[MS Revision Tool 2013] This tool is a work in progress but has been designed to support you as you sit the internationally accredited MS Exam

Transcription

1 2013 [MS Revision Tool 2013] This tool is a work in progress but has been designed to support you as you sit the internationally accredited MS Exam

2 Contents Introduction to this resource and how to use it 3 Category 1- Concepts underlying clinical practice 8 Category 2-Assessment and Intervention for Clinical Practice 33 Category 3- Advocacy 40 Category 4 Education 43 Category 5 Research 55 Contact and Feedback 63 2 Page

3 Introduction to this tool This is a guide to support those of you are planning to undertake the Multiple Sclerosis International Examination. This is the first time we have produced this revision aid, so at the current time it is work in progress and will be updated on a yearly basis. It will also be expanded upon as time goes on. There was a lot of hard work and thinking that was put in to developing this tool and various methods of this resource were made before this one was decided upon. You will find that instead of reams of text (which we did produce the first time), we have instead, produced a resource for you that would be always up to date and will go towards providing you with the information and revision that you require to prepare yourself for this exam. We have presented some words as an introduction to each topic, then hyperlinked to other resources for you to read at your leisure. How to make best use of this tool We have structured this tool around the contents of the MS International Examination. For further information on the content of the exam and other useful information concerning its logistics, go to and have a read. category are as detailed below: The questions that are within the Multiple Sclerosis Nursing International Certification Examination fall into 5 different categories, the areas these cover and the weighting within each 1 CONCEPTS UNDERLYING CLINICAL PRACTICE 23% 2 ASSESSMENT AND INTERVENTION FOR CLINCAL PRACTICE 42% 3. ADVOCACY 10% 4 EDUCATION 17% 5 RESEARCH 8% As you proceed through this guide, you will see that we have made major use of hyperlinks to enable you to read and reflect upon up to date, evidenced based material about specific topic areas within the exam. We have used only trustworthy and reputable sites where accurate research based information can be relied upon. If the links don t work directly from the document, just rightclick with the mouse over the link and then click on Open Hyperlink. In general, we have used 4 major internet sites. These are: 3 Page

4 1. MS Atlas requires you to register first before use; it is free of charge to do so, and is well worth it. It uses real life clinical sessions with patients to demonstrate how to recognise and test for symptoms and clinical signs of Multiple Sclerosis (MS).To register please click on: where you will be directed to: In the middle of this screen there is an orange Register box, please click on this and all will become clear. It takes only a few seconds of your time. 4 Page

5 2. UKMSSNA slide library: (UKMSSNA members only) It is not possible to link you directly to each of the sets of slides in question. You will need to be a member of the UKMSSNA of course and log onto the central slide library page, and then find the necessary teaching session that is indicated. There are plenty of slide decks on here which are updated on an ongoing basis. 5 Page

6 3. MS Trust: By now, you will know this website well so I don t need to explain. 6 Page

7 4. Projects in Knowledge: This is a relatively new website that provides you with a constantly updated e based text book of MS. We have linked you to specific pages in question. As you can see from the contents bar on the left hand side of the screen you need to click on Neurology then e text books tab, then MS edition. Within this site there are regular tests of your knowledge if you want to undertake these you can but you don t have to. The questions are often quite difficult and are NOT at the same level the exam questions will be. 7 Page

8 Category 1: CONCEPTS UNDERLYING CLINICAL PRACTICE: This area of care represents 23% of the overall mark. For this category you will need to revise and review the following: A. Definition of Multiple Sclerosis 1. Disease Course and Classifications 2. Epidemiology and Aetiology 3. Disease Trajectory B. Pathophysiology of disease: 1. Disruption of Blood/ Brain Barrier 2. Immune Dysfunction 3. Destruction of Myelin 4. Role of Oligodendrocytes 5. Axonal Damage 6. Nerve Conduction 7. Neuropathology 8. Neuroanatomy C. Diagnosis of multiple sclerosis: 1. Presenting symptoms 2. Diagnosis of a relapse 3. Diagnosis of clinically isolated syndrome 4. Prognostic indicators 5. Diagnostic tests 8 Page

