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July 2014

07 July 2014

July in London was the seventh sunniest in London since records began. (Official!).

We were fortunate in deciding to take a holiday in deepest Kent this year. Blue skies and glorious colours (and a very happy Lucy the dog!).

Quite a few media stories of young women with clots, seizures, miscarriages, etc., but, sadly, little or no mention of Hughes syndrome – a preventable cause.

We are doing our best to spread the word. In addition to the many lectures, GP meetings and patients’ meetings, we are very fortunate in the work done by Kate and her team and by Lynne Kirwin, with her media contacts.

More recently, we have been joined by Lynn Faulds-Woods, who, despite her busy TV schedule presenting ‘Watchdog’, has thrown her fantastic energy behind our cause.

Some weeks ago we had our summer patients’ meeting. Because of my interest in genetic aspects of Hughes syndrome, I arranged a questionnaire. The results were striking. Nearly 60% of all patients who responded had a close family member (often many family members) with either Hughes syndrome or another auto-immune illness such as multiple sclerosis or lupus.

I will hope to have a detailed analysis of the survey in time for the next month’s blog.

Patient of the month

A dramatic story – Miss L.J. aged 32 had, for a number of years, suffered from a debilitating set of neurological symptoms. She had frequent movement jerks or ‘tics’ – often affecting one or other arm, or involving sudden head movements. Various medicines had been tried, to little avail.

Over recent years these movements had worsened and had developed into prolonged attacks of writhing movements of all the limbs, with facial contortions and with gross twisting spasms of the head and neck.

In retrospect, these symptoms, in much milder form, had been present since late childhood. The patient had been investigated by paediatricians and neurologists and a diagnostic label of Tourette’s syndrome proposed.

Sadly, over the past year, things had deteriorated and Miss L.J. had had increasingly frequent admissions to various casualty departments. Because there was some temporary relief from sedatives such as benzodiazepine, suggestions were made that some of the attacks were, if not hysterical, probably made worse by anxiety.

But the history was interesting. There was a family history of auto-immune disease.

Furthermore, she had had two other problems – firstly, a long history of severe migraine headaches and secondly, years previously, a number of episodes of low platelets.

Tests (including a brain MRI) were normal, with one striking exception – a strongly positive aPL (antiphospholipid) test.

To cut a long story short, Miss L.J. was started on heparin and later changed to warfarin. Striking, exciting improvement!

‘Work in progress’, but I promise to update everyone on this dramatic case.

What is this patient teaching us?

I often tell my students that Hughes syndrome is first and foremost a neurological disease. The two organs seemingly most sensitive to ‘sticky blood’ are the placenta in pregnancy and the brain. Almost any ‘neurological’ feature can be seen in Hughes syndrome, ranging from the common – migraine, memory loss, ‘funny turns’, TIAs and balance problems to the less common – ‘atypical’ multiple sclerosis, stroke, visual disturbance, seizures and finally to the relatively rare – tics, movement disorders and even Tourette’s syndrome.

And treatment? Our original description of antiphospholipid syndrome in the early 1980s included half a dozen patients with chorea (St Vitus’ dance), a condition in which slow, writhing movements of the arms (‘Thai dancing’) are prominent.

Interestingly, in all cases, the chorea ceased when anticoagulation was started, (usually for clotting elsewhere), suggesting that a ‘sludging’ of the blood might have been responsible. Thinning of the blood worked wonders in these patients.

Professor Graham Hughes

The London Lupus & Rheumatology Centre

London Bridge Hospital