Chiari & Syrinx

What Is a Chiari Malformation & Syrinx?

Chiari Malformation and Syrinx

As many of you know I have undergone double brain surgery back in 2015 and unfortunately had many complications to include, Hydrochephalis, inflammatory tissue that wrapped itself around my brain, Chemical Meningitis twice, a bleed on my brain, the patch inserted on my brain rejected and much more I also suffer with a progressive neurological condition called a syringomyelia heres a small overview of the conditions I have:

What is a Chiari Malformation?

A Chiari malformation is when part of the cerebellum, or part of the cerebellum and part of the brainstem, has descended below the foramen magnum (an opening at the base of the skull).

The cerebellum is the lowermost part of the brain, responsible for controlling balance and co-ordinating movement.The brainstem is the part of the brain that extends into the spinal cord.

Usually the cerebellum and parts of the brainstem are located in a space within the skull above the foramen magnum. If this space is abnormally small, the cerebellum and brainstem can be pushed down towards the top of the spine. This can cause pressure at the base of the brain and block the flow of cerebrospinal fluid (CSF) to and from the brain.

What causes Chiari malformations?

The Chiari I malformation is thought to be in most cases related to a small posterior fossa (the cranial bit of the skull that holds the cerebellum). The Chiari II and III malformations are caused by abnormalities in the structure of the brain and spine which develop in the womb before birth. This might be due to genetic factors or other reasons, such as a lack of vitamins and minerals during pregnancy.

Although rare, some Chiari malformations can be caused by an injury or an infection. Chiari I malformations can also be caused by a build up of pressure in the brain, for example, hydrocephalus or tumour, or by the spinal cord being held down (for example, a tethered cord).

Is it hereditary?

It is possible that children born with this disorder may have inherited the gene. Research is currently being carried out to identify this gene. However, the risk of passing a Chiari malformation on from parent to child is very small. Also, if the child does inherit the gene, most will not develop symptoms. 


What are the symptoms of Chiari malformations?

The main symptoms people with malformations might experience are:

•headaches (usually at the back of the head and often made worse by coughing, sneezing, straining or bending over)

•neck pain

•dizziness and balance problems

•unusual feelings in the arms or legs (numbness or tingling)

•muscle weakness and paralysis

•visual problems and involuntary movement of the eyes (nystagmus)

•swallowing problems

•hearing loss and tinnitus

People might also experience nausea (feeling sick), vomiting (being sick), insomnia (difficulty sleeping) and depression. For help with depression, your GP can advise you of mental health services.

What is a Syringomyelia / Syrinx?

Syringomyelia / Syrinx is a generic term referring to a disorder in which a cyst or cavity forms within the spinal cord. This cyst, called a syrinx, can expand and elongate over time, destroying the spinal cord. The damage may result in pain, paralysis, weakness,and stiffness in the back, shoulders, and extremities. Syringomyelia may also cause a loss of the ability to feel extremes of hot or cold, especially in the hands. There is also a disorder that generally leads to a cape-like pain (extreme pain, pressure, and many other painful symptoms in the area where a cape would be) and temperature sensation along the back and arms. Each patient experiences a different combination of symptoms. These symptoms typically vary depending on the extent and, often more critically, to the location of the syrinx within the spinal cord

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