9 Definition of Multiple Sclerosis 1. Disease Course and Classifications It is still impossible to predict the disease course of MS. No two people have exactly the same experience of MS; the disease course may look very different from one person to another. There are commonly recognized classifications of MS which are: Clinically Isolated Syndrome (CIS): first episode of neurological symptoms lasting at least 24 hours. Damage may occur in one place (monofocal) resulting in the experience of a single symptom or more than one place (multifocal) when multiple symptoms might be experienced. If MRI shows demyelinating lesions then chances of having further relapses vary from 30% to 70%. For more information on this, use the MS Trust factsheet go to Radiological MS: this is when incidental evidence of MS lesions is seen on an MRI scan. The person has not experienced any signs or symptoms. Relapsing Remitting MS: characterised by clearly defined exacerbations/relapses of worsening neurologic function followed by partial or complete remissions during which no disease progression occurs. Approximately 85% of people are initially diagnosed with relapsing remitting MS. Primary Progressive MS: characterized by slowly worsening neurologic function from the beginning with no distinct relapses or remissions. The rate of progression may vary over time, with occasional plateaus and temporary minor improvements. Approximately 10% of people are diagnosed with primary progressive MS. Secondary Progressive MS: Following an initial period of relapsing remitting MS, many develop a secondary progressive disease course in which the disease worsens more steadily, with or without occasional flare ups, minor recoveries (remissions), or plateaus. Before the disease modifying medications became available, approximately 50% of people with relapsing remitting MS developed this form of the disease within 10 years. Long term data is not yet available but we hope that current treatments significantly delay this transition. Progressive Relapsing MS: Not so recognized in the UK, relatively rare course of MS (5%), people experience steadily worsening disease from the beginning, but with clear attacks of worsening neurologic function along the way. They may or may not experience some recovery following these relapses, but the disease continues to progress without remissions. 9 Page

10 Variations of MS include Neuromyelitis Optica (NMO) was traditionally known as Devic's disease. For more information on this please use this link: For more information on the different disease classifications of MS: You may also want to link here: of ms.xml imminent? Consider: what is the EDSS threshold that a person with MS reaches when SPMS is 2. Epidemiology and Aetiology: There is considerable variation in the occurrence of MS around the world. This has been ascribed to environmental factors, like exposure to viruses or ionising radiation, or to genetic factors. One factor that is constant is that prevalence rates are higher in places further away from the equator. For more information on this, go to: Age, race and gender play a part in the aetiology of MS. The typical age of onset is There are approximately 2,500,000 people worldwide who have multiple sclerosis with 800,000 living in the UK alone. Research suggests the proportion of women with MS is increasing and it is now accepted that 3.2 women have MS for every man with the condition. Studies in the UK suggest the prevalence rate in England and Wales is between 100 and 140 per 100,000, about 170 in Northern Ireland and 190 per 100, 000 in Scotland. A study of north east Scotland found the level to be 229 in Aberdeen, 295 in Shetland and 402 in Orkney. In the UK studies suggest that 2,500 3,000 people are diagnosed with MS each year, or about a week. Caucasian people are twice as likely to develop MS as non Caucasians. The distribution of MS around the world varies. Generally, the prevalence increases the further away the country is from the equator. Asia, Africa and America that lie on the equator have extremely low levels of MS, whilst Canada and Scotland have particularly high rates. Some ethnic groups show disproportionately low frequencies of MS e.g. Maltese, Innuits, Lapps, Siberians, Hungarian Gypsies, Central Asians and the Maoris of New Zealand. Vitamin D levels are also appearing important in MS management. Vitamin D. Vitamin D deficiency may increase susceptibility to MS; this hypothesis is popular, but not yet substantiated as a causative 10 Page

