Understanding and managing the symptoms of Dandy-Walker Syndrome in adulthood

I am delighted to share this guest post Healthline has contributed to my blog. I chose this topic because, as many of you know, I have this congenital malformation and I have struggled to find information about new symptoms I've had since my early 20s. I wrote about how they affect my life a few years ago.

Healthline articles have been very helpful to me, so I am incredibly honoured that they have helped me provide this information. Thank you, Healthline!

Understanding and managing the symptoms of Dandy-Walker Syndrome in adulthood

Important information about Dandy-Walker Syndrome and how you can manage your condition.

By Nicola Smith for Healthline

Ashley Ashbee's Brain

Dandy-Walker Syndrome is a malformation of the congenital brain in the cerebellum area (the back of the brain). Present in babies at birth, or developed later in life, this complex condition has varying symptoms and severities, making simple daily tasks challenging and exhausting. In this blog post, we're going to take a look at what Dandy-Walker Syndrome is, the symptoms, and how the symptoms can be managed in adulthood.

What is Dandy-Walker Syndrome?

Dandy-Walker Syndrome affects brain development in the cerebellum. The cerebellum is located at the back of your brain where your spinal cord meets your brain. Made of two halves, the cerebellum receives sensory information from the body and brain and then regulates and coordinates your movement. Voluntary movements such as such as posture, balance, coordination, and speech are coordinated by the cerebellum. While it is only a small part of the brain, it stores almost half of the brain's neurons and cells that are needed to process information and regulate movement.

In people with Dandy-Walker Syndrome, different parts of the cerebellum can develop abnormally. The abnormal developments in the cerebellum that can be present in people with Dandy-Walker Syndrome include:

• The central part of the cerebellum may be very small, absent, or abnormally positioned.
• The sides of the cerebellum may be small.
• The fluid filled cavity between a person's brainstem and cerebellum can be abnormally large.

These abnormalities in the cerebellum can affect a person's ability to coordinate their movement and cause other problems with intellect, mood, and other neurological functions.

The majority of people with Dandy-Walker Syndrome present malformation signs and symptoms at birth or within the first year of life. The condition can, however, develop in adulthood if the area of the brain where the brain stem and cerebellum meets does not continue developing normally. This means an adult may not present the intellectual symptoms associated with the condition, but can develop symptoms that affect their coordination and balance.

What are the symptoms of Dandy-Walker Syndrome in adulthood?

The symptoms of Dandy-Walker Syndrome may not appear until late childhood or adulthood in 10 to 20 percent of people with the condition. While there are several intellectual, physical, and neurological symptoms of the condition that may be present in people who have the condition from birth, the symptoms can differ in people who do not experience symptoms until adulthood.

The symptoms of Dandy-Walker Syndrome that can present in adulthood include headaches, an unsteady walking gait, paralysis of facial muscles, increased muscle tone, muscle spasms, and mental and behavioural changes. These signs and symptoms can be indicative of other neurological issues so, at this stage, diagnosis of Dandy-Walker Syndrome is often based solely on imaging. 

How can you manage the symptoms of Dandy-Walker Syndrome in adulthood?

There are several ways adults can manage the symptoms of Dandy-Walker Syndrome from changes in daily activities to medical intervention. One of the most common treatments for people with this condition is the insertion of a ventriculoperitoneal (VP) shunt to relieve fluid build-up in the cerebellum and reduce symptoms. This surgical procedure involves having a small tube placed in the ventricle (middle area) of the brain with the other end finishing in the chest or abdominal cavity.

A surgeon may also try a procedure called an endoscopic fenestration. This procedure involves insertion of an endoscope (a small, flexible viewing device) into the brain to create an opening between the third and fourth ventricle in the base of the brain. In creating an opening in this area of the brain, the aim is for this space to equalize pressure on the brain and central nervous system.

Non-surgical treatment for Dandy-Walker Syndrome can include anti-seizure medication, osteopathy, and physical therapy.

Dandy-Walker Syndrome can make the simplest daily tasks daunting and challenging. As the medical industry continues to research and understand the condition in greater depth, understanding how to manage your symptoms and finding out what can help you right now is paramount. If you have any questions or would like to share your own personal experiences of living with Dandy-Walker Syndrome in adulthood, post them in the comments below.

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Author: Nicola Smith