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The brain



The brain is a miracle machine. And yet in some ways, it is remarkably stupid. If interfered with (eg, poor blood supply or inflammation) it has fairly limited ways of complaining - headaches, memory loss, depression, strokes, seizures - features which are shared with a whole host of other conditions. And yet the problems (as well as possible solutions) can be looked at in a fairly simple way.


Key points

  • Brain involvement in lupus takes many forms

  • Symptoms include: migraine, depression, memory loss, seizures

  • Brain problems often due to 'sticky blood'

  • Can be treated successfully


In lupus there are two main threats to organs such as the brain: inflammation (direct attack by lupus) and poor circulation (sludging or stickiness of the blood in patients with antiphospholipid antibodies and Hughes syndrome). Clearly, different treatments are needed in the two situations - steroids and anti-inflammatory medicines in the first, and anti-clotting agents (eg, aspirin, warfarin, etc.) in the second. Arguably the biggest advance in lupus in recent years has come from the observation that much of the brain problem in lupus comes from sticky blood, which is potentially very treatable.


The outlook or prognosis for brain disease, provided that the danger of blood clotting is recognised and dealt with, is surprisingly good. We have many patients who have had a distressing past history of depression, memory problems, fits, phobias and even psychosis, who are now leading completely normal lives, free of neuro. psychiatric symptoms.


Anatomy

Medical students learn about the central nervous system, comprising the brain and spinal cord (the computer), and the peripheral nervous system (the wiring to the far flung areas) from the scalp to the toes, and doctors are trained to distinguish between these vitally different areas. However, all families who have looked after relatives with stroke, for instance, know the broad outline of brain problems which will depend on the site of the damage. For example, speech problems or a stroke indicate one-sided weakness, balance problems (the cerebellum), leg weakness and bladder disturbance (the spinal cord), and so on. Signs pointing to peripheral nervous system problems include numbness, pins and needles, formication (an odd name derived from ants where there is a feeling of crawling sensations under the skin), and the finding by the doctor of absent (or impaired) reflexes with the tendon hammer.


Obviously, it is the highly complex brain which gives rise to the huge variety of features, ranging from absences, to panic attacks, depression, and memory problems.


Migraine and headache

Headache has long been considered a common problem in lupus.

A big breakthrough came with the discovery of antiphospholipid syndrome. We now recognise that by far the most important cause of headaches in lupus patients is sticky blood. Many lupus patients have a history of teenage migraine, often going away for years before returning. It is absolutely critical to test for Hughes syndrome (aPL testing). One of the most satisfying results in lupus is the improvement and even disappearance of headache (and memory problems) when anti-clotting medicine is started.


Stroke and transient ischaemic attack (TIA)

The most feared complication of lupus is stroke or, its early warning cousin, TIA. The latter is a related but less severe event involving a period of difficulty in speech or movement lasting from seconds to minutes.


We now recognise that, in lupus, stroke (like headache and many of the other conditions discussed in this chapter) is the extreme consequence of clotting due to Hughes syndrome. Early treatment is vital and effective. A patient found to be positive for antiphospholipid antibodies (aCL and LA positive) and especially with small 'dots' on the brain MRI scan should be treated urgently with anticoagulants such as heparin or warfarin. If treatment is fast and effective, the patient is spared any worsening of what could be a clotting disaster.


Depression

Depression, in all its forms, is an important part of lupus and is often under-recognised. Too often, mild depression is attributed simply to the stress of having a chronic illness (eg, fatigue, joint pains). But in many cases, the depression can dramatically improve if the underlying lupus is recognised and treated successfully. Often it is difficult for doctors and patients alike to dissect out a depression component from all the other features of the disease. The decision of whether to add anti-depressant to the overall lupus treatment is a difficult one and requires experience on the doctor's part.


Psychosis

Very rarely, the brain involvement in lupus leads to more severe brain disturbance, including psychosis. During such an episode the patient may hear voices. The good news is that even in the most severe examples (eg, when the patient has been thought to have schizophrenia), the outcome is usually good. With treatment (usually both lupus treatment and anti-psychotic medication) there is every reason to expect full recovery.


Memory loss

This is a major feature of patients with Hughes syndrome (sticky blood). Given that the brain needs a good circulation to function, it is not surprising that impairment of the oxygen supply leads to a variety of symptoms. Memory loss varies from mild (I have to write everything down) to severe and more frightening (eg, one patient could not remember which exit of the traffic roundabout led to her home). One of the truly dramatic advances in lupus has been the memory improvement in patients with lupus and Hughes syndrome when treated with anti-clotting agents such as aspirin, heparin or warfarin.


Balance and hearing

We now recognise that many of the neurological features of lupus (eg, balance problems, seizures and atypical multiple sclerosis) are closely linked to antiphospholipid syndrome (Hughes syndrome) and may well respond better to anti-clotting medicines than to steroids.


Balance problems are usually due to circulation impairment in the middle ear. Not surprisingly, ringing in the ears (tinnitus) is sometimes a feature.


Vision disturbances

Vision problems range from focusing difficulty to loss of vision. There are many causes: for example, drugs such as steroids can affect the pressure in the eye and alter focal length. Alterations in blood pressure and circulation to the eye can also lead to a variety of symptoms. One of the most dramatic (and potentially treatable) symptoms is sudden loss of part or all of the field of vision, secondary to blood clotting in Hughes syndrome.


Multiple sclerosis

A number of our patients with lupus and Hughes syndrome who have features such as balance disorder, pins and needles, and visual disturbance are, not surprisingly, labelled as having possible multiple sclerosis (MS).


Distinguishing the two conditions can be very difficult but vitally important, and it is one of the main clinical research projects in our unit.


St. Vitus' dance

Medically known as chorea, this jerking movement of the head, arms and body, is one of a variety of movement disorders seen in lupus and Hughes syndrome. Neurologists recognise a wide spectrum, ranging from periodic 'tics' through to cases resembling Parkinson's disease.

Seizures

Occasionally lupus is characterised by the dramatic development of fits or seizures. Again, it is now recognised that this brain disturbance is often associated with sticky blood and can respond to anti-clotting drugs. This observation is having a profound effect on the subject of neurology in general. For example, one recent study suggested that up to 20% of all cases of unexpected teenage epilepsy tested positive for antiphospholipid antibodies!


Undiagnosed lupus

From the above, it is obvious that brain involvement in lupus and Hughes syndrome takes many forms. There are, without doubt, a number of patients out there with memory loss, or seizures, or the symptoms of MS, for example, in whom lupus or Hughes syndrome is the underlying cause. So treatable! So important!

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