11 factor. Nor is the recommended dose that should be taken. So far studies have shown that patients with the highest level of Vitamin D ( nmol/l) had a significantly lower risk of MS than the subgroup with the lowest levels (15 63 nmol/l). Furthermore, populations having a high oral intake of vitamin D have a decreased risk of MS. It appears that vitamin D, besides maintaining bone health and calcium metabolism, is thought to play an immunomodulatory role in the central nervous system. Consider what is meant by the terminology Prevalence and Incidence? References: PM Rothwell, D Charlton. High incidence and prevalence of multiple sclerosis in south east Scotland: evidence of a genetic predisposition. J Neurol Neurosurg Psychiatry : IAF van der Mei et al. Regional variation in multiple sclerosis prevalence in Australia and its association with ambient ultraviolet radiation. Neuroepidemiology : Scotland and genetics 3. Disease Trajectory MS is an unpredictable disease with outcomes ranging from a fairly benign course to rapidly progressive disability. Patients with new onset MS will want to know what to expect in terms of disease progression and future disability, but there is currently no "crystal ball" to predict the future for an individual patient Over the years there has been an emergence of prognostic factors identified in studies of patient cohorts which can help Neurologists as they assess the likelihood of a poor prognosis versus a more moderate course. Such assessments may provide valuable information with regard to implementing and monitoring a treatment plan for MS. Current studies seeking biomarkers related to disease activity patterns and responses to specific medications may one day enable the clinician to prospectively select and optimize disease modifying therapy at the individual level. For more information on this, go to: What is the typical disease trajectory in MS? 11 Page

12 Pathophysiology of the Disease 1. The Blood Brain Barrier The brain and spinal cord are protected from potentially toxic chemicals and organisms by the Blood Brain Barrier (BBB). This is comprised of the least permeable capillaries found in the body, lined with epithelial cells that are tightly packed together forming tight junctions. THE BBB is an important part of the MS disease process. Usually, only water, glucose, essential amino acids, lipids and lipid soluble molecules can pass through it. Hormones, certain drugs and metabolites do not normally cross over but if they do appear in the brain they are pumped out thus protecting the delicate nerves from compounds that might adversely affect its functioning. 12 Page

13 However, alcohol, anaesthetics and certain drugs are lipid soluble and can pass through the BBB. The immune system does not normally cross into the brain and spinal cord; it is able to protect itself without it. As we know though, in people with MS the BBB becomes leaky and immune cells are able to migrate through causing havoc. Disturbance of the BBB is part of the disease process in MS. The release of a number of inflammatory factors contribute to its breakdown resulting then in an influx of lymphocytes, complement, antibodies, macrophages and activated T cells. Go to: for a little more. This inflammatory effect will target the myelin which is essential for nervous impulses to travel around the body. Eventually the myelin will become damaged and demyelination will occur. If there is demyelination, there is loss of function. For more information about the BBB please go to: 13 Page

14 Does the immune system cross normally cross through the BBB? 2. Immune Dysfunction Multiple Sclerosis is characterised by chronic inflammation of the central nervous system (CNS) with pathological hallmarks of inflammation, demyelination, brain atrophy and axonal loss occurring predominantly in the white and grey matter of the CNS. There are multiple signs and symptoms due to the variation in disease severity and the different sites within the CNS that are affected by demyelination. Multiple Sclerosis, as a disease, has been identified and discussed in literature since the 18 th Century, such people were said to have palsy or a paralysis. Physicians also divided the condition into active, passive, functional or organic. One of the most famous physicians who contributed to what we know today about MS was Jean Martin Charcot who worked with people with neurological conditions in Paris in the 1800 s. He is often identified as the man who first discovered MS as he defined and framed the disease and gave it a name people could recognise. He described a condition occurring in young adults with tremor and paralysis, who at post mortem were noted to have grey patches (plaques) scattered through the spinal cord, brain and brain stem. He was able to separate the tremor of MS from Parkinson s disease. The development of knowledge of this condition is fascinating; to learn more, click here for a brief history detailing the timeline of developments. MS is a fascinating condition. It is a condition that affects the central nervous system and as a consequence the symptoms people experience will be related to damage within the brain and spinal cord. But one key fact of this disease is that it is driven by a faulty immune system and is therefore classed as an auto immune disease. There are over 80 different auto immune diseases and you will come across many different ones in your nursing career. 14 Page

