Sticky Blood-Hughes Syndrome Support

Professor Hughes December 2013 blog

For us, 2013 ended on a bang – strong winds, heavy rain, widespread flooding. Our small schnauzer, Lucy, has not been keen even to put her toes out of the back door!

My apologies for missing two months of blogs. I found that in the UK, the blogs were published on a website called ‘Health Unlocked’ and not on the Hughes Syndrome Foundation website, which I have been telling folks to look on. In future, you would be able to catch the blog on two other sites: and

Also, thanks to the generous sponsorship of INOVA (a California-based company making testing kits for various diseases, including auto-immune diseases), Kate now has copies of all previous blogs, bound in a single volume covering the past three years’ publications which is available at:

In October I did something very silly – I travelled to Melbourne, Australia and back for two days! I had accepted an invitation over a year ago, to give a lecture on Hughes syndrome to an Australian Psychiatry Congress. And it was very well worth the effort, from my point of view.

We know that Hughes syndrome can affect almost any part of the body but, in my opinion, one of the commonest (and most frightening) features, memory loss, often goes undiagnosed. So sad, as the improvement in memory loss in many Hughes syndrome patients when anticoagulation is started, can be truly dramatic. The interest and enthusiasm of these Australian psychiatrists, yet another specialty interested in Hughes syndrome, was so very stimulating.

Patient of the Month

Teenage glandular fever, St Vitus Dance and hip pain. What can they have in common? Well, for a start, they were all features in the history of Mrs F.W. – this month’s patient of the month.

Mrs F.W., aged 53, was referred for a second opinion. She had a severe, complex neurological illness. Her history of frequent, severe migraines went back over 30 years, few of the standard migraine medicines proving very successful. Over the past 5 – 7 years, things had worsened. She suffered a number of ‘mini’ strokes’ (TIAs) and an acute onset of ‘Meniere’s disease’ (severe balance problems). In addition, she developed sleep problems and severe memory problems.

Finally, she developed jerking movements in the arms, thought by her referring doctor to be chorea (St Vitus Dance).

She was investigated by a neurologist, who added temporal lobe epilepsy to the list of problems. This neurologist suspected Hughes syndrome – despite the negative blood tests – party because of the history. She also noted that Mrs F.W. had previously suffered a number of miscarriages and one late pregnancy loss.

When I saw the patient, there were two signs (which are so important in diagnosis): she had blotchy skin circulation (“livedo”), and very dry eyes and mouth. There were writhing choreiform (“Thai dancing”) movements of the right hand.

There were two additional features in the story. She had developed quite severe glandular fever in her late teens – in the patient’s opinion, all the other problems had started from there. Or so it seemed.

The second additional piece of the puzzle was ‘aches and pains’. These had been muscular – on and off for a number of years – and more recently with discomfort on movement in both hips.

What is this patient teaching us?

So many lessons. Firstly, that Hughes syndrome has a particular affinity for the nervous system – migraine, TIA and stroke, epilepsy, sleep disturbance, movement disorders – and, of course, memory loss are all symptoms of ‘sticky blood’ and the brain’s protestations at poor blood supply.

Clinically, this patient had Sjogren’s syndrome – with dry eyes and mouth, and widespread aches and pains. The blood tests were also suggestive, with strong levels of antibodies (both antinuclear antibodies – ANA – and thyroid antibodies). But no antiphospholipid antibodies.

The multiple miscarriages – and especially, tragically, the late pregnancy loss – are clues to the diagnosis, as in the finding of blotchy skin livedo.

This story has a very happy outcome. The patient was started on anticoagulants – first heparin and then warfarin. Total disappearance of the headaches and chorea, improvement in the balance problems and memory loss, and, to our surprise, marked improvement in sleep pattern.

And the hip pain? Very interesting. Disappearance of the bilateral hip pain. Perhaps I’ll keep this topic for a future ‘blog’. But remember – the hip requires a good blood supply, just as does the middle ear balance organ and the brain.

Finally, back to an old theme. Negative tests. For me, this is one of the most important lessons of all. Tests aren’t everything. We see so many patients who fit the diagnosis of Hughes syndrome in whom present conventional tests are negative.

Sixty years ago, that happened in ‘sero-negative’ rheumatoid arthritis. Fifty years ago, that happened in ‘sero-negative’ lupus (concepts which are now widely accepted). But the testing moved on.

I believe we must recognise this fact and focus on the clinical picture.

This patient developed ‘auto-immune’ disease (probably Sjogren’s and Hughes syndrome) following a severe attack of glandular fever. The E-B virus, the cause of the glandular fever, has long been a leading suspect in the events that trigger the explosion in the immune system that leads to diseases such as lupus, Sjogren’s, Thyroid and Hughes syndrome.

3 Replies

A great blog as usual, and fits so aptly around my family history through many members. MaryF


Loved this blog, very informative, and can relate to a lot of the symptoms, I'm glad I did not have to wait until my fifties to be diagnosed 38 was late enough.


A very interesting blog indeed, I am still being told mine doesn't run through the family but I've got a family of heart diseases...where I lost my Dad & a sister, one brother & uncle having problems with theirs, also Aunties having strokes & another Uncle just had a severe stroke! :( makes you wonder? x


You may also like...