Veronica With Four Eyes

How I Explain Chiari Malformation

During the second week of my first year in high school, I experienced what is known as a thunderclap headache- meaning a headache that came on suddenly and with severe pain. Everyone around me figured that it was a tension headache or that I was nervous about starting high school, and I agreed with this assessment as well.

Then, I started having more difficulty seeing the board in class and started having trouble walking and balancing. I also started getting migraine headaches on top of my existing headache, and simultaneously became photophobic (sensitive to bright lights) and photosensitive (sensitive to flashing lights).

While starting at a new school can knock someone off their feet, it was clear that there was something else going on as well.

Shortly after my first hospitalization with severe migraine from an unknown cause, my mom was recommended a book called “Young Adult and Pediatric Headache Management” and read it cover to cover. The book itself was immensely helpful, but one line in particular changed my life:

“Consider the possibility of Chiari Malformation.”

What is Chiari Malformation?

From the National Institute of Health

“Chiari malformations are structural defects in the base of the skull and cerebellum, the part of the brain that controls balance.  Normally the cerebellum and parts of the brain stem sit above an opening in the skull that allows the spinal cord to pass through it (called the foramen magnum).  When part of the cerebellum extends below the foramen magnum and into the upper spinal canal, it is called a Chiari malformation. Chiari malformations may develop when part of the skull is smaller than normal or misshapen, which presses on the brain and forces the cerebellum to be pushed down into the spinal canal.  The pressure on the cerebellum and brain stem may affect functions controlled by these areas and block the flow of cerebrospinal fluid (CSF)—the clear liquid that surrounds and cushions the brain and spinal cord.”

From my own perspective

I am not a doctor, but if someone asked me to explain Chiari Malformation, here is what I would mention:

Chiari Malformation is a neurological condition that is caused by my brain being too big for my skull- my brain overflows from my skull and descends into my spinal column area- I literally have too much brain to contain. Because of the pressure on the occipital lobe/the back of my brain, I live with several neurological symptoms, and the condition contributes to my existing visual impairment/low vision as well. Chiari Malformation is present at birth, though people may not experience symptoms until they are older- in my case, I was 14 years old.

There are multiple types of Chiari Malformation, though for the purposes of this post I will be talking only about Chiari Malformation Type 1, which is the most common type and the type I live with.

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What are the symptoms of Chiari Malformation?

From the National Institute of Health

Some individuals with Chiari Malformation may not show any symptoms.  Symptoms may change for some individuals, depending on the compression of the tissue and nerves and on the buildup of CSF pressure.  Symptoms may include:

  • Headache, especially after sudden coughing, sneezing, or straining
  • Neck pain
  • Hearing or balance problems
  • Muscle weakness or numbness
  • Dizziness
  • Difficulty swallowing or speaking
  • Vomiting
  • Ringing or buzzing in the ears (tinnitus)
  • Curvature of the spine (scoliosis)
  • Insomnia
  • Depression
  • Problems with hand coordination and fine motor skills
  • Difficulty swallowing
  • Excessive drooling, gagging, or vomiting
  • Breathing problems
  • Difficulty eating and an inability to gain weight

From my own perspective

Chiari Malformation symptoms can change over time, or new symptoms can develop as well. In my case, the most significant Chiari Malformation symptoms I’ve experienced include:

  • Constant headache that gets worse after coughing or sneezing
  • Chronic back, neck, head, and shoulder pain
  • Vision loss- Chiari Malformation is present in 30% of people with my eye condition, accommodative esotropia
  • Ringing in the ears
  • Numbness in the hands and feet
  • Leg spasms
  • Mild difficulties with swallowing
  • Problems with coordination and fine motor skills, including trouble walking
  • Chronic migraines with flashing lights as a trigger
  • Sensitivity to light (related to migraines)

It’s worth noting that I do not necessarily experience all of these symptoms now, but they are the most significant symptoms I have dealt with over the years.

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How is Chiari Malformation diagnosed?

From the National Institute of Health

“Currently, no test is available to determine if a baby will be born with a Chiari malformation.  Since CMs are associated with certain birth defects like spina bifida, children born with those defects are often tested for malformations.  However, some malformations can be seen on ultrasound images before birth.

Many people with Chiari malformations have no symptoms and their malformations are discovered only during the course of diagnosis or treatment for another disorder.  The doctor will perform a physical exam and check the person’s memory, cognition, balance (functions controlled by the cerebellum), touch, reflexes, sensation, and motor skills (functions controlled by the spinal cord).  A doctor also may order imaging tests to diagnose a Chiari malformation or show hydrocephalus or bone abnormalities that might be associated with the disorder.”

From my perspective

As a short answer- very, very slowly

I was diagnosed with Chiari Malformation over four years after my initial thunderclap headache, and about three and a half years after a neurologist I was seeing at the time mentioned the strong possibility of Chiari Malformation being the cause for my symptoms. During those four years, I was referred to several neurologists and medical centers in the region, but all of my MRI scans were coming back normal. While a lot of neurologists we saw agreed that I likely had Chiari Malformation, I could not be definitively diagnosed without an abnormal MRI. My condition was finally diagnosed in October 2015 after the Chiari Malformation showed up on an annual MRI scan done by my neurologist.

It’s worth noting that while Chiari Malformation can co-exist with other conditions such as Ehlers-Danlos Syndrome, Syringomyelia, tethered cord syndrome, and similar conditions, I do not have any of these other conditions.

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How is Chiari Malformation treated?

From the National Institute of Health:

“Chiari Malformations that do not show symptoms and do not interfere with a person’s activities of daily living may only need regular monitoring by a physician with diagnostic imaging.  Medications may be prescribed to ease headache and pain.

In many cases, surgery is the only treatment available to ease symptoms or halt the progression of damage to the central nervous system.  Surgery can improve or stabilize symptoms in most individuals.  More than one surgery may be needed to treat the condition. The most common surgery to treat Chiari malformation is posterior fossa decompression, which creates more space for the cerebellum and relieves pressure on the spinal cord and should help restore the normal flow of CFS.  It involves making an incision at the back of the head and removing a small portion of the bone at the bottom of the skull (craniectomy).  In some cases, the arched, bony roof of the spinal canal, called the lamina, may also be removed (spinal laminectomy).

In some instances the surgeon may use a procedure called electrocautery to remove the cerebellar tonsils, allowing for more free space.  These tonsils do not have a recognized function and can be removed without causing any known neurological problems.”

From my perspective

After trying a lot of different migraine medications that caused adverse side effects, I stopped taking medication for symptoms related to my Chiari Malformation in 2013, except for trying Aimovig in 2019 for one month- long story short, it did not go well. I did Botox for cervical dystonia and it helped a lot with controlling shoulder pain related to the condition, though I have not had it in a few years. I made the personal decision to not go on long-term pain medication as I do not like taking those types of medications due to the side effects.

While surgery can be a great way to help treat the condition, it is not a cure and many people are not eligible to receive the surgery for a variety of reasons. Some of the greatest things that have helped me with living with Chiari Malformation include:

  • Massage therapy
  • Acupuncture (to control shoulder/back spasms)
  • Postural Restoration physical therapy
  • Assistive technology tools for mobility, vision, etc
  • Living an otherwise healthy lifestyle- eating healthy/regularly, drinking water, being involved with my community, etc

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Disclaimer

All information, content, and material of this website is for informational purposes only and are not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider. If you believe you are having a medical emergency, please call 911 or go to the nearest emergency room.

 

 

How I Explain Chiari Malformation