Summary of: Headache Disorders in Patients with Ehlers-Danlos Syndromes and Hypermobility Spectrum Disorders
Original article by: Lilian Yao, Kavya Subramaniam, Katherine M. Raja, Abi Arunachalam, Aubrey Tran, Tripti Pandey, Sahana Ravishankar, Sahan Suggala, Caitlyn Hendrickson, and Andrew J. Maxwell.
Summary by: Stephanie Milka, HMSA Journal Editor
Full aticle at: https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2024.1460352/full
Introduction
Ehlers-Danlos syndromes (EDS) and Hypermobility Spectrum Disorders (HSD) are conditions that affect the body’s connective tissues. These tissues provide support in skin, joints, and blood vessels. Individuals with these conditions often have very flexible joints and fragile skin. There are many types of EDS, but the most common is hypermobile EDS (hEDS).
Individuals can experience a range of symptoms that include but are not limited to fatigue, stomach and bladder issues, anxiety, dizziness, and headaches. Headaches can be one of the first and most disabling symptoms patients experience. Unfortunately, the timely and accurate diagnosis, as well as the introduction of treatment, is hindered because many clinicians aren’t fully aware of the association between headache disorders and hypermobility related conditions.
This article summary explains the types of headaches individuals with hypermobility-related conditions might experience and how they are treated. It also highlights the importance of awareness among healthcare professionals and the need for a multidisciplinary approach to managing these complex cases.
Migraine
Migraines are a common type of headache in people with hEDS and HSD. In the general population, migraines affect about 14% of people. However, in individuals with hypermobility, this number can be significantly higher, ranging from 40% to 75% in different studies. People with hypermobility also tend to have migraines that start earlier in life, occur more frequently, and may be more disabling.
Migraines can involve four different phases:
1. Prodrome (Premonitory Phase): This occurs hours or even days before the headache. Symptoms may include tiredness, mood changes, food cravings, neck stiffness, and increased sensitivity to light or sound.
2. Aura: Not everyone experiences this phase. If present, it may include visual disturbances (like seeing flashing lights or zig-zag lines), numbness, tingling, or speech difficulties. Auras usually last less than an hour and can precede or accompany the headache phase.
3. Headache Phase: This is the main phase, typically involving a moderate to severe throbbing pain that is located on one side of the head. It can last from a few hours up to 72 hours and is often made worse by movement. Common symptoms include nausea, vomiting, and sensitivity to light and sound.
4. Postdrome (Recovery Phase): After the headache subsides, many people feel exhausted, confused, or have difficulty concentrating for a day or two.
Management of migraines is multifaceted. Broadly, it can be divided into non-pharmacological treatment, pharmacological treatment, and neuromodulation. Non-pharmacological options include lifestyle modification, trigger avoidance (e.g., sleep deprivation, dehydration, missed meals) and neuropsychology. Pharmacological treatment options are divided into abortive and preventative treatment.
Abortive treatments (taken when a migraine starts): includes triptans, anti-nausea drugs, and pain relievers
Preventative treatments (taken regularly to reduce the number of headaches): includes blood pressure medications, antidepressants, anti-seizure drugs, and newer options such as calcitonin gene-related peptide (CGRP) inhibitors
When considering medications for migraine treatment clinicians need to be aware of any comorbidities an individual has, especially in the hypermobile population due to associations with dysautonomia, which can be contraindicated with many medications. Table 1 provides a list of the most common migraine preventative treatments, along with how it helps, and side effects to be aware of.
It should be noted that individuals with a hypermobility syndrome are at risk of developing medication overuse headache due to a higher prevalence of headache disorders and presence of comorbid non-headache pain (e.g., widespread musculoskeletal pain, abdominal, and pelvic pain). Medication overuse headache is a headache occurring on 15 or more days per month in an individual with a pre-existing primary headache disorder that develops as a result of regular analgesic overuse. If this situation occurs you should speak to your primary clinician so that a plan can be put together to relieve the medication overuse headaches and find an alternative solution for migraine treatment.
For information on neuromodulation please see the original article.
Orthostatic Headaches
Headaches are described as orthostatic when there is a postural component to them. Orthostatic headaches get worse when standing and improve when lying down. There are multiple causes of orthostatic headaches (Table 2), but they are most common in individuals with Postural Tachycardia Syndrome (PoTS), a condition often found in hEDS/HSD. PoTS causes a rapid heartbeat and dizziness when standing. Symptoms can also include:
• Headache
• Lightheadedness
• Fatigue
• Palpitations
Diagnosing PoTS involves measuring heart rate and blood pressure when lying down and standing. Treatment options for PoTS include increasing fluid intake (around 2–3 liters per day), increasing salt intake (if appropriate), and using compression garments to improve blood flow. Physical activity, especially exercises done while sitting or lying down, can help improve circulation. Medications such as beta-blockers, fludrocortisone, or midodrine may also be prescribed to help manage heart rate and blood pressure.
Spontaneous Intracranial Hypotension (SIH)
Spontaneous intracranial hypotension (SIH) occurs when there is a spontaneous leak of cerebrospinal fluid (CSF)—the fluid that surrounds and cushions the brain and spinal cord. This can happen without any obvious cause, but individuals with connective tissue disorders like hEDS and HSD are at higher risk due to increased fragility of the spinal dura (the outer layer covering the spinal cord).