15 Auto immunity means that cells from the immune system attack self, cells from the immune system attack the myelin sheath and an intact neuron is essential for nerve conduction. If the myelin sheath is damaged then the action potential required for nerve conduction is going to be impaired and the individual will begin to develop impaired or loss of function. What other auto immune conditions do you know of? Look at:. Are auto immune conditions more common in one gender than the other? So, what part of the immune system is to blame when considering the physiology of MS? You are probably already aware of this, but to remind yourself further go to: verstehen/flash/ to view a resource that will further develop your knowledge and understanding of how this condition occurs. The website is German, but you will see a button on the left hand side of the home page with a British flag, click on this and it will be translated it into English. Take a look at see the connections and be prepared to spend some time listening to the dialogue and watching the fascinating video clips. Sometimes analogies help when revising the immune system and how it goes awry in MS. This article from the MS Trust and Way Ahead is from 2003 is a good one. Go to: and see what you think. Projects in Knowledge as a resource also discuss the immune dysfunction that happens in MS. Go to: It is worthwhile you carrying out some background reading on the Immune System. One piece of reading is easily found in the electronic resources and is: Storey, M and Jordan S (2008) An overview of the immune system. Nursing Standard What are the main players within the immune system involved in the development of MS? 15 Page

16 Oligodendrocytes and the destruction of Myelin Oligodendrocytes are the cells within the CNS that produce myelin. The myelin sheath gives the whitish appearance to the white matter of the brain. Myelin cells are included in the category of Glial cells. Glial cells function to support the processes of neurons in a variety of ways. The glial cells forming myelin sheaths are called oligodendrocytes in the central nervous system and Schwann cells in the peripheral nervous system. There are gaps (approximately 1 micrometer wide) formed between myelin sheath cells long the axons are called Nodes of Ranvier. Since fat serves as a good insulator, the myelin sheaths speed the rate of transmission of an electrical impulse along the axon. The electrical impulse jumps from one node to the next at a rate as fast as 120 meters/second. This rapid rate of conduction is called saltatory conduction. Myelinated axons are rare in the autonomic nervous system. Invertebrates do not have myelinated sheaths. Go to for a You Tube video on how myelin is destroyed in MS. Axonal Damage MS as a neurodegenerative disease in which axonal loss is the major cause of irreversible neurological disability in MS patients. Neurological deficits in MS patients have two pathogeneses: acute inflammatory demyelination and axonal degeneration. Disability caused by inflammatory demyelination clinically dominates the early stages of RR MS but it is reversible. Axonal transection occurs at sites of inflammation and begins at disease onset but is clinically silent in RR MS because the CNS compensates for neuronal loss. Once a threshold of axon loss is exceeded, MS patients enter an irreversible secondary progressive stage. In SP MS, axonal degeneration is caused by chronic demyelination and may be irreversibly progressive. Axonal loss can remain clinically silent for many years, and irreversible neurological disability develops when a threshold of axonal loss is reached and compensatory CNS resources are exhausted. This view of MS provides a rationale for early aggressive anti inflammatory and neuroprotective therapies. Axonal degeneration is as the major determinant of irreversible neurological disability in people with MS. It begins at disease onset and correlates with the degree of inflammation within lesions, indicating that inflammatory demyelination influences axon pathology during relapsing remitting MS (RR MS). 16 Page

