Ehlers-Danlos and Cerebrospinal Fluid Problems

Here are 2 articles from the http://www.upright-health.com/ that explain how the connective tissue laxity of EDS can lead to disturbances in CSF volume, pressure, and circulation.  These changes can in turn affect the pituitary gland and hypothalamus, which then lead to a cascade of hormonal problems, not to mention terrible headaches.

Ehlers-Danlos, CSF and MS

Ehlers-Danlos syndrome often affects the design and function of the skull and spine. Moreover, while most cases are not affected there is a significant incidence of multiple sclerosis (MS) in patients with Ehlers-Danlos Syndrome (EDS) due to their increase in susceptibility caused by structural problems in the skull and upper cervical spine.

In fact, the connection of EDS to MS underscores the role of upper cervical subluxations, Chiari malformations and cerebrospinal fluid flow (CSF) in neurodegenerative diseases such as multiple sclerosis (MS).

As far as design of the skull is concerned, Ehlers-Danlos syndrome is sometimes associated with underdevelopment, called hypoplasia, of the different parts of the skull including the posterior fossa of the base. The posterior fossa contains the cerebellum and brainstem. The cerebellum is a cauliflower shaped structure in the back of the skull at the bottom of the brain.

Hypoplasia, in this case shortening, of the occipital portion of the posterior fossa in Ehlers-Danlos causes crowding of the cerebellum. In some cases it causes a significant increase in the pitch of the tentorium cerebelli over the posterior fossa. A short base also forces the cerebellum forward and downward toward the foramen magnum. In addition, the decrease in space tends to squeeze CSF out of the brain.

Thus, hypoplasia of the posterior fossa predisposes certain EDS patients to Chiari malformations in which the cerebellum gets squeezed into the foramen-magnum or comes in contact with the base of the skull. Typically it is the tonsils of the cerebellum that are affected.

Hypermobility of the cervical spine can cause chronic subluxations of the upper cervical spine. Chronic subluxations of the upper cervical spine can sometimes cause a pannus formation on the odontoid process of the second cervical vertebra (C2), also known as axis.

It serves as a pivot joint. Most of the rotation in the neck takes place around the odontoid process.

During foward and backward head and neck movement, the odontoid process is generally held in proper position in the atlas vertebra by the transverse ligament.

Hypermobility in Ehlers-Danlos, decreases the ability of the transverse ligament to hold the odontoid in place. This causes excess motion between the odontoid process in the pocket of the atlas vertebra causing chronic irritation and swelling. This chronic irritation can cause a cyst, called a pannus in this particular case, to form on the odontoid process of axis. The pannus cyst formation invades and, consequently, compresses the contents of the spinal canal. In addition to EDS, pannus formations are also seen in other conditions such as rheumatoid arthritis.

Among other things, the upper cervical spinal canal contains the subarachnoid space for the flow of CSF between the brain and cord. Compression of the contents of the spinal canal can thus affect CSF flow going into and out of the cranial vault.

While Chiari malformations have been reported in some EDS cases, I suspect that other cases may be associated with benign intracranial hypertension (BIH) and still others with normal pressure hydrocephalus (NPH).

In any case, EDS patients often get MS signs and symptoms and the problem stems from poor CSF flow.

Humans are predisposed to problems with blood and CSF flow in the brain and cord due to changes in structure as a result of upright posture. Ehlers-Danlos or EDS is just one of many conditions.


The Pituitary Gland and Hypothlamus

The pituitary gland and hypothalamus are located in the brain. The pituitary is functionally related to, physically connected to, and, along with the target organs, controlled by the hypothalmus.

The hypothalamus and pituitary gland may play a role in many of the signs and symptoms associated with neurodegenerative diseases

certain inherited connective tissue disorders such as Ehlers-Danlos syndrome. In this regard, they may all share similar causes of hypothalamic-pituitary dysfunction related to obstruction of cerebrospinal fluid (CSF) flow.

Signs and symptoms of hypothalamus or pituitary problems can include: heat intolerance, sensitivity to cold, disruption in sleep cycles, short shallow breathing, fatigue, cog fog, decreased memory, irritability, mood swings, muscle cramps, increased urination and constipation.

