Chiari malformation – What are the signs and symptoms of Chiari malformations?

Chiari malformation - - what are the signs and symptoms?

Chiari malformation – – what are the signs and symptoms?

A Chiari malformation, previously called an Arnold-Chiari malformation, is where the lower part of the brain pushes down into the spinal canal.

There are four main types, but type 1, called Chiari I, is the most common.

In someone with Chiari I, the lowest part of the back of the brain extends into the spinal canal. This can put pressure on the brainstem, spinal cord, and obstruct the flow of fluid.

This page focuses on Chiari I malformations.

Are Chiari I malformations serious?

The severity of Chiari malformations can vary from person to person, but generally:

Chiari I malformations aren’t considered life-threatening

some people experience painful headaches, movement problems and other unpleasant symptoms (see below), but many people won’t have any symptoms

there’s a chance of developing syringomyelia (where a fluid-filled cavity called a syrinx develops in the spinal cord), which can damage the spinal cord if not treated promptly

surgery can usually stop the symptoms getting worse and can sometimes improve them, although some problems may remain

Talk to your doctor about what the condition means, what the implications may be for your health and what treatment you may need.

Symptoms of Chiari I malformations

Many people with a Chiari I malformation will not have any symptoms. Sometimes they’re only found after an (MRI) scan of the brain is carried out for another reason.

If symptoms do develop, they can include:

headaches – these are usually felt at the back of the head and may be brought on or made worse by coughing, straining, sneezing or bending over

neck pain

dizziness and balance problems

muscle weakness

numbness or tingling in the arms or legs

blurred vision, double vision and sensitivity to light

swallowing problems

hearing loss and tinnitus

feeling and being sick

difficulty sleeping (insomnia) and depression

If you develop syringomyelia, you may also experience problems using your hands, difficulty walking, pain, and problems with bladder or bowel control.

If you’ve been diagnosed with a Chiari malformation, you should contact your doctor for advice if you develop any new symptoms or your symptoms worsen.

Treatments for Chiari I malformations

Treatment for Chiari I malformation depends on whether you have any symptoms and how severe they are. You might not need any treatment if you don’t have any symptoms.

Painkillers can help relieve any headaches and neck pain.

If your headaches are severe or you have problems caused by the pressure on your spinal cord (such as movement difficulties), surgery may be recommended.

Surgery

The main operation for Chiari malformation is called decompression surgery.

Under general anaesthetic a cut is made at the back of your head and the surgeon removes a small piece of bone from the base of your skull. They may also remove a small piece of bone from the top of your spine.

This will help reduce the pressure on your brain and allow the fluid in and around your brain and spinal cord to flow normally. Read an NHS leaflet about decompression for Chiari malformation (PDF, 111kb).

Other procedures that may be necessary include:

Endoscopic third ventriculostomy (ETV) – a small hole is made in the wall of one of the cavities of the brain, releasing trapped fluid. See treating hydrocephalus for more information.

Ventriculoperitoneal shunting – a small hole is drilled into the skull and a thin tube called a catheter is passed into the brain cavity to drain trapped fluid and relieve the pressure. See treating hydrocephalus for more information.

Untethering – some children with a type 1 Chiari malformation have a tethered spinal cord, which means it is abnormally attached within the spine. Untethering involves separating the spinal cord and releasing tension in the spine. Read an NHS leaflet on tethered spinal cord (PDF, 193kb).

Spinal fixation – some people with Chiari I will have a hypermobility syndrome, such as Ehlers-Danlos syndrome, and may require surgery to stabilise their spine.

The aim of surgery is to stop existing symptoms getting any worse. Some people also experience an improvement in their symptoms, particularly their headaches.

However, surgery sometimes results in no improvement or symptoms getting worse. There’s also a small risk of serious complications, such as paralysis or a stroke.

Talk to your surgeon about the different surgical options and what the benefits and risks of each are.

Causes of Chiari I malformations

The exact cause of Chiari I malformations is unknown. It tends to be present from birth, but is normally only found in adulthood when symptoms develop or when an MRI scan is done.

Many cases are thought to be the result of part of the skull not being large enough for the brain.

Chiari I malformations can also develop in people with a tethered spinal cord, a build-up of fluid on the brain (hydrocephalus), and some types of brain tumour.

