Spectrum Disorders, including hypermobile Ehlers-Danlos Syndrome (hEDS), Marfan’s Syndrome and other Connective Tissue Disorders (CTDs). Clinically, I have found a close association between HSDs/CTDs and a myriad of other complex and intermingling conditions such as fibromyalgia, Chronic Fatigue Syndrome/Myalgic Encephalomyelitis (CFS/ME), dysautonomia, multiple allergies, and gut issues etc. These associations are supported by research (1)(2). Although some patients may not have these formal diagnoses, symptoms such as fatigue, dizziness related to positional changes, and pain are commonly reported in our patients with HSDs/CTDs. The degree and severity of these symptoms also vary widely amongst those who experience it.
Sharon Hennessey, my physiotherapy colleague and I work closely together at PhysioTec. Some patients come to see us upon referral from their General Practitioners and Specialists, and others have found us via word of mouth or online support groups. As this is a complex condition and an area of special interest for us, we have regular focus group meetings to discuss new research in the field and what we have learnt from interactions with our patients. These meetings have helped us develop and refine the patient resources we distribute, and provide effective assessment and treatment strategies. We also stay informed via the generous medical and non-medical community who have developed and regularly update some wonderful resources online (3). Often those with hypermobility benefit from the support of a multi-disciplinary team who are HSD/CTD-aware. Such a team may consist of a General Practitioner, Cardiologist, Geneticist, Rheumatologist, Gastroenterologist, Immunologist, Paediatrician, Psychiatrist, Psychologist, Gynaecologist, Occupational Therapist, Women’s Health Physiotherapist, Dietician, and Nutritionist etc.
As the condition presents very differently in every patient, an individualised treatment plan is critical. As a clinician and patient myself, I experience first hand the symptoms, including the frustrations and difficulty manoeuvring through the medical system which is not quite yet fully accepting of the diagnosis or condition. According to a recent speech by Lara Bloom, CEO of The Ehlers-Danlos Society, the average time taken to an EDS diagnosis is 12 years. Twelve years is an extraordinarily long “diagnostic odyssey” which would have traumatised, deconditioned (physically and psychologically), and let down many of those on the journey. I am heartened to be constantly working with this really resilient group of people who are not just patients but also often my teachers. Some of them have no choice but to educate themselves medically, that they become the “Project Managers” of their condition and comorbidities, and sometimes also of their families.
All our new hypermobile patients book their first appointment for an hour. This will allow us ample time to assess, formulate a treatment plan, educate and discuss any issues they might have. At the first appointment, depending on the priorities of the client, we may perform the following assessments:
The physiotherapy intervention may comprise of the following:
I have found my assessment and management strategies evolving over time as knowledge surrounding HSDs and CTDs continue to expand. Also symptom presentation vary widely amongst those with the condition, hence no two persons will receive the same assessment and treatment plan. I try to keep my website updated regularly of any new useful information that might come in. So, do keep an eye out for my new posts!
Before I sign off, I wish you all the best on your hypermobility journey!
Dr Chen is a registered member of the following organisations:
This narrative review by Martin VT & Neilson D (2014) is on the relevant research related to headaches associated with Hypermobile Ehlers-Danlos Syndrome (hEDS) and Marfan syndrome as these were the most studied Connective Tissue Disorders (CTDs) in current literature. This paper was published in the Headache journal by the American Headache Society. The first author, Martin V.T is from the Department of Internal Medicine of the University of Cincinnati at the United States of America.
The authors searched PubMed for articles with terms including joint hypermobility, joint hypermobility syndrome, EDS, Marfan syndrome, and specific headache disorders (e.g. migraine, cervical artery dissection, Arnold Chiari malformation, intracranial hypotension, atlanto-occipital instability, cervical disc disorders, cervical hypermobility, and temporomandibular disorders (TMD).
The underlying causes related to migraine headaches are currently unknown. However, the information outlined below will detail some prevalence statistics and proposed mechanisms related to migraines.
Frequency of migraine attacks was found to be 1.7 times increased, and headache-related disability was 3.0 times greater in patients with hEDS compared to controls.
One of the studies reported a 63% prevalence of migraines in people with Marfan syndrome.
Potential mechanisms related to migraine in patients with EDS-HT/JHS and Marfan syndrome
Several potential mechanisms were proposed to explain the increased prevalence of migraine in patients with CTDs in general.
Medical Management for Migraines
Intracranial Hypotension/CSF Leaks
Cerebral Spinal Fluid (CSF) leak may be suspected in those with orthostatic headaches, i.e. headaches aggravated by being upright (standing) and improved in a lying down, supine position.
In one study, 66% of patients with spontaneous CSF leaks were associated with subtle signs of CTDs. CTDs may be associated with CSF leaks due to laxity of the dura.
Dysautonomia and Coat-Hanger Headaches
Dysautonomias were found to be correlated with “coat-hanger headaches” in some studies, i.e. headaches aggravated by moving to upright positions.
“Coat-hanger” headaches are located in at the base of the skull (occipital), neck, and shoulder regions in a “coat-hanger” distribution.
The authors proposed support stockings and fludrocortisone as initial medical therapies to relieve symptoms followed by midrodrine if those treatments failed to work. They also proposed beta blockers in low dosages in those with hyperadrenergic form of POTS.
Chiari Malformation Type 1 (CM1)
CM1 is a developmental disorder of the lower part of the brain resulting in a downward displacement of the brain.
