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Doctors throughout
the world are now recognising the importance of subtle forms of
brain involvement in lupus as well as the more obvious brain problems.
Never underestimate brain involvement. It may vary from mild depression,
to memory loss, to much more severe problems such as seizures.
In general there are two main causes of brain disease in lupus.
The first is lupus disease itself which can cause alterations in
the brain activity. The second is the clotting disorder associated
with some lupus patients, the antiphospholipid or Hughes syndrome.
It is very, very important for the doctor to try to distinguish
between these two major causes of neurologic involvement as the
treatments are clearly very different.
Depression
Depression is an important manifestation of lupus - in some it
is the presenting sign of the disease. Many patients and, certainly,
many doctors wrongly attribute depression in lupus merely to having
a chronic illness and all that goes with it. This is not correct.
The disease itself causes depression. Depression is an integral
part of lupus in some patients - indeed management of the lupus
often itself lifts the depression. The management of depression
in lupus rests on a combination of treating the underlying lupus
itself as well as possibly adding in antidepressant therapy. One
of the medical advances in the last decade has been the introduction
of newer milder antidepressants with less of the severe side-effects
which so hampered older treatments.
Headaches
Headaches are common in lupus. In some patients a history of headache
going back to their early teens is a feature of the disease. They
may be a part of the lupus itself or may be associated with a clotting
syndrome. They may or may not have a migrainous element with flashing
lights and visual disturbances. In any patient with lupus who suffers
from headaches a systematic search for known causes should be carried
out including blood pressure checking, examination of the sinuses,
examination of the blood for 'sticky blood' and ultimately, if
indicated, a brain scan (either an NM scan or a CT scan).
Fits
Sometimes lupus first starts in the most dramatic way with a
seizure or a series of epileptic fits. This is usually when the
patient is untreated and the disease fairly active. It is sometimes
associated with high fever. Fits or seizures are one of the non-specific
ways the brain reacts to severe illness. Once the lupus is treated
further fits are the exception rather than the rule.
Movement disorders
The same applies to movement disorders. Occasionally patients
develop chorea (St Vitus Dance) with jerky hand movements or head
movements. This is simply a manifestation of abnormal brain function
and, once again, is often associated with the 'sticky blood' (Hughes)
syndrome.
Spinal chord
Rare, but extremely acute and very dangerous, is spinal cord
involvement, which may lead to permanent paralysis. It is now recognised
that immediate treatment with both steroids and possibly anticoagulants
may reverse this. Fortunately it is a very rare manifestation of
neurologic lupus.
Psychiatric disturbance
During severe lupus flares patients can experience a variety of
psychiatric disorders varying from mild personality disorders to
severe psychotic behaviour. Some lupus patients are wrongly diagnosed
as having schizophrenia at the onset of their illness. Interestingly,
treatment of the lupus in these patients results in total improvement
in the psychiatric features. This is one of the most important
observations to come out of lupus research as it provides possible
insights into other mental disease. Patients with the antiphospholipid
(sticky blood) syndrome suffer memory variants, from subtle ('I
couldn't remember what I had gone into the shop for') to severe
memory loss. Lupus doctors are now beginning to realise how common
and important this aspect of the disease is. Clearly, any patient
who feels that this is a major feature of the disease requires
full neurologic examination, possibly including MRI, as well as
testing for the antiphospholipid syndrome.
Treatment
Firstly, it is important to realise that brain involvement in
lupus is extremely common. Secondly, in the vast majority of patients
there is complete resolution of the problem with time and most
patients get better. If the brain symptoms start dramatically,
for example with fits or severe neuropsychiatric disease, the treatment,
as with most active forms of lupus, is with steroids and immunosuppressive
drugs. The doses of steroids used are less than in the old days
- for example 60mg daily in the majority of the worst cases - rarely
is a higher dose than this required. An alternative way of giving
steroids is by 'pulse' injections on an intermittent basis. This
is becoming more popular as it is a simple and more rapidly effective
way of giving steroids, especially in an emergency.
A separate form of brain involvement in lupus is associated with
Hughes syndrome (the antiphospholipid syndrome). In this form of
the disease the cause is totally different: blood clots or 'sticky
blood'. In patients where this is suspected, brain scans are usually
required. These may show localised areas where brain blood supply
has not been adequate. The treatment in these patients is different
and requires thinning of the blood, either with aspirin or, in
more severe cases, with anticoagulants such as warfarin (coumadin).
For less dramatic brain involvement the choice of treatment in
many ways is more difficult. Many, many patients are not treated
who perhaps should be treated. In some patients the depression
is a major problem and requires conventional antidepressive treatment.
The more modern pills for depression are very superior to older
medications and cause far less side-effects. The opinion of a psychiatrist
or psychologist may need to be sought as to whether medical treatment
is appropriate, especially where there might be dangers of drugs
interacting.
In summary, the vast majority of patients who have brain involvement
can be treated successfully with a full return to normal daily
activities.
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