17 Nerve Conduction, Neuropathology and Neuroanatomy Whilst much of this section may not be directly asked about in the exam, it is however useful to remind yourself how impulses are transmitted within the CNS. The nervous system is the network of cells and fibres within the body that relays information both physical and cognitive about micro and macro environments in and around the body, using electrical impulses as its signalling device. The nervous system is divided, on an anatomical basis, into: 1. The peripheral nervous system (PNS) The PNS consists of nerves outside the brain and spinal cord. It is divided into the afferent sensory nerve fibres and the efferent motor nerve fibres. It also consists of the autonomic nervous system which is divided into the sympathetic and parasympathetic components. 2. The central nervous system (CNS) Although both will be discussed on this module, it is the CNS that will be the main focus. What are the components of the CNS? This is easy; there are only two, the brain and the spinal cord. It is the primary command and coordination centre receiving and processing information and instructing a response. The CNS has 3 main functions, these are: 1. Receiving information in the form of chemical or electrical stimuli I 17 Page

18 2. Processing information 3. Causing some sort of effect to happen that can either be physical or chemical in nature. Action Potential & Neurotransmitters The cerebral cortex within the brain consists of several hundred billion neurons. The Neurone is the basic unit of the nervous system and is responsible for the receiving and transmitting of the electrical signals that are the primary function of the nervous system. It is important that you are able to identify the components of a Neurone, so you may wish to practice labelling the structure of a nerve cell. You may wish to draw and label the Neurone yourself to get an idea of how it functions. Diagram 1. Structure of a nerve cell. Neurones can be considered to have 3 zones which are: 1. Receiving Zone impulses are received by the dendrites which can be contacted by other nerve cells at junctions known as synapses 2. Conducting zone signals pass down the conducting part of the cell, the Axon 3. Output Zone The signal reaches the nerve terminals (via the Axon) which make synaptic contact with other cells 18 Page

19 How the nerve impulse is generated resting and action potential. Membrane Potential Information is conveyed along a neuron by electrical signals. The resultant waves of this electrical activity are by a process known as an action potential. Before understanding what an action potential is one must learn what a cell membrane potential means. Cell membranes are polarized and maintain a resting membrane potential. They maintain this by distributing ions across the membrane so that inside of the cell is more negative than the outside. The two ions involved are Na+ and K+. There is high concentration of Na outside the cells and low K inside. This unequal distribution is controlled by Na/K pump, which pumps three Na ions from inside to outside in exchange for two K ions. This makes the cells to have a net negative charge inside and a net positive charge outside as illustrated in diagram 2. Diagram 2. The ionic distribution that contributes to resting membrane potential. 19 Page

20 Membrane Potential When a nerve cell is stimulated, usually by a chemical, mechanical or electrical stimulus, an action potential is generated and generates a nervous impulse. During an action potential the membrane potential goes from a negative value ( 70mV) to a positive value (30mV) within few milliseconds. The stimulus, in the case neurotransmitters, opens Na channels allowing Na to diffuse into the cell leading to positive ions entering inside the cell causing the membrane potential to become more positive, an event called depolarization. Sodium channels open very briefly and close again. The K channels then open and more potassium move out of the cell causing the membrane potential once again to become negative. This is termed as repolarization. Action potentials occur only when the membrane in stimulated (depolarized) enough so that sodium channels open completely. The minimum stimulus needed to achieve an action potential is called the threshold stimulus. See diagram 3 for illustration. Diagram 3. An action potential. 20 Page

