Devic’s disease – what are the signs of Devic’s disease? Is this a type of MS?

Devic's disease

Devic’s disease

Neuromyelitis optica (NMO), also known as Devic’s disease, is a rare neurological condition.

Neurological conditions are caused by disease or damage to the brain, spinal cord or nerves.

NMO most commonly affects the optic nerves and spinal cord, which can lead to optic neuritis and transverse myelitis (see below).

Some people may only experience optic neuritis or myelitis but may have the aquaporin-4 antibody (also see below). In such cases, a person is said to have an NMO spectrum disorder (NMOSD).

Each person with NMO will experience different symptoms and require individually tailored care and support.

Some of the main symptoms of NMO include:

muscle weakness – reduced strength in one or more muscles that can affect mobility

impaired eyesight

nerve pain – which can be a sharp, burning, shooting or numbing pain

spasms and increased muscle tone – from nerve damage that affects muscle control

bladder, bowel and sexual problems

NMO UK has more information about the symptoms of NMO.

Optic neuritis

Optic neuritis is inflammation of the nerve that leads from the eye to the brain. It causes a reduction or loss of vision, and can affect both eyes at the same time.

Other symptoms of optic neuritis include eye pain, which is usually made worse by movement, and reduced colour vision where colours may appear ‘washed out’ or less vivid than usual.

Transverse myelitis

Transverse myelitis is inflammation of the spinal cord. It causes weakness in the arms and legs which can range from a mild ‘heavy’ feeling in one limb, to complete paralysis in all four limbs.

It may cause numbness, tingling or burning below the affected area of the spinal cord and increased sensitivity to touch, cold and heat. There may also be tight and painful muscle contractions (known as tonic muscle spasms).

Relapses in NMO

An attack or relapse of NMO results in the nervous system becoming inflamed. The inflammation usually occurs in the optic nerve and spinal cord, and causes new symptoms or the recurrence of previous symptoms.

Less common symptoms of NMO can include unexplained nausea and vomiting, unexplained itching and tonic spasms (painful muscle contractions). In someone with known NMO, these symptoms may signify a new relapse.

NMO symptoms can range from mild to severe. In some cases, there may only be one attack of optic neuritis or transverse myelitis, with good recovery and no further relapses for a long time.

However, in severe cases, there can be a number of attacks which lead to disability. Disability occurs because the body can’t always fully recover from damage caused by the attacks on the spinal cord and optic nerve.

NMO UK has more information about NMO relapses.

What causes NMO?

NMO is an autoimmune condition, which means a person’s immune system (the body’s natural defence against illness and infection) reacts abnormally and attacks the body’s tissues and organs.

An antibody against a protein called aquaporin-4 is present in the blood of up to 80% of people with NMO.

Antibodies are proteins produced by the body to destroy disease-carrying organisms and toxins.

In NMO, the immune system attacks aquaporin-4 which damages the myelin sheath (the protective layer that surrounds nerve cells in the brain and spinal cord and helps transmit nerve signals).

Who’s affected by NMO?

NMO is a rare condition. In Europe, it’s estimated that there’s one case of NMO for every 100,000 people. In the UK, it’s thought that NMO affects less than 1,000 people.

NMO can affect anyone but it’s more common in women than men, with about four females being affected for every male.

Although the condition is thought to be more common in people of Asian and African descent, an increasing number of white (Caucasian) people are also being diagnosed.

Diagnosing NMO

It’s important that NMO is correctly diagnosed. It can sometimes be confused with multiple sclerosis, which also affects the brain and spinal cord and has similar symptoms. However, the treatment is different.

A neurology specialist will discuss your symptoms and medical history with you.

You’ll have a magnetic resonance imaging (MRI) scan of your brain and spinal cord. Some people with NMO (up to 60%) have lesions on their brain and spine, which are different to the lesions of someone with MS.

A blood sample will be taken and tested for aquaporin-4 antibodies.

A lumbar puncture is another test you may have. A sample of cerebrospinal fluid (CSF) is taken from the spine using a hollow needle that’s inserted into the lower part of the spine.

The fluid sample will be sent to a laboratory to be tested and to look for evidence of conditions affecting the brain, spinal cord or other parts of the nervous system.

In some cases of transverse myelitis, there’s an increase in the level of proteins or white cells.

NMO UK has more information about how NMO is diagnosed.

Treating NMO

There’s no cure for NMO, so treatment aims to manage attacks and symptoms, and prevent relapses.

