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 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 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
(antiphospholipid) 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 MRI
scan or a CT scan).
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 also an important feature of the
antiphospholipid syndrome (Hughes syndrome). 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.
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.
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.
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
Firstly, it is important to realise that brain involvementajority 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
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 anti-depressive
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.