Chiari malformations can sometimes run in families. It’s possible that some children born with it may have inherited a faulty gene that caused problems with their skull development.

But the risk of passing a Chiari malformation on to your child is very small. And remember: even if your children do inherit it, they may not experience symptoms.

Multiple Sclerosis – what are the signs and symptoms of MS?

Terri wears a tie to promote MS awareness

Terri wears a tie to promote MS awareness

Symptoms of multiple sclerosis

Multiple sclerosis (MS) can cause a wide range of symptoms and can affect any part of the body. Each person with the condition is affected differently.

The symptoms are unpredictable. Some people’s symptoms develop and worsen steadily over time, while for others they come and go.

Periods when symptoms get worse are known as “relapses”. Periods when symptoms improve or disappear are known as “remissions”.

Some of the most common symptoms include:

fatigue

vision problems

numbness and tingling

muscle spasms, stiffness and weakness

mobility problems

pain

problems with thinking, learning and planning

depression and anxiety

sexual problems

bladder problems

bowel problems

speech and swallowing difficulties

Most people with MS only have a few of these symptoms.

See your GP if you’re worried you might have early signs of MS. The symptoms can be similar to several other conditions, so they’re not necessarily caused by MS.

Read more about diagnosing MS.

Fatigue

Feeling fatigued is one of the most common and troublesome symptoms of MS.

It’s often described as an overwhelming sense of exhaustion that means it’s a struggle to carry out even the simplest activities.

Fatigue can significantly interfere with your daily activities and tends to get worse towards the end of each day, in hot weather, after exercising, or during illness.

Vision problems

In around one in four cases of MS, the first noticeable symptom is a problem with one of your eyes (optic neuritis). You may experience:

some temporary loss of vision in the affected eye, usually lasting for days to weeks

colour blindness

eye pain, which is usually worse when moving the eye

flashes of light when moving the eye

Other problems that can occur in the eyes include:

double vision

involuntary eye movements, which can make it seem as though stationary objects are jumping around

Occasionally, both of your eyes may be affected.

Abnormal sensations

Abnormal sensations can be a common initial symptom of MS.

This often takes the form of numbness or tingling in different parts of your body, such as the arms, legs or trunk, which typically spreads out over a few days.

Muscle spasms, stiffness and weakness

MS can cause your muscles to:

contract tightly and painfully (spasm)

become stiff and resistant to movement (spasticity)

feel weak

Mobility problems

MS can make walking and moving around difficult, particularly if you also have muscle weakness and spasticity (see above). You may experience:

clumsiness

difficulty with balance and co-ordination (ataxia)

shaking of the limbs (tremor)

dizziness and vertigo, which can make it feel as though everything around you is spinning

Pain

Some people with MS experience pain, which can take two forms:

Pain caused by MS itself (neuropathic pain) – this is pain caused by damage to the nervous system. This may include stabbing pains in the face and a variety of sensations in the trunk and limbs, including feelings of burning, pins and needles, hugging or squeezing. Muscle spasms can sometimes be painful.

Musculoskeletal pain – back, neck and joint pain can be indirectly caused by MS, particularly for people who have problems walking or moving around that puts pressure on their lower back or hips.

Problems with thinking, learning and planning

Some people with MS have problems with thinking, learning and planning – known as cognitive dysfunction. This can include:

problems learning and remembering new things – long-term memory is usually unaffected

slowness in processing lots of information or multi-tasking

a shortened attention span

getting stuck on words

problems with understanding and processing visual information, such as reading a map

difficulty with planning and problem solving – people often report that they know what they want to do, but can’t grasp how to do it

problems with reasoning, such as mathematical laws or solving puzzles

However, many of these problems aren’t specific to MS and can be caused by a wide range of other conditions, including depression and anxiety, or even some medications.

Mental health issues

Many people with MS experience periods of depression. It’s unclear whether this is directly caused by MS, or is due to the stress of having to live with a long-term condition, or both.

Anxiety can also be a problem for people with MS, possibly due to the unpredictable nature of the condition.

In rare cases, people with MS can experience rapid and severe mood swings, suddenly bursting into tears, laughing or shouting angrily for no apparent reason.

Sexual problems

MS can have an effect on sexual function.