21 Look at this YouTube clip to further familiarise yourself with action potential: Uv8M Where the myelin sheath is present, it dramatically reduces the flows of Na and K across the neuronal cell membrane and prevents dissipation of the electrical signal along the length of the axon. Since ion flow only occurs at gaps in the myelin (nodes of Ranvier) the electrical signal effectively jumps from node to node along the axon (instead of travelling smoothly and more slowly). Flow of the signal in this way dramatically speeds up electrical transmission up to 100 times and is of great importance for the maintenance of normal bodily functions such as balance which requires rapid feedback between nerve, muscle and environment. Presenting symptoms of MS include: Blurred or double vision Thinking problems Clumsiness or a lack of coordination Loss of balance Numbness Tingling Weakness in an arm or leg. Diagnosis of a relapse A relapse is defined as: patient reported or objectively observed events typical of an acute inflammatory demyelinating event in the CNS [central nervous system], current or historical, with duration of at least 24 hours, in the absence of fever or infection Polman CH, Reingold SC, Banwell B, et al. Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol. 2011;69: A relapse generally lasts for at least 24 hours, for some it can last weeks or months, and frequently requires treatment with steroids. There is also a recognised phenomenon called pseudo relapses. A pseudorelapse is defined as an acute worsening of neurological symptoms which initially appears as a relapse, but it is typically 21 Page

22 associated with an increase in body temperature, perhaps due to infection, exercise, or heat exposure and do not need steroid treatment. Pseudorelapses are often transient and typically resolve when the causative physiological stress (such as the infection, or heat exposure) has been removed or resolved. In practice it can be challenging to distinguish between the 2, but only true relapses need treating. Patients can find identification of a relapse is challenging. Many require ongoing education and information relating to this, the MS Society in the UK and Australia have produced a valuable patient information resource that you might want to take a look at to prepare you for when you are talking about relapses to patients such as Tom. Go to: to take a look. Here is a useful short video on You Tube looking at the impact of a relapse. WARNING: there are a couple of swear words on this Video, please do not watch if you will be offended: There is an excellent slide set on the UKMSSNA that would be a good revision tool for this section. Please go to for the specific section on relapses (UKMSSNA members only) Diagnosis of clinically isolated syndrome CIS is a term used to describe an initial neurologic episode suggestive of MS. Such cases may prove to be a first MS attack; revised diagnostic criteria may shorten the time to demonstrate definite MS even allowing immediate diagnosis for some depending on the MRI findings, but these criteria must still be met before an MS diagnosis can be confirmed. The McDonald Criteria have been validated in and should be applied only to patients with typical CIS. Please look up the McDonald criteria and read more by going to: Here is a patient overview concerning CIS from Canada to get you started today/8 questions you need to ask about clinically isolatedsyndrome.aspx When patients present with a first episode suggestive of a demyelinating event (clinically isolated syndrome [CIS]), it is important to determine how likely they are to convert to clinically definite MS (CDMS). Treatment is now approved and recommended for patients with CIS who have imaging features highly suggestive of MS, and who are likely to convert to CDMS, as discussed in Chapter 3. However, 20% to 37% of patients with CIS may not develop CDMS and might be spared the potential cost and adverse effects, as well as any inconveniences, of MS disease modifying therapies But it is important to remember that a number of other conditions can mimic MS. Hence, a diligent search for a differential diagnosis is generally warranted at the time of CIS and especially for cases that do not have a typical MS presentation or course. 22 Page

