Lupus and the Brain
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 to distinguish between these two forms of brain disease; the former requires immunosuppressive treatment, the latter urgently requires anticoagulants.
Neurological abnormalities present in approximately 40-50% of those with lupus. Obviously this depends on presenting features and investigations. It is recognised that the figure is probably far higher if one includes the more ‘subtle’ forms of brain disease such as mood disturbances and agoraphobia. Some of the main brain manifestations are listed below.
HeadachesThese 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.
Fits and seizuresOccasionally 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 occur in isolation. In the vast majority of lupus patients, once the disease is brought under control there is no further risk of seizures.
Movement disordersOccasionally, 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.
Spinal cord diseaseThis is a rare manifestation of lupus and is more associated with antiphospholipid syndrome.
Memory lossThis 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 such as Mini Mental scores can be conducted to see if there has been serious change in memory. This is particularly important in those patients with the Antiphospholipid 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.
InvestigationsIn 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 an MRI scan 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.
TreatmentIf the brain disease is thought to be due to APS then anticoagulants 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. Doses are reduced as quickly as possible depending on severity of symptoms and potential long term damage. For severe cerebral lupus the addition of an immunosuppressive such as cyclophosphamide is extremely helpful and reduces the severity of 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.