Men with MS often find it hard to obtain or maintain an erection (erectile dysfunction). They may also find it takes a lot longer to ejaculate when having sex or masturbating, and may even lose the ability to ejaculate altogether.

For women, problems include difficulty reaching orgasm, as well as decreased vaginal lubrication and sensation.

Both men and women with MS may find they are less interested in sex than they were before. This could be directly related to MS, or it could be the result of living with the condition.

Bladder problems

Bladder problems are common in MS. They may include:

having to pee more frequently

having a sudden, urgent need to pee, which can lead to unintentionally passing urine (urge incontinence)

difficulty emptying the bladder completely

having to get up frequently during the night to pee

recurrent urinary tract infections

These problems can also have a range of causes other than MS.

Bowel problems

Many people with MS also have problems with their bowel function.

Constipation is the most common problem. You may find passing stools difficult and pass them much less frequently than normal.

Bowel incontinence is less common, but is often linked to constipation. If a stool becomes stuck, it can irritate the wall of the bowel, causing it to produce more fluid and mucus that can leak out of your bottom.

Again, some of these problems aren’t specific to MS and can even be the result of medications, such as medicines prescribed for pain.

Speech and swallowing difficulties

Some people with MS experience difficulty chewing or swallowing (dysphagia) at some point.

Speech may also become slurred, or difficult to understand (dysarthria).

How best can we honor National Dysphagia Awareness Month for 2016? The National Foundation of Swallowing Disorders would live to hear your ideas..

Dysphagia and Multiple Sclerosis

Dysphagia and Multiple Sclerosis

As some regular readers know I have been working in the field of healthcare for over a decade now. The main area I’ve worked in was that of survey research with people with various different medical conditions. One of which was multiple sclerosis which became a key part of my work.

One area we looked at was the impact of different specific symptoms on the lifestyles of our respondents and now readers. You may find this discussion blog post of swallowing difficulties of interest. In fact around 40% of people with multiple sclerosis will suffer from dysphagia or difficulty swallowing.

Anyhow this month is National Dysphagia Awareness Month and its organisers The National Foundation of Swallowing Disorders are running a survey on how best to honour the month. So I thought the best contribution our end would be to provide a link so you can take part in the research. So here it is.

Many thanks in advance.





Achalasia – what you need to know but were afraid to ask!

Achalasia is a disorder of the gullet (oesophagus) where it loses the ability to move food along. The valve at the end of the gullet also fails to open and allow food to pass into your stomach.

Achalasia

Achalasia

As a result, food gets stuck in your gullet and is often brought back up.

A ring of muscle called the lower oesophageal (cardiac) sphincter keeps the opening from the gullet to the stomach shut tight to prevent acid reflux (acidic stomach content moving back up into the gullet).

Normally, this muscle relaxes when you swallow to allow the food to pass into your stomach. In achalasia, this muscle does not relax properly and the end of your gullet becomes blocked with food.

Achalasia is an uncommon condition that affects about 6,000 people in Britain. It is sometimes known as cardiospasm.

What are the symptoms?

Symptoms of achalasia may start at any time of life and usually come on gradually.

Most people with achalasia have dysphagia, a condition where they find it difficult and sometimes painful to swallow food. This tends to get worse over a couple of years.


It may cause you to bring back up undigested food shortly after meals and some of the vomited food may have been held up in your gullet for some time.

Bringing up undigested food can lead to choking and coughing fits, chest pain and heartburn.

Occasionally, vomit may dribble out of your mouth and stain the pillow during the night. If it trickles down your windpipe, it can cause repeated chest infections and even pneumonia.

You may experience gradual but significant weight loss.

However, in some people achalasia causes no symptoms and is only discovered when a chest X-ray or other investigation is performed for another reason.

What is the cause?

Achalasia is caused by damage to and loss of the nerves in the gullet wall. The reason for this is unknown, although a viral infection earlier in life may be partly responsible.

Achalasia may also be associated with having an autoimmune condition, where the immune system attacks healthy cells, tissue and organs. One recent study found people with achalasia are significantly more likely to have an autoimmune condition such as Sjogren’s syndrome, lupus or uveitis.

Although achalasia can occur at any age, it is more common in middle-aged or older adults.

There is no evidence to suggest that achalasia is an inherited illness. Women with achalasia can have a normal pregnancy and there’s no reason why their children will not develop normally.