23 There are a number of conditions considered clinical variants of MS, which may or may not represent different diseases Neuromyelitis optica (NMO, also called Devic's disease) Inflammatory demyelinating disease of the optic nerve and spinal cord, characterized by optic neuritis (often bilateral) and transverse myelitis, occurring simultaneously or independently. Neurologic deficits may become permanent and severe. Disease course may be monophasic or relapsing. The cord may be swollen during acute attacks. Most experts consider NMO to be a separate disease from MS. Acute MS (Marburg variant; malignant MS, fulminant MS): Rare and highly aggressive form of MS, with frequent and severe relapses that rapidly progresses to disability and death. Disease course is classically monophasic. MRI typically shows extensive multifocal diffuse white matter lesions, usually in the cerebral hemisphere that may be enhancing, sometimes with perilesional edema. ADEM: Classically monophasic inflammatory demyelinating disease characterized by encephalitis like symptoms in addition to those resulting from demyelination. MRI shows multifocal large lesions often with synchronous enhancement and poorly defined margins. Cortical grey matter, thalamic, and basal ganglia lesions are more commonly seen than in MS. Spinal cord lesions may be large and swollen. Neuropathology is characterized by pronounced perivascular inflammation. ADEM may occur in association with immunization/vaccination for measles, mumps, or rubella, or systemic viral infection and more commonly affects children, although it also occurs in adults. Relapses within the first 3 months may occur; however, when symptoms recur at longer intervals, the diagnosis is more likely to be MS. Prognostic indicators It is difficult to discuss prognosis with people affected by MS, not least because much of the data over the long term does not include those taking DMTs. The overall life expectancy for multiple sclerosis patients is 7 years less than normal, or 75% to 85% of expected survival (Weinshenker and Ebers 1987; Sadovnick et al 1992). Mortality increases with disability. Case fatality ratios are 1:5 for patients with Kurtzke disability scores of less than or equal to 7, but 4:4 for those with scores greater than 7 (Sadovnick et al 1992). The suicide rate is increased 7.5 fold (Sadovnick et al 1991). The lifetime risk of suicide is 2% and more common in the less disabled young patients within 5 years of diagnosis (Sadovnick et al 1992; Stenager et al 1992). The cause of death in 50% of a clinic population and in approximately 75% of all multiple sclerosis patients is from complications of multiple sclerosis, usually pneumonia (Sadovnick et al 1991; Reder et al 2012). Patients most commonly die when disability scores approach 8.0. Brainstem lesions occasionally cause loss of inspiratory drive, causing the patient to stop breathing; this is most common at night. Deaths from malignancy are less common than in age matched controls (Sadovnick et al 1991). 23 Page

24 The future course of multiple sclerosis is poor if there are cerebellar or pyramidal symptoms, slow timed walk test at baseline, early sphincter symptoms, multi site onset, frequent early attacks, development of progression or a primary progressive course, and age over 40 years at onset. Good prognostic signs include optic neuritis, sensory symptoms, and an exacerbating remitting course (Weinshenker and Ebers 1987; Phadke 1990). The course is a more important predictor than age of onset. Development of a progressive course is the strongest predictor of poor outcome. The second strongest predictor is the number of relapses in the first 2 years. After a first demyelinating episode, a second attack is more likely in younger patients with abnormal CSF, with more than 8 T2 MRI lesions (dissemination) or 1 Gd enhancing lesion (activity). Long duration, low disability multiple sclerosis is likely to remain stable. Complete recovery is more likely with mild severity and mono lesional exacerbations. Clinic based cohorts have more severe multiple sclerosis than population based groups, where many patients remain stable or progress minimally over 10 years (Pittock et al 2004). Multiple sclerosis reduces quality of life throughout its course. Brain atrophy is often present in mild to moderate multiple sclerosis. Atrophy is most likely to progress when there are Gd enhancing lesions at baseline (Simon et al 2000). Ventricles in relapsingremitting multiple sclerosis increase in size by 5% per year, compared to 1% to 2% in normal controls. In most cases of multiple sclerosis that come to medical attention, disease activity never sleeps and atrophy progresses relentlessly in all multiple sclerosis subtypes. However, there are a large number of subclinical cases with benign courses and presumably much milder inflammation. Favourable Females Low rate of relapses per year Complete recovery from the first attack Long interval between first and second attack Symptoms predominantly from afferent systems (i.e.,. sensory symptoms) Younger age of onset Low disability at 2 to 5 years from the disease onset Unfavourable Males High rate of relapses per year Incomplete recovery from the first attack Short interval between first and second attack Symptoms predominantly from efferent systems (i.e.,. symptoms of motor tract involvement) Older age of onset Significant disability at 2 to 5 years from the onset acute onset 24 Page

