Palliative Care in Neurological Disease

A Team Approach

Judi Byrne, Penny McNamara, Jane Seymour, Pam McClinton

• Publisher: CRC Press
• Year: 2021
• ISBN:
  9780429533457
• Edition: 1
• Topic: Medizin
• Subtopic: Anästhesiologie & Schmerztherapie

The particular needs of people with progressive long-term neurological conditions - including Parkinson's Disease, Motor Neurone Disease (MND), Multiple Sclerosis (MS) and Huntington's disease - may make care delivery and planning the last stages of life ethically, practically and clinically challenging.

CHAPTER 1 Progressive neurological conditions: epidemiological and clinical picture

DOI: 10.1201/9780429091438-1

Francesca Crawley

There are many different progressive long-term neurological conditions (PLTNCs). This chapter focuses on multiple sclerosis (MS) and Parkinson’s disease (PD), which are two of the more common conditions, and on motor neurone disease (MND) and Huntington’s disease (HD), which are much rarer. In the UK, each general practitioner will have an average of three people with MS on their case load, but only a one in ten chance of a patient with MND.

Reference is made in this chapter to the ‘incidence’ and the ‘prevalence’ of each disease. The ‘incidence’ is the number of new cases of the disease developing in a year and the ‘prevalence’ is the number of patients with that disease at any point in time.

MULTIPLE SCLEROSIS

Epidemiology of MS

Multiple sclerosis (MS) is the most common central nervous system disease to affect young adults. It is more common in women than in men (2:1). Disease onset is generally in the third or fourth decade of life.

There is no database of patients with MS in the UK. We base our estimate of the total number of individuals with the disease on local population studies, extrapolated to include the whole country. By using these estimates, there are thought to be about 85 000 people with MS in the UK. Worldwide the prevalence is thought to be around 2 500 000. In the UK we think that about 2500 people are diagnosed with MS each year.¹

People with MS are not distributed evenly throughout the world. The prevalence of the disease increases as you travel further away (north or south) from the equator. For example, in the UK, this means that there are more people with MS per 1000 of the population in northern Scotland than in London. Looking more globally, the incidence is higher in Canada than in an equatorial country such as India.

The risk of developing MS if you move from a low risk part of the world, for example India, to a high risk part of the world, for example the UK, depends on the age at which you migrate. If you move as a child, your risk will become similar to that of others living in the country to which you move, but if you move as an adult you maintain the level of risk from your native country. This shows the interaction of genetic and environmental factors on the risk of developing this disease.

In general in the UK, the risk of developing MS is about one in 700. In addition to this risk varying with where in the UK an individual lives, it is also increased by having family members with the disease. If you have a first degree relative (parent, sibling, child) with MS, your risk of developing the disease is one in 40, and if you have a second degree relative (cousins, aunts/uncles, nephews/nieces) it is one in 100.² MS is a progressive neurological disorder which people often live with for many years. Overall, an individual’s life expectancy is only slightly shortened by MS, although this varies greatly from one individual to another.

The precise environmental factors which trigger MS remain largely unknown and multiple studies have had conflicting results. Possible triggers which have been identified include age at infection by Epstein Barr virus and climate induced changes in vitamin D levels. None of the theories are proven.

Pathology of MS

It is thought that MS is caused by the body’s own cells attacking cells within the central nervous system (CNS). This is called an autoimmune reaction. The attacking cells are a type of white blood cell called T lymphocytes. These T lymphocytes enter the CNS and attack the myelin which surrounds the cell. This is called demyelination. When myelin is damaged the nerve impulse cannot pass so easily down the nerve cell. This means that messages cannot get through to the correct place. For example, if there is damaged myelin between the pathway from the brain to the legs, walking is likely to be difficult. If the myelin is damaged between the brain and the eye, vision will be affected.

In the early stages of MS, remyelination can occur. This means that the damaged myelin can be repaired. However, the repaired myelin is often thinner and does not conduct the nerve impulses as well as the original myelin did. Over time however, the cells that produce myelin (oligodendrocytes) become damaged and myelin is not replaced. The nerve which was protected by the myelin (the axon), then dies. If just a small part of the axon dies, the CNS is able to reroute messages, however, if it is a larger area of damage the rerouting can not happen and the pathway is permanently blocked.

Types of MS

Most people with MS have relapsing remitting MS at the onset of their disease. This means that they develop a neurological problem which persists for a while (usually a few weeks; the relapse) and then recover (the remission). It is easy to understand what is happening pathologically.

In the relapse, there is demyelination. Once remyelination has occurred there is a remission from the symptoms. For the purpose of clinical trials, a relapse has been defined as lasting more than 24 hours in the context of a normal body temperature and that to consider attacks to be separate they should be at least 30 days apart.³

The severity, the frequency and the gap between relapses is hugely variable and impossible to predict. Most people with relapsing remitting MS have one or two relapses a year. Over years, this pattern of disease may change into progressive MS. Here relapses become less frequent, but disability increases. This is termed ‘secondary progressive MS’.