How is it diagnosed?

If your GP thinks you have achalasia, you will be referred to hospital to have some diagnostic tests performed.

Barium swallow

A barium swallow involves drinking a white liquid containing the chemical barium, which allows the gullet to be seen and videoed on an X-ray.

In achalasia, the exit at the lower end of your gullet never opens properly, which causes a delay in barium passing into your stomach.

An ordinary chest X-ray may show a wide gullet.

Endoscopy

A flexible instrument called an endoscope is passed down your throat to allow the doctor to look directly at the lining of your gullet and stomach. Trapped food will be visible.

The endoscope can be passed through the tight muscle at the bottom of your gullet and into your stomach to check there is no other disorder of the stomach.

Read more about having an endoscopy.

Manometry

Manometry measures pressure waves in your gullet. A small plastic tube is passed into your gullet through your mouth or nose and the pressure at different points in your gullet is measured.

In achalasia, there are usually weak or absent contractions of the gullet and sustained high pressure in the muscle at the lower end of the gullet. The high pressure means the muscle does not relax in response to swallowing, causing symptoms of achalasia.

How is it treated?

The aim of treatment is to open the lower oesophageal sphincter muscle so food can pass into the stomach easily. The underlying disease cannot be cured but there are various ways to relieve symptoms which can improve swallowing and eating.

Dilatation (stretching the muscle)

A balloon (about 3-4cm in diameter) is used to stretch and disrupt the muscle fibres of the sphincter muscle at the lower end of your gullet.

This usually improves swallowing but may need to be performed several times or repeated after one or more years. Balloon dilatation does carry the risk of oesophageal rupture which may require emergency surgery.

Surgery

Under general anaesthetic the gullet is accessed through the abdomen (tummy) or, rarely, the chest. The muscle fibres of the lower oesophageal sphincter that fail to relax are divided. This usually leads to a permanent improvement in swallowing.

The operation is now performed by keyhole surgery (laparoscopy) and only requires an overnight stay in hospital.

Recovering from treatment

There are a few things you can do after dilatation or surgery to reduce symptoms:

  • chew your food well
  • take your time eating
  • drink plenty of fluids with your meals
  • always eat food sitting upright
  • use several pillows or raise the head of your bed so that you sleep fairly upright, which prevents stomach acid rising into your gullet through the weakened valve and causing heartburn

If heartburn develops after treatment, consult your GP as medication may be needed to reduce the acid reflux. Sometimes your surgeon may suggest you take this routinely to prevent problems after surgery. Read about treatments for acid reflux.

You should also see your GP if you still have swallowing difficulties or are continuing to lose weight.

It’s normal for chest pain to persist for a while after treatment – drinking cold water often gives relief.

Cancer risk

If the gullet contains a large amount of food that does not pass into the stomach in the normal way, the risk of cancer of the oesophagus (gullet) is slightly increased.

The increased risk is likely to be most significant in long-term untreated achalasia. It’s therefore important to get appropriate treatment for achalasia straight away, even if your symptoms are not bothering you.

According to Cancer Research UK, compared with the general population:

  • men with achalasia have an eight to 16 times higher risk of oesophageal cancer
  • women with achalasia have a 20 times higher risk of one particular type of oesophageal cancer (adenocarcinoma)

However, cancer of the oesophagus is very uncommon and although your risk is slightly increased, it remains highly unlikely.

Difficulty swallowing? Find out how other people cope with Oropharyngeal dysphagia!

Difficulty swallowing Oropharyngeal dysphagia

Difficulty swallowing Oropharyngeal dysphagia

Swallowing disorders (or more properly called Oropharyngeal dysphagia) can occur at any stage of somebodies life. It can occur for a whole range of reasons and can even be hereditary! Oropharyngeal dysphagia is often associated with conditions like multiple sclerosis (MS), Parkinson’s disease, stokes, brain tumours, amyotrophic lateral sclerosis, Bell’s palsy, and myasthenia gravis.

In this post we are looking at the underlying causes of your swallowing problems, how they were treated and with what success.   It would be great if you could use the comments box below to share your Oropharyngeal dysphagia experience.

Current treatments include surgery, Palatal lift prosthesis, electrical stimulation as well as more general physiotherapy, using a bolus or just a change in diet.

