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You are here: Home / Articles / APS: What Rheumatologists Should Know about Hughes Syndrome

APS: What Rheumatologists Should Know about Hughes Syndrome

February 17, 2016 • By Graham R.V. Hughes, MD, FRCP

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The ramifications of these observations are many. Let me give two clinical examples:

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February 2016

Firstly, temporal lobe epilepsy in APS may be under-recognized. One of my patients, a woman with classical APS, became an EEG technician. During her training she used four of her siblings as guinea pigs. Three had abnormal temporal lobe activity. It’s interesting to see how often a family history of epilepsy crops up in the history taking of patients with APS.

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The second clinical anecdote has been published elsewhere.22 A 42-year-old patient had a past history of mild lupus. However, her major problem—and one with a significant impact on her busy life—was recurrent seizures, both petit mal and grand mal, the latter requiring specialist care and the combination of a number of anti-epileptic drugs. During her stay in London, she developed a DVT (she was found to be aPL positive), and routine anticoagulation with warfarin was started. An immediate and unexpected bonus was a marked reduction in the frequency and severity of the seizures, requiring far less aggressive anti-epileptic treatment.

‘THE STILLBIRTH SCANDAL: Every day as many as three babies in the UK are stillborn who could have lived. One in 200 births to British women will be stillborn. It is time for the NHS to adapt new policies and a new attitude.’ —The Times (London), Nov. 28, 2012

Memory Loss

Possibly the commonest manifestation of APS is memory loss. Often, the problem only sees the light of day when the patient is asked about the symptom. So many patients confess to worries about Alzheimer’s. To date, detailed clinical studies are few and far between. From time to time, and, it must be said, in a fairly desultory fashion, we have carried out psychometric testing on selected APS patients—and the results have often been surprising. Even more so, given the striking improvement seen in memory tests when anticoagulation is commenced.23

Multiple Sclerosis

In view of the prominence of neurological features, including visual disturbance and fluctuating myelopathy, seen in APS, it is not surprising that a number of cases are diagnosed as MS.24,25

The subject is far from straightforward. Some in neurology declare that positive aPL tests are, in fact, sometimes seen in classical MS. I would prefer to turn the picture around. Such patients, at least some, may be APS first and MS second. Although anecdotally a number of such cases respond to anticoagulation, in others the response is, at best, borderline. Uthman has recently reviewed the fairly substantial literature on APS vs. MS.26

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Other Neurological Features

These include balance problems (a number of cases presenting as Meniere’s), chorea (sometimes severe),27,28 anosmia, sleep disturbance (including cataplexy and narcolepsy) and, recently, autonomic disturbance with a series of cases of POTS (postural orthostatic tachycardia syndrome).29

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Pages: 1 2 3 4 5 6 7 8 9 10 11 | Single Page

Filed Under: Conditions, Systemic Inflammatory Syndromes Tagged With: Antiphospholipid Antibody Syndrome (APS), brain, Clinical, Diagnosis, Hughes Syndrome, joint, patient care, pregnancy, rheumatologist, stroke, symptom, thrombosisIssue: February 2016

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