"Off the Notebook" Columnist : By HITESH KAKKAD

One of the many problems that Chiarians can also have is Ehlers-Danlos Syndrome. This is one of many different types of inherited and acquired disorders, as well as degenerative conditions of the craniocervical junction that can cause an array of neurological signs and symptoms such as dysautonomia and multiple sclerosis. Craniocervical syndromes can affect CSF and blood flow going into and out of the brain. They can also cause compressive myelopathies of the cervicomedullary cord.  They can cause the lower brainstem and upper spinal cord to malfunction due to compression.

Ehlers-Danlos Syndrome is a hereditary disorder of connective tissues.  It is important to note that you have to inherit the condition, which means you can't get it from multiple sclerosis. You have to be born with it. On the other hand, if you have the condition it makes you highly susceptible to getting multiple sclerosis. It is an inherited connective tissue disorder associated with loose tissues that can cause numerous problems such as overly flexible joints (hypermobile), skin that overstretches, dilated blood vessels and organs, as well as scoliosis of the spine to name a few.

Furthermore, some Ehlers-Danlos patients have a short clivus. The clivus is the slanted part of the base of the skull mentioned above. It is the front side of the posterior fossa. The front of the belly side of the brainstem lies behind it. A short clivus tends to indicate a lower ceiling height in the posterior fossa which further crowds the cerebellum and brainstem. The short clivus predisposes certain cases of EDS to Chiari malformations that put pressure on the belly side of the brainstem in front of the foramen magnum, which likewise can affect CSF flow.

As a result of increased elasticity of connective tissues, Ehlers-Danlos patients often have hypermobile joints, including the joints of the spine. The hypermobility of joints can cause subluxations and dislocations in the hands, feet, elbows and shoulders, as well as scoliosis and kyphosis in the spine. 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.

While Chiari malformations have been reported in some EDS cases, it is suspected that other cases may be associated with benign intracranial hypertension (BIH) and still others with normal pressure hydrocephalus (NPH). EDS patients often get MS signs and symptoms and the problem stems from poor CSF flow.

By studying and trying to solve problems with CSF flow seen in Ehlers-Danlos cases, we can learn more about the role the design of the skull and upper cervical spine play in Chiari malformations and CSF flow in other neurodegenerative conditions that affect the brain and cord such as Alzheimer's Disease, Parkinson's Disease and multiple sclerosis. 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.

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Hitesh Kakkad is both an International Advisor and an "Off the Notebook" Columnist for the International Chiari Association (ICA), which is a nonprofit organization. Kakkad, who has Chiari, is from Mumbai (also known as Bombay), India.


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Source : Chiariassociation.org , 24th June 2013