So with that in mind we would like to think about some of the following questions about your swallowing difficulties:-

What was the cause swallowing problem?  Was it a medical issue?

What was the effect of swallowing difficulties on your lifestyle?   In particular how has it impacted your eating habits and diet?

What treatments for Oropharyngeal dysphagia did you receive and dis they actually work?

If you had to give one piece of advice for somebody who has just started to show signs of a swallowing disorder what would it be?

Thanks very much in advance.  It would be great if you could share this with other people who might be interested in sharing their experiences.

 

suzyqw I am seeing a speech pathologist. She has me doing all kinds of mouth & tongue exercises. She also taught me to turn my head to the side & point my chin down to make swallowing easier. That is what has helped me more than anything. I realize that it is common for us as ms patients. I don’t want more drugs to so call fix the problems. My food seems to get stuck in the middle of my throat. It’s random but I can tell when I start eating something if it will happen or not. I have also noticed it seems to intensify with hot foods. See a speech pathologist. They can help with swallowing.
fedupandconfused the most difficult thing is that just when you think you’ve finished with noticing things something else crops up and because its random you don’t know if it’s worth mentioning or even if it’s related and besides you don’t want to sound neurotic. I’ve not been diagnosed as yet but swallowing has been one of those things that seemed odd to keep happening. Initially i thought it was random but over the last year it’s progressed. The first time was when I ate a chunk of tuna and it got stuck I literally lost my breath and thought I would die. I was able to take a gulp of water down inbetween bringing bits back up. At the time I put it down to tuna being fairly dry. Then over time with various different foods on occasion I have managed to swallow but it felt like I’d got it stuck in my lungs and i haven’t been able to cough it up. And recently I’ve noticed I need to let my food completely cool down before I eat it which is strange as I’ve always eaten my food really hot. And now I have to let it cool down, eat a little bit, leave it then go back again when the last lot has gone down. I have also recently noticed when I swallow it seems to stick like a ball in my stomach or inbetween the osophegus and into the stomach. I find myself standing up to get it to “slide down” and once that ball/knot has gone I can start the whole eating process again. It could be coincidence and I never realised it’s another possible symptom til I saw this site.
[…] Since the dawn of humanity, one thing that motivates us on the basest level is eating. Early humans had to learn to hunt to put food in their mouths and stomachs. Modern men go through life, doing their jobs to earn the means to be able to put food on their plate. When it comes to the basic means of survival, being able to eat the food that we need in order to live is one of the very first ones that need to be met. And because this is a basic need, eating of course comes naturally to us. We eat everyday of our lives, from the very first moment we were born. Eating then involves digesting. The first process of digestion starts at the mouth, where food is ground and chew so it can be swallowed. Eating and even swallowing itself seems like such an inconsequential act, made so normally and so innocuously by every human being on earth. So what happens when a person has difficulty swallowing? […]
BonnieLass1 mj1daisy I wish I had a clue. Everyone’s MS is so different. I randomly just forget how to swallow. It’s strange that something that should be so natural suddenly becomes something you have to actually think about doing. I would say that your doctor or a second opinion would be the way to go. Some things are hard to explain when it comes to MS and weird symptoms like not being able to swallow. It is scary though and I always end up choking and throwing up because I can’t catch my breath when it happens. I wish you the best of luck.

mj1daisy I have difficulty with swallowing. It’s kind of sporadic with both solids and liquids. I had a barium swallow done and because I thought I just had acid reflux but it turned out to be “nonspecific motor in coordination of the esophagus” that was before I was diagnosed with MS so they went even ruling an MS symptom out. I wonder what they would say now???
It feels like a rock or somethings hard gets stuck and I have to stop swallowing for 30 seconds or more for it to stop hurting. Could it be esophageal spasms??
BonnieLass1 I have trouble swallowing but I have not received any treatment for it. Mine is MS relapse related and seems to happen randomly. I noticed that it was more when I was fatigued. I don’t have to be eating..I just forget to how to swallow. I panic normally then choke and throw up from it. Tears running down my face and gasping for air. Its something that I can’t seem to predict and normally when I am fatigued it happens more often. The higher dose of solumedrol that I am on the less it happens. Its something that I need to discuss in my next visit I am sure.