LUPUS: A Guide for Nurses
Like almost every other organ the brain can be involved in lupus. Recently it has been recognised that much more subtle forms of brain disease (depression etc) are commoner in lupus than was previously thought.
If one thinks about it logically, if the brain is inflamed or affected in any way, it has only limited ways of reacting - headaches, depression, speech disturbances etc - and all of these can be seen at some stage in different patients attending a lupus clinic.
In simple terms there are two main types of brain involvement in lupus: that of generalised lupus and the clotting problems associated with the antiphospholipid syndrome. It is vital for the physician to distinguish between these two forms of brain disease; the former requires treatment for the lupus, the latter urgently requires blood thinning agents - anticoagulants.
Most large studies of lupus put the percentage at around 50%. Obviously this depends on how hard the physicians look for brain disease. It is recognised that the figure is probably far higher if one includes the more "subtle" forms of brain disease such as mood disturbances, agoraphobia etc. Some of the main brain manifestations are listed below.
These are a very common feature of lupus and often antedate the diagnosis by many years. They may be generalised and occasionally are migrainous with flashing lights and vomiting. While they often improve when the lupus is adequately treated, in some patients the headaches prove a resistant problem to conventional lupus treatment.
Occasionally lupus starts in a very dramatic way with a seizure or fit - especially in teenagers, and especially where there is a high fever. In some patients the seizures are prolonged and in others they are a "one off" event. In the vast majority of lupus patients, once the disease is brought under control there is no further risk of seizures.
Occasionally, patients present with a movement disorder or tremor. One of the more well known is chorea (St Vitus Dance) and in these patients the disease is often wrongly diagnosed as "rheumatic fever".
This is a rare manifestation of lupus and is more associated with the "sticky blood" antiphospholipid (Hughes) syndrome.
This is one of the most important features of the disease and is often not picked up early by the patient or physician. For most people it is difficult to know when memory loss is "normal ageing" or when it is abnormal. In some hospitals, formal psychometric analysis is made on patients to see if there has been serious change in memory. This is particularly important in those patients with the antiphospholipid (Hughes) syndrome when memory loss may be the first and major feature of very small blood clots affecting the brain. In these patients there is often dramatic improvement when treatment is started with anticoagulants.
These are described in 'Psychiatric Problems in Lupus' in detail.
In addition to the usual blood tests to assess lupus disease activity, other tests of brain function may be required. The most important of these is the brain scan (MRI) which helps to distinguish brain clots from other causes of brain involvement. Occasionally an EEG (an electrical test of brain activity) is required. Where there are severe brain problems or real diagnostic difficulties a lumbar puncture may be required to exclude a secondary infection in the brain and spinal fluid.
If the brain disease is thought to be due to "sticky blood" then anticoagulants (usually warfarin or aspirin) are started immediately. If the disease is thought to be part of a generalised lupus flare then an increase in steroids (e.g. to maximum of 60mg daily) for a short period is generally used. Recently it has been suggested that for severe cerebral lupus the addition of an immunosuppressive such as cyclophosphamide is extremely helpful and cuts short the duration of the brain disease.
Apart from those patients who have suffered multiple strokes where there may be irreversible brain disease, the prognosis of brain involvement in lupus is excellent. Even in those patients who present with a very extreme form of brain disease such as schizophrenia or seizure, total recovery can be expected with treatment and a return to normal lifestyle predicted.