September is Chiari (pronounced kee-AH-ree) Malformation awareness month. Chiari Malformations are structural abnormalities of the cerebellum, the part of your brain that controls muscular movements such as coordination, posture, balance, and speech; as well as the spinal cord. Chiari Awareness Month aims to raise awareness about the chronic symptoms those with the conditions often experience. One commonly experienced symptom by those with Chiari Malformation is chronic, debilitating head and neck pain.
Chiari Malformation is not very common, however, it is being diagnosed at a higher rate than previously due to the increasing use of modern imaging diagnostic tests. According to WebMD, Chiari Malformations are more likely to affect females than males. Due to some people never developing symptoms as a result of this condition, it is impossible to estimate exactly how many people have Chiari Malformation.
Symptoms of Chiari Malformation
Some people with Chiari Malformations experience no symptoms, however, others may experience chronic symptoms including:
- Weakness in muscles
- Vison issues
- Hearing problems (including tinnitus)
- Neck pain
- Difficulty swallowing or speaking
- Balance and coordination issues
The symptoms can vary based on the type and severity of the Chiari Malformation.
Types of Chiari Malformation
There are four types of Chiari Malformations:
Type I is the most common type found in children. This occurs when the cerebellum extends into the foramen magnum, which is the opening at the base of the skull. Normally only the spinal cord passes through this opening. Type I is the only type of Chiari Malformation that can be acquired after birth, however, it is also possible to be born with this type of Chiari Malformation. Type I can be acquired through injury, infection, or harmful substances, due to the resulting excessive draining of spinal fluid. However, this isn’t as common as congenital Type I Chiari Malformation, which occurs during fetal development. The causes of congenital Type I Chiari Malformations are not yet fully understood.
Type II is normally only seen in children with spina bifida. Type II is known as the “classic” Chiari Malformation. Type II Chiari Malformation occurs when both the cerebellum and the brain stem extend into the foramen magnum. This is often diagnosed during pregnancy through ultrasounds, though it can also be diagnosed after birth in early infancy.
Type III is the most serious type of Chiari Malformation, as it has a higher mortality rate. Type III is also a rare type. Type III Chiari Malformation occurs when some of the cerebellum and the brain stem stick out through an abnormal opening at the back of the skull. This type of Chiari Malformation can cause life-threatening complications, as well as possible neurological issues. Seizures can also occur with this type of Chiari Malformation.
Type IV occurs when the cerebellum is either underdeveloped or incomplete. Parts of the skull and spinal cord may also be visible. This is another rare form of Chiari Malformation.
Conditions associated with Chiari Malformations
Hydrocephalus is an excessive build-up of cerebrospinal fluid (CFS) in the brain. This can be caused by a Chiari Malformation blocking the normal flow of this fluid. This can cause pressure within the head that can lead to defects, including an enlarged or misshapen skull. If left untreated, this can become fatal. Hydrocephalus can occur with any type of Chiari Malformation, but most commonly occurs with Type II.
Spina bifida is the incomplete closing of the bone and membranes surrounding the spinal cord. As said above, people with Chiari Malformation Type II usually have spina bifida.
Syringomyelia is when a cyst forms in the spinal cord’s central canal. This can cause pain, stiffness, and weakness in the neck, shoulders, back, and limbs.
Tethered cord syndrome occurs when the spinal cord abnormally attaches itself to the tissue at the bottom of the spine. This condition is progressive, as it gets worse as the child grows. This condition can result in the damage to nerves that control the muscles in the lower body and legs.
Spinal curvature is common in people who have syringomyelia or Chiari Malformation Type I. If the spine bends either to the left or right, this is called scoliosis. If the spine bends forward, this is called kyphosis.
Diagnostic tests and treatment
In the process of diagnosing Chiari Malformation, a doctor may order an MRI, CT scan, or X-ray. An MRI is the test that is used to diagnose Chiari Malformation most often. The doctor will also check things such as your motor skills, balance, and response to touch. This is because all of these functions are controlled by the spinal cord and the cerebellum.
If the Chiari Malformations cause no symptoms, then treatment may not be necessary. However, if pain is caused by this condition, it can be treated with pain medication. Surgery may be required to fix any functional abnormalities and prevent any progressive damage to the central nervous system.
Whether it be during September or throughout the rest of the year, organisations such as Conquer Chiari encourage people impacted by Chiari Malformation to spread awareness however they can. Whether it be through something as simple as sharing awareness posts on Facebook, or through something a bit more complicated like wearing clothing and jewellery mentioning Chiari, hosting a fundraiser, or participating in annual Chiari Awareness Month events such as the Conquer Chiari Walk.
Like with any other chronic illness, the spread of awareness and information regarding Chiari Malformations is incredibly important. Not only does this encourage understanding towards those who experience Chiari Malformations from those who don’t, but it also opens the doors towards new research regarding the causes and treatments of Chiari Malformation.
If you’re wanting to learn more about Chiari Malformations and the lives of those who experience it, you can visit websites such as the Mayo Clinic (https://www.mayoclinic.org/diseases-conditions/chiari-malformation/symptoms-causes/syc-20354010) and Conquer Chiari (https://www.conquerchiari.org/index.html).
About The Author
Amy Clements is a 20-year-old who has lived with chronic pain, the result of Fibromyalgia, since childhood. In her teens she was diagnosed with Complex Regional Pain Syndrome in her wrist, which was the result of a netball injury. Amy lives in New Zealand and studies Business part-time at University. She enjoys reading novels and writing. She especially enjoys writing about her experience with chronic illness.