Every person with NMO is affected differently and some may have much milder symptoms than others. However, early treatment is usually needed to prevent further episodes and permanent disability.

Medication is used to reduce nerve inflammation, suppress the immune system and treat any pain. Rehabilitation techniques, such as physiotherapy, can also help with any reduced mobility that the relapses cause.

At these centres, research is ongoing to find possible future treatments for NMO.

To be referred to one of these centres, a GP referral letter is all that’s needed. These specialist services are nationally funded, so GP practices won’t have any additional costs for referring.

NMO UK has more information about treatments for NMO.

Driving

Optic neuritis (inflammation of the optic nerve) could affect your ability to drive.

Hypermobility – What are the signs and symptoms of joint hypermobility?

Joint hypermobility

Joint hypermobility

Symptoms of joint hypermobility

Many people with joint hypermobility have few or no problems related to their increased range of movement.

 

Being hypermobile does not necessarily mean you will have any pain or difficulty. If you have symptoms, it is likely that you have joint hypermobility syndrome (JHS).

Symptoms of joint hypermobility syndrome

JHS can cause a wide range of symptoms, including:

pain and stiffness in the joints and muscles – particularly towards the end of the day and after physical activity

clicking joints

back pain and neck pain

fatigue (extreme tiredness)

night pains – which can disrupt your sleep

poor co-ordination

recurrent joint dislocations – such as a dislocated shoulder

recurrent soft tissue injuries – such as sprains and sports injuries

A person with JHS may also have a number of other symptoms related to weaknesses in the connective tissues throughout their body. Some of these symptoms are described below.

Digestive system problems

JHS can cause symptoms that affect your digestive system, because the muscles that squeeze food through your digestive system can weaken.

This can cause a range of problems, including:

gastro-oesophageal reflux disease (GORD) – where stomach acid leaks from your stomach to your gullet, causing symptoms such as heartburn

gastroparesis – where the stomach has difficulty emptying its contents into the small bowel, which can cause bloating and nausea

constipation

irritable bowel syndrome (IBS) – a disorder that affects the digestive system, causing tummy pain, diarrhoea and constipation

Postural orthostatic tachycardia syndrome (POTS)

JHS can also cause abnormalities in the part of your nervous system that controls bodily functions you do not actively think about, such as the beating of your heart. This is known as your autonomic nervous system.

These abnormalities can cause problems when you stand up or sit in the same position for a while. Your blood pressure can drop to low levels, making you feel sick, dizzy and sweaty. You may also faint.

In some people, these abnormalities can lead to postural orthostatic tachycardia syndrome (POTS). POTS causes your pulse rate to increase rapidly within a few minutes of standing up. You may also experience:

dizziness or fainting

headaches

tummy upsets

sweating

a sensation of anxiety

purple puffy fingers and feet

a pounding or fluttering heart beat (heart palpitations)

Other problems

People with JHS may have other related conditions and further symptoms, including:

stress incontinence – a type of urinary incontinence that occurs because the pelvic floor muscles are too weak to prevent accidental urination

hernias – where an internal part of the body, such as an organ, pushes through a weakness in the muscle or surrounding tissue wall

in women, pelvic organ prolapse – where the organs inside the pelvis slip down from their normal position

varicose veins – swollen and enlarged veins, usually blue or dark purple

flat feet – where the inner part of your feet (the arch) is not raised off the ground when you stand

headaches

drooping eyelids

a tendency to bruise easily and develop stretch marks

thin or stretchy skin

Although a link is not entirely certain, it is thought that some people with JHS may be at an increased risk of developing osteoarthritis earlier in life than usual.

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.

Thrombosis – Frequently Asked Questions about Thrombosis

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a)      What is thrombosis?

b)      What is the differences between arterial thromboembolism (ATE) , venous thromboembolism (VTE) and pulmonary embolism (PE)? 

c)       What is hospital-acquired VTE?

d)      What are the early signs and symptoms of the different types of thrombosis?

e)      What treatments are available?

f)       What are the long term effects of thrombosis?

g)      What are the plans for World Thrombosis Day 2019?

h)      Where can people go for more information?

Five Common Symptoms of Vitamin D Deficiency




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Over 40% of adults in the US have vitamin D deficiency, according to a 2011 study. It is estimated that about 1 billion people worldwide are deficient. In this video we’re looking at the 5 biggest symptoms linked with low vitamin D level