Ehlers Danlos Neurological Manifestations

EDS is a term used to refer to a collection of diseases that include more than a dozen different conditions. The disorders are all characterized by the fact that they damage the body’s neurological tissue. Still, different kinds cause symptoms and difficulties in many other body regions, including the skin.

Even though most of EDS’s visible symptoms manifest themselves in the joints and skin, there is a relationship between EDS and an elevated risk of some brain-related issues. EDS, particularly the hypermobile and classical variants of the condition, can create difficulties with brain or spine development when weak connective tissue causes nerve cells to be damaged or malformed. It is not entirely understood how a connective tissue disease affects the central nervous system, but all the signs and manifestations are mentioned below.

Neurological symptoms of Ehlers-Danlos syndrome

People who have EDS are at an increased risk of developing a variety of brain-related disorders. Brain problems are most common in the hypermobile and classical forms of the illness. Still, other types of EDS are occasionally linked with diseases of the central nervous system, including Alzheimer’s disease.

Neurological issues in EDS:

  • Chiari malformation: It is an abnormal development of the brain stem that happens when a portion of the brain stem grows outside the skull. According to some experts, it is possible that weakened connective tissue at the intersection of the head and spine contributes to the condition’s progression or perhaps causes it.
  • Idiopathic intracranial hypertension (IIH): This disease is characterized by unusually high pressure in the fluid that surrounds the brain and causes it to bulge outward. However, some investigations have shown that there may be a relationship between IIH and EDS, despite the fact that the origin of the illness is unknown.
  • Brain aneurysms: Vascular EDS results in weaker blood vessels that are more susceptible to rupture. The rupture of a weakening area in a blood artery (also known as an aneurysm) is particularly hazardous when it happens in a portion of the body where regular blood flow is required for proper function. In addition to aneurysms in the aorta (the major blood vessel that transports blood from the heart), specific investigations have discovered that individuals with vascular EDS are at higher risk of brain aneurysms, which are not yet fully understood.
  • Headaches: In comparison to the general population, those with EDS have a greater incidence of migraine headaches. Scientists are still trying to figure out if there is any relationship between EDS and headaches.

Other neurological symptoms have been linked to EDS in addition to the ones listed above. Although just a limited number of instances have been reported, experts have not yet identified the underlying source of the symptoms. On the other hand, the link with EDS implies that the symptoms may be caused by the disorder’s effects on the brain and the nervous system, respectively. The following are examples of possible brain-related symptoms:

  • Fatigue
  • Disruptions in sleep
  • Problems with concentration
  • Muscle weakness
  • Stroke
  • Epilepsy

In addition to brain-related issues, EDS has been linked to problems with the spine and other areas of the central nervous system, such as the following:

  • Many people have kyphoscoliotic type EDS, which causes gradual curvature of the spine that is severe. It is possible that this curvature will cause breathing, digesting, and mobility difficulties if it is severe.
  • Instability of the cranium and the spine: EDS can create instability in the joints that connect the head to the spine as well as between the bones of the spine. Because of the excessive degree of movement in these joints, the risk of injury to sensitive nerve tissue is increased.
  • Tethered cord syndrome (TCS): In individuals with EDS, TCS is a disorder in which an aberrant connection forms between the spinal cord and surrounding tissue, which can be life-threatening. Pain, numbness, and other neurological problems are possible as a result of the disease.
  • Tarlov cyst syndrome: During this disease, fluid-filled cysts form around the spinal cord, which is painful. Cysts can exert pressure on nerve tissue, resulting in neurological symptoms such as discomfort and difficulty controlling one’s bladder or bowel movements. Tarlov cysts have been connected to EDS by some experts, although scientists have not yet discovered a clear relationship between the two conditions.

Other symptoms associated with Ehlers-Danlos Syndrome.

The most prevalent types of EDS manifest themselves in the skin and joints, where the symptoms are most noticeable. Among the signs and symptoms are:

  • Joints that are loose and exceptionally flexible: Infants with EDS may have difficulty sitting, standing, or walking. Furthermore, because the joints are not properly supported by connective tissue, patients are in danger of dislocation or injury to their joints. Chronic joint pain can also be a sign in some cases.
  • Skin that is soft, flexible, and delicate has a velvety feel and is quite elastic in many cases. The upshot is that patients bruise quickly and that wounds may break apart easily, leaving a characteristic wrinkled scar pattern. In addition, skin wounds might be challenging to heal since the skin may not be able to retain sutures without ripping.

The following are examples of EDS that affect other areas of the body:

  • Spondylodysplastic type EDS is characterized by skeletal anomalies such as low height and bent limbs.
  • Periodontal type EDS is characterized by irregularities in the gums that might result in tooth loss and other issues in the future.
  • Cardiac-valvular type is a kind of heart valve. In patients with EDS, anomalies in the heart occur, which significantly impair the organ’s ability to function.
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