The pituitary gland is part of the endocrine system of the body. The endocrine system secretes hormones directly into the bloodstream, unlike the exocrine system that uses ducts. The endocrine system is similar to the nervous system in that they are both informational signal systems and the pituitary and hypothalamus are part of the brain

The hypothalamus is responsible for certain metabolic processes and other activities of the autonomic nervous system responsible for maintaining homeostasis

such as: hunger, appetite, temperature regulation, sleep cycles, reproduction, stress and water balance

The hypothalamus also synthesizes and secretes certain neurohormones often called hypothalamic releasing hormones that stimulate or inhibit the secretion of pituitary hormones.

The pituitary gland is contained in a small separate subcompartment in the base of the skull called the sella turcica.

Hormones of the Pituitary

To understand how the pituitary gland and hypothalamus can be involved in producing some symptoms of neurodegenerative diseases and inflammatory conditions one must first look at the hormones produced and secreted by the pituitary. The hormones of the pituitary are under the control of releasing factors and hormones produced and secreted from the hypothalamus by way of a special arterial capillary blood supply system. Technically, the connection between the hypothalamus and pituitary gland is called the hypothalamic-hypophyseal portal system.

The hormones released by the pituitary gland play an important role in many functions needed to maintain homeostasis (balance) in the body as mentioned above.

For example, adrenocorticotrophic hormone plays an important role in maintaining blood pressure and blood sugar levels. Vasopressin, which is also known as antidiuretic hormone controls water volume in the body by way of the kidneys and arterioles. Follicle stimulating hormone controls sexual function and fertility. Growth hormone stimulates growth of tissues and bone. Luteinizing hormone controls sexual function and fertility in males and females. Oxytocin stimulates the uterus to contract during labor and the breasts to release milk. Prolactin stimulates female breast development and milk production. Thyroid stimulating hormone stimulates the release of hormones from the thyroid gland that regulate metabolism.

Hypopituitarism

problems with the pituitary gland can cause signs and symptoms such as the ceasing of menstrual periods, infertility, fatigue, and intolerance to stress and infection.

In adults, symptoms of empty sella syndrome and pituitary problems can develop slowly and vary greatly depending upon the hormones affected and their target organs. Symptoms are abdominal pain, cessation of menstrual periods, decreased appetite, decreased sexual interest (especially in men), failure to release milk, fatigue, headache, infertility in women, lack of sex drive in women, loss of armpit or pubic hair, loss of body or facial hair in men, low blood pressure, sensitivity to cold, visual disturbances, weakness and weight loss. Other symptoms can include facial swelling, hair loss, hoarseness or changing voice, joint stiffness and weight gain.

Thyroid Stimulating Hormone

In the early stages, hypothyroidism due to pituitary malfunction can be associated with increased sensitivity to cold; constipation; weight gain; water retention; low heart rate; fatigue; decreased sweating; muscle cramps and joint pain; dry, itchy skin; thin, brittle fingernails; rapid thoughts; depression; poor muscle tone; female infertility and problems with menstrual cycles; and elevated blood cholesterol levels.

Beta Endorphin Hormone

Beta endorphins released by the pituitary gland are used as analgesics to numb or dull pain in the body. The pain dulls because endorphins binds to opioid receptors and activates them. Beta endorphins are about eighty times more powerful at decreasing pain than morphine. Beta endorphins are also believed to be helpful in slowing the growth of cancer cells. They are further associated with promoting a feeling of well-being and increased relaxation.

The Suprasellar Cistern CSF and Pituitary Compression

Many of the signs and symptoms of pituitary malfunction are also found in neurodegenerative diseases. This could suggest similar causes. One possible cause is increased CSF volume or pressure in the suprasellar cistern.

increased CSF volume and pressure in the brain causes compression of nerves and blood vessels. An increase in CSF volume and pressure in the ventricles can cause compression of the structures that surround them or press the surface of the brain against the cranial vault. The surface of the brain contains major blood vessels and compression due to increased CSF volume or pressure can decrease blood flow in and out of the brain.

One of the primary causes of increased CSF volume, pressure, backjets and turbulance in the cisterns of the brain is most likely due to upper cervical misalignments.

Misalignments are from subluxations we EDS folks suffer in most of our joints.  Usually such misalignments “only” cause pain, but when they happen in the cervical spine, they can become life threatening.

 

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6 thoughts on “Ehlers-Danlos and Cerebrospinal Fluid Problems

    1. Zyp Czyk Post author

      After having triggered an instant, and luckily short, 45 degree tilt of my world after an awkward neck movement, I’m much more careful with it.

      It is scary to think that some random glance over the shoulder or tilt of the head could trigger such devastating consequences, but isn’t that just the way things go in these vulnerable bodies we inhabit.

      Like

      Reply
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