Lupus and the Brain
serious and dramatic, such as psychosis.
It is very important to remember lupus patients can suffer from ordinary tension headaches and migraine just as anyone can. Depression and other emotional changes can develop but are often a reaction to being ill, rather than being directly caused by autoantibodies.
In general terms there are two main types of brain involvement in lupus: that of generalised lupus, and that of clotting problems associated with antiphospholipid syndrome. It is vital to distinguish between these two forms of brain disease; the former requires immunosuppressive treatment, the latter anticoagulants.
Some level of neurological abnormalities present fairly commonly in patients with lupus. 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:
HeadachesGeneral headaches are a very common feature with lupus but are rarely due to the lupus condition itself. They may be generalised and can be migraineous with flashing lights and vomiting. While they can improve when the lupus is adequately treated, in some patients the headaches prove a resistant problem to conventional lupus treatment. Lupus patients can also of course experience symptoms unrelated to their disease such as headache and migraine, which occur at a similar level as occurs in the general population. However, we always assume there could be a link until things are carefully investigated if the headaches are persistent and limiting activities. This reassurance of nonconnection between lupus and the most common ordinary headaches and migraines can be invaluable to the concerned patient.
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 patients with lupus, once the disease is brought under control there is no further risk of seizures.
Memory lossThis is an important feature of the disease, should not be overlooked, and is understandably worrying to the patient. For most people it is difficult to know when memory loss is part of normal ageing, due to poor sleep or when it is abnormal. In some hospitals, formal psychometric analysis 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 may be dramatic improvement when treatment is started with anticoagulants.
For those who have been investigated and without red-flag signs, without a clear root cause, improving sleep and sleep patterns, increasing exercise gently sometimes with physio guidance, and improving general mood can bring significant improvement.
Change in MoodSometimes, and by getting to know your patients over time, a patient’s approach to decisions and life events can be an indicator that their SLE is more active, when observed alongside objective SLE blood tests. Depressive moods, unusual decisions, and lack of enjoyment in previous activities can all be pointers to brain changes. But these must be interpreted with objective bloods tests, such as decreasing complement (C3 &/or C4), and rising double-stranded DNA (dsDNA) to be attributable to lupus. Alongside family members, nurses in contact with patients in regular appointments and between their appointments can be the first to notice changes, so do raise these observations with appropriate colleagues when pertinent.
Movement disordersOccasionally patients can present with a movement disorder or tremor but this is uncommon. One 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, presenting with weakness and loss of sensation, usually in both legs and without back pain or sciatica. It may be associated with antiphospholipid syndrome, and as with any significant symptom it needs careful management, especially as this can be a life-changing condition. Disturbance of bladder and/or bowel function is not common, but when present as well indicates severe disease and is a medical emergency requiring urgent admission that day for investigation to establish the cause and the best treatment.
InvestigationsIn addition to the usual blood tests to assess lupus disease activity, other tests of brain and spinal cord function may be required. The most important of these is an MRI scan which helps to distinguish clots from other causes of neurological involvement. An EEG (electroencephalography, an electrical test of brain activity) is required for suspected seizures. 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 or subarachnoid haemorrhage.
TreatmentIf the brain disease is thought to be due to APS then anticoagulants are used, though when these are started depends on the presentation. If the disease is thought to be part of a generalised lupus flare then an increase in steroids for a short period is generally used (e.g. to maximum of 60mg orally daily, or an intravenous pulse treatment). Doses are reduced as quickly as safely 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 can reduce the severity and 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 occur with treatment and a return to normal lifestyle predicted.