May 2007: GPs have pivotal role in managing MS
When should GPs suspect MS?
What is the natural history of MS?
What treatments are available for MS?
When should GPs suspect MS?
What is the natural history of MS?
What treatments are available for MS?
Multiple sclerosis (MS) is a chronic inflammatory and degenerative disease of the CNS that often presents in young people. There are different subtypes of MS. The most common is relapsing–remitting MS.
The NICE guideline advises that the diagnosis of MS should be made by a doctor with specialist neurological experience.1 However, patients usually first present to their GP with neurological symptoms. The difficulty in diagnosing relapsing-remitting MS is that the key criteria require evidence of dissemination in time and space, and these are often not evident when patients initially present to doctors.
A common presentation is a clinically isolated syndrome. This indicates inflammatory pathology localised to a single area of the CNS. It may or may not be followed by a further episode, which is necessary for a definite diagnosis of MS. The time course of symptoms is characteristic. Patients tend to deteriorate over days, remain at a nadir for a week or two, and then recover over weeks. Recovery is sometimes incomplete.
Examples of common presenting syndromes are:
• Optic neuritis: usually unilateral pain on eye movements followed by visual blurring or visual loss of variable severity. Optic atrophy commonly ensues. An MRI can help to predict the risk of developing MS. Study findings vary, but if brain lesions are present the risk of developing MS is 60-90% at 10-14 years. The risk is around 20% at 10-14 years if brain lesions are not present
• Transverse myelitis (spinal cord syndrome): combinations of numbness and weakness in the arms, legs or trunk associated with urinary or bowel symptoms
• Brainstem syndrome: combinations of unsteadiness, vertigo, nausea, double vision, trigeminal neuralgia, cranial nerve palsies, internuclear ophthalmoplegia (slow or incomplete adduction of one eye with nystagmus in the other) or cerebellar signs
• Sensory symptoms: a sustained episode of numbness or pins and needles anywhere. Sensory loss in MS may occur in a variety of patterns depending on the nature of the central tract involvement, which is sometimes patchy. It is not typically dermatomal. The time course of the sensory loss, worsening over days and recovering after weeks, can be helpful in diagnosis
• Lhermitte's symptom: an electrical sensation in the back when the neck is flexed, which is indicative of cervical cord inflammation.
In addition, as MS may affect any part of the CNS, more unusual presentations are possible.
When a patient presents with a clinically isolated syndrome it is important to ask directly for a past history of any of the other common symptoms. Patients do not always seek medical attention as their symptoms usually resolve without treatment and they may not link them with their current problem. This is important as a previous history of a neurological episode, if accompanied by residual physical signs in the corresponding location, can provide evidence of dissemination in time and space. This is the key concept in the McDonald diagnostic criteria,2 which were revised in 20053 (table 1, attached).
Patients with syndromes suggestive of MS should be referred to a neurologist for further evaluation. An MRI scan is usually arranged, together with blood tests to help exclude alternative diagnoses (for example, systemic lupus erythematosus, sarcoidosis and Lyme disease).
The diagnosis of MS is predominantly clinical, based on two neurological episodes. The McDonald criteria do allow a diagnosis of MS to be made after one clinical episode if certain MRI criteria are met. Sensitivity and specificity are reasonable in this situation, but not 100%. For guidance on the diagnosis of relapsing-remitting MS, see figure 1, attached.
The need for additional investigations depends on the weight of clinical and radiological evidence for the diagnosis. Lumbar puncture to examine the cerebrospinal fluid for oligoclonal bands is required less often these days, but is sometimes useful, as are visually evoked potentials.4
The diagnosis should usually be made, and the news broken, by a neurologist. The patient should be told that this is a chronic condition with a wide spectrum of severity: for some people it is very mild, for others more serious. While there is no cure at present, MS is a treatable condition. Ongoing support and follow-up should be offered.
A diagnosis of MS is distressing. Written information may be helpful and further information and support can be provided by the MS nurse specialist and organisations such as the MS Society and MS Trust.
Continuing education and support from the MS nursing team ensures that patients are in a position to participate in decision-making about their future management.
Life expectancy is usually normal. Prognosis is variable according to the subtype of MS and disease activity. Many patients remain in work and mobile for many years.
The rules on driving depend on whether the individual has any significant and relevant disability. The DVLA should be informed of the diagnosis and may assess each person with MS on an individual basis.
Flu jabs and travel vaccines
People with MS should be offered immunisation against influenza. There is no evidence that vaccinations increase the risk of relapse.
Measures to ensure a general healthy lifestyle such as a balanced diet and regular exercise are recommended.
A diet rich in linoleic acid is recommended by the NICE guideline1 as it may reduce progression of disability. Linoleic acid is found in sunflower, corn, soya and safflower oils.
Steroids are sometimes used in disabling relapses, but do not improve recovery although they may shorten the duration of the attack.6 The evidence for IV steroids compared with oral steroids is inconclusive.
