Sjogren's Syndrome
NEUROLOGICAL PROBLEMS IN SJOGREN’S SYNDROME
by Dr Annabel Coote and Dr Michael Snaith,
Department of Rheumatology, Royal Hallamshire Hospital, Sheffield
It is known that certain neurological complaints occur more frequently amongst people with Sjogren’s syndrome than the general population.
The neurological (or nervous) system includes the brain, spinal cord and nerves. It has been estimated that fewer than one in five Sjogren’s syndrome sufferers will develop neurological symptoms and it is important to remember that these are mild and/or transient.
Sjogren’s syndrome is classified as being either primary or secondary. Secondary disease accompanies illnesses such as rheumatoid arthritis, lupus, scleroderma or polymyositis. Your doctor will have told you if you have one of these conditions. Primary Sjogren’s syndrome occurs in isolation. When people with the secondary type develop neurological symptoms it can sometimes be difficult to tell whether these are due to the Sjogren’s or to the associated illness.
I will now explain how different parts of the nervous system can be affected.
The nerves
The nerves, which supply power and sensation to the body, sometimes do not work as effectively in Sjogren’s syndrome. This can produce various problems:
Carpal tunnel syndrome
A single nerve is~affected at the wrist producing pins-and- needles, numbness, pain and sometimes weakness in the hand. These symptoms occur mainly in the thumb, index and middle finger. They are often worse at night. Carpal tunnel syndrome occurs more frequently in people whb have Sjögren’s syndrome associated with rheumatoid arthritis.
Electrical tests are often used to confirm the diagnosis. Carpal tunnel syndrome can be treated with steroid injections into the wrist. Sometimes surgery is required. This is a straightforward operation that can be performed under local anaesthetic. If left untreated, the muscles around the base of the thumb can waste, resulting in worsening of hand function.
Peripheral neuropathy
Neuropathy means any disease of the nerves. This is less common than carpal tunnel syndrome. The peripheral nerves that serve the extremities (e.g. hands and feet) fail to function normally. This is usually due to vasculitis, which is inflammation of the tiny blood vessels that nourish the nerves. Some cases are possibly due to inflammation of the nerves themselves.
The symptoms are of numbness and tingling which usually start in the feet and can progress very slowly up the legs. There can be associated muscle weakness. A similar peripheral neuropathy can occur in other conditions such as diabetes, and it is important for doctors to exclude other causes. Although peripheral neuropathy can progress, this is usually very slow and it can stabilise. Electrical tests are used for diagnosis. Treatments include steroids and immunosuppressant drugs such as azathioprine.
Cranial neuropathy
This is involvement of one or supply the head and neck. By far the commonest nerve affected is the trigeminal nerve. This supplies sensation to the face and powers the muscles of the jaw. Trigeminal neuropathy causes symptoms such as numbness, pins-and-needles or, rarely, pain. These symptoms are usually well localised affecting either an area of skin around one eye, the cheek or the chin. Trigeminal neuropathy often resolves spontaneously. If it does not, various drugs can help the discomfort.
Very rarely, cranial neuropathy can affect the optic nerve. This causes disturbance of vision in one eye which is usually transient.
The brain and spinal cord
The brain and spinal cord are a lot less likely to be affected by Sjogren’s syndrome than the peripheral nerves. In the past doctors have studied groups of people with Sjogren’s syndrome and found that some have developed symptoms such as weakness of one or more limbs, fits or double vision. It is very important to remember that strokes, epilepsy and multiple sclerosis are, unfortunately, not uncommon amongst the general population and Sjogren’s syndrome sufferers are no different. Therefore if we were to follow a big group of apparently healthy people, inevitably we would find that a few developed each of these conditions. Rarely, Sjogren’s syndrome can mimic these illnesses, perhaps by causing inflammation of blood vessels within the brain. The symptoms that I described above are therefore slightly more common in people with Sjogren’s syndrome, but often settle spontaneously without causing permanent disability.
Several investigations are used if brain or spinal cord disease are suspected. Clear pictures of both these structures can be obtained using a MRI scanner. This involves lying on a bed that is passed through a tunnel. Most people do not find this a problem, but some who feel a bit claustrophobic choose to take a sedative tablet beforehand. It can be helpful to analyse the fluid which bathes the spinal cord and brain. We perform a lumbar puncture to collect a small sample.
This is a simple procedure. With the patient lying on his/her side, we freeze the skin at the base of the spine and pass a tiny needle between two of the vertabrae. Blood tests also have an important role in investigating neurological symptoms. They can be particularly helpful in excluding other causes. Patients who are thought to have inflammation of the brain or spinal cord that isn’t settling spontaneously need treatment. We use drugs such as steroids and immunosuppressants which can be given in tablet form or in a drip.
Summary
In summary, neurological problems affect a minority of people with Sjogren’s syndrome and are often mild. There are treatments available for more troublesome or persistent symptoms.
Sjogren's Syndrome : An Advisory Guide for Patient's and Doctors : £2.50
This booklet is produced by BSSA and is an invaluable source of information on Sjogren's syndrome both to newly diagnosed patients and also G.P.s, junior hospital staff, dental surgeons and nurses who will find the contents helpful in understanding the problems of paitients with the disorder.
Note:
WebHealth has additional articles about Sjogren's Syndrme at:
Systemic Features By:
Dr Ian Griffiths, Dr Elizabeth Price, Dr Clive Kelly and Dr Patrick Venables
and
The Problems of Sjogren's Syndrome By:
Dr CW Hutton, Consultant Rheumatologist, Demford Hospital, Plymouth
and
Sjogren's Syndrome and the Gastro-Intestinal Tract By
Doctor Richard Mount Ford MD, FRCP, FRCR.
Consultant Physician, Department of Gastroenterology, Bristol Royal Infirmary
and
Neurological problems in Sjogren's Syndrome by Doctor Annabel Coote and Doctor Michael Snaith, Department of Rheumatology, Royal Hallamshire Hospital, Sheffield.
and
Sjogren's Syndrome and the Respiratory System
A Brief History of Sjogren's Syndrome By
Doctor Ian D Griffiths FRCR, Consultant Rheumatologist,
Freeman Hospital, Newcastle-Upon-Tyne
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