Studies suggest that people who initially have relapsing remitting MS develop secondary progressive MS after about 10 years.⁴ Other individuals develop progressive MS from disease onset. This is called primary progressive MS. These individuals tend to be older at the time of disease onset, with a mean age of onset about 40 years. A final type of MS is benign MS. Here attacks are mild and often years apart.

Symptoms from MS

Symptoms in MS depend on the part of the nervous system in which demyelination occurs. For example, the optic nerve, which relays visual information from the eye to the brain, is often affected. This is usually only one eye and causes a painful reduction in vision. Colour vision is often particularly affected. Alternatively, demyelination in the balance area of the brain (the cerebellum), causes difficulty walking, slurred speech and problems with coordination. Involvement of the part of the brain that is involved in sensation causes numbness or tingling. If the patient is not seen acutely, these signs have often resolved by the time they are seen in clinic.

Other symptoms from MS include bowel and bladder disturbance, memory problems, depression and personality change. Fatigue is also common, affecting up to 75% of patients.⁵ Many patients rate fatigue as their main problem in MS. This type of fatigue differs from the fatigue experienced in depression. MS associated fatigue is often worse with heat, exercise or later in the day and is often relieved by a short rest. Depression associated fatigue is more non specific and often associated with other depressive symptoms such as sleep disturbance. Many of these symptoms come and go in the initial phases of the disease, but as MS progresses, symptoms and therefore disability, accrue.

Diagnosis of MS

Patients with primary progressive MS usually present with a gait disorder. This has often come on very gradually and the individual has adapted to maintain functional ability. These individuals often have very abnormal neurological examinations at their first presentation in an outpatient clinic.

MS is often suspected by a clinician on the basis of the history and examination of the patient, but investigations are mandatory. Magnetic resonance imaging (MRI) is now the most standard investigation. Imaging of the brain and sometimes the spinal cord is performed. Typically, MS causes lesions in the white matter of the brain, particularly around the ventricles. Gadolinium enhancement is seen with lesions which are less than about 4–6 weeks old. These new enhancing lesions are about 5–10 times more common than the clinical relapse frequency. For this reason, they have been used as surrogate markers in clinical trials. In the right clinical context these can be diagnostic, but if there is any doubt a lumbar puncture is performed to examine the cerebrospinal fluid for the presence of oligoclonal bands. These can be found in other neurological diseases other than MS but up to 95% of patients with clinically definite MS will have them.⁶

Electrical tests (evoked responses) are occasionally done. These involve examining the conduction time in a nerve or nerves. Demyelination causes slowing of the impulse and thus a prolonged conduction time. Blood tests are required to exclude other conditions which may mimic MS.

Initial treatment of MS

The initial management of MS involves explaining the diagnosis to the patient. It is desirable that this is done by a member of the neurological team who has enough experience to be able to answer any questions accurately. Many patients find early involvement with an MS nurse to be of great benefit. Some MS centres run courses over a series of evenings to introduce patients and relatives to the nature of MS and to educate them about different aspects of the disease.

Initially, relapses are often treated with steroids. These are increasingly given as oral tablets (methylprednisolone). Methlyprednisolone speeds up recovery from an individual relapse, but does not alter the final outcome. There is no effect on long-term disability. Various studies have shown a role for disease modifying drugs in the management of MS. These include interferon,^7,8,9 CAMPATH, natalizumabl¹⁰ and mitoxantrone.¹¹These drugs are used in an attempt to prevent disease progression: both relapse rate and disability. In the UK, they are available via specialist MS clinics; sometimes via clinical trials. The ideal treatment would stop relapses, halt disease progression and reverse any disability: unfortunately we do not yet have such a drug.

Symptomatic treatment is also commonly employed in MS. For example, pain may be managed with conventional painkillers such as paracetamol or anti inflammatory drugs, or with less conventional drugs, such as anticonvulsants and antidepressants. Bladder involvement is often best managed by a continence advisor, often in the community. This may involve drugs or sometimes catheterisation. For some individuals self catheterisation can be done a couple of times a day, thus draining the bladder effectively but avoiding a permanent catheter.

Depression is often treated with antidepressant tablets and with psychological support, if this is available locally. Fatigue can sometimes be helped with medication, but is often best managed by a more pragmatic approach. In such an approach, education of all parties (patient, carer, and employer) to maximise flexibility and to pace day to day activities can be beneficial. For example, an afternoon sleep may help tremendously, but some patients need encouragement to take this. Similarly, an employer may agree to this if the importance and benefit is explained.

At many stages of MS, the involvement of the specialist therapist, including physiotherapists, occupational therapists and speech and language therapists is tremendously important. For example, a physiotherapist may be able to preserve independent walking and at another stage in the disease may offer advice on a wheelchair. Occupational therapists may look at adapting an individual’s home, to enable him or her to live there more safely. Speech and language therapists are often involved in assessing swallowing.

MOTOR NEURONE DISEASE

Epidemiology of MND

In...