IV steroids may also be given in an outpatient setting or in the community.
Beta interferons and glatiramer acetate are the mainstays of pharmacological treatment in relapsing-remitting MS. It is generally agreed that these drugs reduce the rate of relapse by around a third7–9 and are therefore indicated for mobile patients with at least two relapses in the previous two years. They are not recommended by NICE because of their relative expense10 but have been made available for prescription by neurologists in an agreement between the drug companies and the NHS. This is known as the risk-sharing scheme. Indications for the three interferon beta preparations and glatiramer are essentially equivalent.11
Common side-effects include flu-like symptoms and injection site reactions, which often settle with time. Full blood count, urea and electrolytes and liver function tests should be regularly monitored. Deranged liver function tests, leucopenia and anaemia may necessitate adjustment or cessation of treatment. Patient choice is important in deciding which treatment is best for individual patients; frequency of injection and side-effect profile are common reasons for preference.
Patients with severe, frequent and disabling relapses resistant to standard treatment are sometimes prescribed mitoxantrone in the specialist setting. Mitoxantrone can be useful in specially selected patients but there is a risk of serious side-effects.12 Natalizumab may also be useful in this situation in the future. It has been shown to reduce relapses by around 60%,13 but in treatment trials there was a small but serious risk of progressive multifocal leucoencephalopathy when it was combined with interferon.
Patients often enquire about complementary therapies, including reflexology and massage, fish oils, magnetic field therapy, neural therapy, massage plus body work, t'ai chi and multimodal therapy. It should be explained that while there is some evidence that they might help some patients, it is insufficient to make a more firm recommendation.1,5
Progressive forms of MS
The natural history of MS is variable. Some patients with relapsing-remitting MS run a benign course, accumulating minimal disability even after many years.14 This appears to be more common in young women, with fewer relapses and long remissions early in the course of the disease and complete recovery in between. In addition, optic neuritis rather than weakness at onset seems to be a predictor of a better prognosis.15 However, after ten years of relapsing-remitting MS around 50% of patients will have converted to a pattern of secondary progressive MS.1 Sometimes patients with established secondary progressive MS have superimposed relapses. Progression is diagnosed clinically by observation of increasing disability over six to twelve months. Disease-modifying treatments are sometimes given if relapses are still prominent. There are no established treatments other than physiotherapy for the progressive phase of the condition, in which inflammation is less prevalent and neurodegeneration predominates.
Around 10–15% of patients with MS present with a different clinical picture of steady deterioration from the onset. This is called primary progressive MS and it has a worse prognosis than relapsing-remitting MS.16 Occasionally patients with primary progressive MS can have relapses too. The diagnosis of primary progressive MS is based on a year of steady deterioration of neurological symptoms, most often a progressive paraparesis (see table 1,attached).
Managing chronic illness
Common symptoms of chronic MS are listed in table 2, attached. The key to management is a multidisciplinary approach with teamwork between GPs and neurologists, physiotherapists, occupational therapists, dietitians, social workers, speech and language therapists, specialist nurses and others. Depression, fatigue and pain are common and should be actively sought and addressed. Some of the symptomatic treatments that are sometimes used are shown in table 2, attached. Patients with complex needs should be considered for referral to rehabilitation services.17 The carers of patients with MS may require support and their needs should not be forgotten, including the possibility of respite care for patients with advanced disease.
There is no evidence that pregnancy influences the overall course of the condition over time. There is a slightly reduced risk of relapse during pregnancy and a slightly increased risk in the puerperium.1 Parents should be reassured that the risk of their children developing MS is minimal; while larger than the risk in the general population, it is still very small.18
Disease-modifying therapy is contraindicated in pregnancy and the risks and benefits of withdrawal should be discussed on an individual patient basis.
Patients with MS have a chronic condition requiring multidisciplinary input. The GP's role is crucial in recognising and referring patients with syndromes likely to be caused by MS, in maintaining and helping monitor treatment, and in supporting and coordinating ongoing care for patients with this chronic disabling condition.key points MS Key points
key pointsTable 1: Revised McDonald Criteria for multiple sclerosis Figure 1 Figure 1: Relapsing-remitting MS
Figure 1Table 2 Table 2: Common symptoms in MS: assessment and management recommendations
Table 2Useful information
The Multiple Sclerosis Society provides information and support for patients and clinicians.
Helpline tel: 0808 800 8000
The Multiple Sclerosis Trust is an independent charity with information for patients, carers and clinicians.
MSdecisions.org.uk is a website funded by the DH providing advice to patients regarding their choice of therapy.
Dr Thomas M Jenkins
clinical research fellow
Professor Alan J Thompson
MD, FRCP, FRCPI
Head of department, Department of Brain Repair and Rehabilitation, Institute of Neurology, Queen Square, London