The downward displacement puts pressure on the brainstem and cranial/cervical nerves, obstructs flow of CSF in the region, and also compresses on the blood vessels in brain such as the vertebral artery and posterior inferior cerebellar artery (PICA). Headaches at occipital and suboccipital regions have been reported in those with CM1, and the symptoms are often aggravated by activities that increase intra-cranial pressures such as coughing, Valsalva maneuvers, exertion, and head dependency. The authors noted that patients with CM1 and CTD were more likely to suffer from POTS, sleep apnea, cervical spine subluxation and/or atlantoaxial instability, dysphagia and nausea.
Carotid Artery Dissections (CADs)
CADs result from a tear or lesion in the carotid artery.
It can be associated with a spontaneous mechanism, or occur after activities such as coughing, chiropractic manipulations, and trauma (e.g. sports, whiplash injury). It often presents as headache on the one side (unilateral).
New Daily Persistent Headache (NDPH)
NDPH are continuous headaches that commence on a specific day for 24 hours after onset. They are usually on both sides (bilateral) and 64% report throbbing or pressure like symptoms. They can be in the occipito-nuchal and retro-orbital regions of the head. The underlying cause is unclear but they are often preceded by infection, recent hysterectomy, or stress. One study found evidence of neck hypermobility in 12 patients with NDPH.
It was proposed that hypermobility of the neck causes NDPH due to irritation of the C1 to C3 cervical afferent nerves that converges on the trigeminal nucleus of the brainstem with the trigeminal nerve.
Physiotherapy focusing on muscle strengthening to stabilise the hypermobile neck, activity modification, posture retraining, and proprioceptive training is the treatment of choice.
Cervicogenic headaches are headaches originating from the neck (cervical spine).
Causes of cervicogenic headache reported in literature include cervical facet arthropathy, tumours/fractures/infections/trauma of the upper cervical spine, C2 neuralgia, neck tongue syndrome, occipital neuralgia, and atlantoaxial arthritis/instability. Other less studied causes include cervical disc herniations, cervical spondylosis, scoliosis and spinal stenosis. In a study, 61% of patients report headaches in the occipital area, with a mild to moderate intensity, and of daily frequency.
Atlantoaxial instability can be diagnosed via flexion and extension x-rays or MRIs of the cervical spine but is best examined by rotational 3D CT scan to identify rotational instability of the craniocervical structures.
Management consists of physiotherapy except for those with atlantoaxial instability. Injections such as epidural steroids or facet blocks may be used in those with herniated discs or facet hypertrophy. Surgical interventions such as laminectomy and cervical fusion for cervical disc disorders, may be done in patients who fail conservative therapies. Those with atlantoaxial instability require neurosurgical consultation about the need to stabilise C1 and C2 surgically.
Temporomandibular Disorders (TMDs)
TMDs are diagnosed using clinical or radiographic scans.
Headaches are reported in the temporal/masseter regions and is triggered by worsening of the TMD. TMD is associated with migraine and chronic daily headaches.
Patients with joint hypermobility have increased jaw opening (>40mm from upper to lower incisors) and are more prone to jaw dislocations. A study also found jaw clicking to be 1.7 times more likely in hypermobile patients than controls. Physiotherapy intervention, patient education and occlusive splints are the proposed management strategies.
Medication Overuse Headaches
A study found that patients with EDS have on average 8 different pain locations, and 51% were taking chronic narcotics for pain relief.
As EDS can be a multi-system disorder, if it common for patients to have medication overuse and related headaches. The authors recommend a multi-modal and multi-disciplinary approach.
Headaches and migraines in people with hEDS/EDS and Marfan syndrome can be associated with a variety of reasons.
The reasons are as follows:
Post by: drchenphysio.com
Limitations of this Review
As this is a summary narrative review, the contents written in this review do not encompass all literature related to headaches in those with hEDS and Marfan syndrome, and as such should be read and applied with discretion. Note that narrative reviews, albeit useful resources, are scientific evidence with a high risk of bias as a systematic, or more reproducible methodological approach was not applied in the crafting of such papers. An example of a study design with low risk of bias is a systematic review with meta-analysis, which was probably not applied in this paper due to scant existing research. This is also a summary review of the published paper, so readers beware that not all information from the original article may have been captured in this blog post.
A study by Rahnama L et al in 2015 was done to assess the thickness of the deep stabilising extensor muscles of the neck (cervical multifidus muscle, CMM) whilst contracting their shoulder muscles in 6 different directions.
The study compared the contraction of the CMM using a diagnostic ultrasound device between 20 healthy men, and 20 men with chronic mechanical neck pain.
The research found that contracting the shoulder muscles increased CMM thickness regardless of direction of force exertion. The increase in CMM thickness was observed in both healthy, and patient groups. In the patient group however, less CMM thickness changes were observed. The study also found that an increase in force production by the shoulder muscles also increased CMM thickness, with a larger effect being observed in the healthy participants compared to the patient population.
Shoulder strengthening exercises such as lateral raise, front raise, upright rows with resistance bands or dumbbells can strengthen the deep stabilising muscles of the neck.
The findings of the study indicate that the deep neck stabilising muscles are activated during performance of tasks by the upper extremities potentially as a means of stabilising the neck. Although this is not to be directly implied from the findings of the study, it can be postulated that shoulder strengthening exercises such as lateral raise, front raise, upright rows with resistance bands or dumbbells can strengthen the deep stabilising muscles of the neck.
Rahnama, Leila et al. 2015. Differences in Cervical Multifidus Muscle Thickness During Isometric Contraction of Shoulder Muscles: A Comparison Between Patients With Chronic Neck Pain and Healthy Controls. Journal of Manipulative & Physiological Therapeutics , Volume 38 , Issue 3 , 210 – 217