The most typical symptom of SIH is a headache that is worse when standing and improves when lying down. This type of headache often becomes more severe as the day goes on or after physical strain like coughing or sneezing. Other symptoms can include neck pain or stiffness, nausea, hearing changes, balance issues, and, in severe cases, cognitive or visual disturbances.
Diagnosing SIH often involves imaging studies such as brain and spinal MRI, which may show signs of low pressure or evidence of a leak.
Initial treatment typically includes bed rest, hydration, caffeine, and avoiding activities that increase pressure in the head or spine. If symptoms persist, an epidural blood patch, a procedure where a small amount of the patient’s blood is injected into the spine to seal the leak, may be used
Because SIH can mimic or overlap with other headache disorders such as migraine, it is important for clinicians to consider this diagnosis in patients with hypermobility and positional headaches.
Craniocervical Instability (CCI)
CCI refers to loose joints at the base of the skull and top of the spine. In hEDS, weak ligaments can allow too much movement in this area. Symptoms can include:
• Neck pain
• Dizziness
• Trouble swallowing
• Sleep problems
Diagnosing CCI requires special imaging while moving the neck. Treatment starts with a neck brace or physical therapy, and surgery may be considered in severe cases. This condition is still being studied, and diagnosis can be challenging.
Cervicogenic Headaches
These headaches come from problems in the neck, such as arthritis or disc problems. In people with hEDS or HSD, unstable joints or scoliosis may cause strain on the neck. Symptoms include:
• Headache starting in the neck and moving to the head
• Neck stiffness
• Pain with neck movement
Treatment may include physical therapy and nerve blocks, though diagnosis can be tricky as symptoms may overlap with migraine.
Temporomandibular Joint Disorders (TMD)
Temporomandibular joint disorders (TMD) affect the joint that connects the jawbone to the skull. This joint can become painful or misaligned, especially in people with joint hypermobility, such as those with hEDS and HSD. In these individuals, the ligaments and connective tissues around the jaw may be looser than normal, increasing the risk of joint instability and irritation.
Symptoms of TMD can include:
• Jaw pain, especially when chewing or speaking
• Clicking, popping, or grinding sounds when moving the jaw
• Headaches, particularly around the temples or sides of the face
• Ear pain or a sensation of fullness in the ears
• Limited ability to open the mouth wide or a locking sensation
TMD may develop gradually or be triggered by jaw clenching, trauma, or stress. In individuals with hypermobility, even mild overuse or strain can worsen symptoms.
Diagnosis is usually made through a physical exam and a discussion of symptoms.
Treatment options include:
• A soft diet to reduce strain on the jaw
• Avoiding wide mouth opening or hard foods
• Jaw exercises and physical therapy
• Stress management techniques to reduce clenching or grinding
• Bite splints or night guards to protect the joint during sleep
• Pain relief through medications or local injections, including Botox in select cases
In most cases, symptoms improve with conservative treatment. Surgery is rarely needed and usually reserved for severe or persistent cases.
Tension-Type Headache (TTH)
TTH feels like a tight band around the head and is usually milder than migraines. It may come and go or become chronic. Unlike migraines, TTH usually doesn’t cause nausea or sensitivity to light. TTH doesn’t appear to be more common in people with hEDS/HSD, but it may still occur.
Treatment includes stress management, good sleep, and over-the-counter pain relievers. Chronic cases may benefit from medications like amitriptyline.
Chiari Malformation Type 1 (CM-1)
In CM-1, part of the brain (the cerebellar tonsils) pushes down into the spine. This can block fluid flow and cause:
• Headaches worsened by coughing or straining
• Neck pain
• Dizziness or trouble with balance
CM-1 is more common in people with hEDS. It’s diagnosed with a magnetic resonance image (MRI) scan. Surgery may help if symptoms are severe or if there’s pressure on the brain or spinal cord. However, it’s important to rule out conditions like SIH first, which can mimic CM-1.
Chiari Malformation symptoms can sometimes worsen over time or be triggered by events such as trauma, surgery, or changes in cerebrospinal fluid pressure. In patients with hEDS, overlapping structural issues at the base of the skull and upper spine may increase complexity and should be carefully assessed before deciding on surgery.
More conservative treatments may be tried first, such as avoiding activities that increase pressure in the head or neck, using a soft cervical collar during flare-ups, and working with physical therapists who understand hypermobility-related disorders. These approaches may help reduce symptoms for some individuals and can be part of a stepwise treatment plan before considering surgery.
New Daily Persistent Headache (NDPH)
NDPH is a rare type of headache disorder, that starts suddenly and becomes a daily, headache. It can feel like a migraine or tension-type headache and may follow an illness or stressful event. It can be hard to treat, but doctors may try different medications depending on symptoms. In some cases, this may actually be due to a spinal fluid leak and needs further testing.
Conclusion
Headaches are a common and often disabling part of life for people with hEDS and HSD. They can be caused by different mechanisms and may require various treatments. Because of the complexity of these conditions, working with a team of specialists who understand hEDS and HSD and its related issues is key to finding the right care.
If you have hEDS or HSD and experience frequent headaches, talk to your doctor about the types of headaches you have and ask about treatment options that consider your unique needs.
Original Article: Mehta D, Simmonds L, Hakim AJ, Matharu M. Headache disorders in patients with Ehlers-Danlos syndromes and hypermobility spectrum disorders. Front Neurol. 2024;15:1460352. doi: 10.3389/fneur.2024.1460352.
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