25 Later cerebellar involvement Involvement of only one CNS system at the time of onset Early cerebellar involvement Involvement of more than one CNS system at the time of onset To read more about this. here is an article you will find interesting: Click here for even more on this: Diagnosis of MS The classic diagnosis of MS is dissemination of lesions in time and place. Demonstration of dissemination in time used to require either a second clinical attack or changes on a second MRI scan at least 3 months after a clinical event, thereby necessitating a delay in diagnosis. However, the recently revised McDonald criteria allow the simultaneous presence of asymptomatic gadoliniumenhancing and non enhancing lesions on a single MRI to constitute dissemination in time. Dissemination in time can also be demonstrated by a new T2 and/or gadolinium enhancing lesion on follow up MRI compared with a baseline MRI, irrespective of the interval between them.5 Dissemination in space now requires at least one asymptomatic T2 lesion typical of demyelination in at least two of the following central nervous system (CNS) areas: periventricular, juxtacortical, infratentorial, or spinal cord.5 The revised diagnostic criteria should be applied only in patients with typical CIS presentations suggestive of MS, and alternative diagnoses should be excluded.5 Some patients will not meet these criteria at the time of initial attack or even shortly thereafter. These patients will continue to require further monitoring for new CNS events consistent with MS. 25 Page

26 The diagnosis of MS is often a long drawn out process resulting in them living with ongoing uncertainty and anxiety. Go to: is ms/information about ms/diagnosis for a short video from people who underwent this. This elongated process is partly because there is no one single test currently available that definitely confirms or excludes the diagnosis of Multiple Sclerosis. There are also a range of other neurological conditions that have similar features. An early diagnosis is important as for some the earlier the treatment is started the more of a possibility that therapy will be able to modify the progression of their disease. Part of the diagnosis (go to multiple sclerosis/what weknow about ms/diagnosing ms/index.aspx) of MS will require the Neurologist to carry out a Neurological Examination that will provide him with clues as to what condition the patient is presenting with. This examination takes place, usually, in outpatients although you may see part of it carried out in the ward environment. Once the examination is completed, then the necessary investigations will be ordered. A diagnosis of MS should be made by an experienced Neurologist, it is often termed as a jigsaw as many tests have to be carried out to make a definite diagnosis of MS, making the diagnosis can be long and drawn out and can take months for some. Primarily it is down to the Neurologist putting pieces of the jigsaw of his examination and results of investigation together; it is therefore based on the results of the patient s clinical history, neurological examination and other diagnostic tests including an MRI scan. It also involved the exclusion of any other diagnostic possibilities. There are criteria for diagnosing MS, called the McDonald Criteria ( ). These criteria lead and support the diagnosis and facilitates an accurate one. These criteria are widely used worldwide and balance the need for early diagnosis, so treatment can be initiated, and the importance of avoiding false positive diagnosis which can be extremely detrimental to a person s health and wellbeing. 26 Page

27 Neurological Examination: There is no single universally accepted sequence of assessments that will make up a neurological examination. But most neurologists start with an assessment of the patient s mental status then move onto assessment of cranial nerves, motor and sensory system, co ordination and gait. It is not possible, and probably not essential for you, to go into detail about any particular aspects of this examination, but there are plenty of resources on the internet should you wish to investigate this further, however the following will provide an overview for you: 1, MENTAL STATUS: the patient will be asked questions that will establish their: 27 Page

28 Level of consciousness Orientation Speech Language Memory Insight and judgment Abstract thought Calculation ability 2. Cranial Nerves: Whilst it is not required of you as a student nurse to recall the names and functions of each of the 12 pairs of cranial nerves that arise in the brain, it is important to know that disease can affect them and the consequences of this. If you click here you will be able to begin to read about these and begin your appreciation of them. There are numerous rhymes that can help recall their names, some are a bit cheeky, others not. There are some 'fun' methods of learning about the cranial nerves which maybe a good starting point, here is a 12 minute You Tube video that is worth a listen, it can help you learn the names of them and develop your knowledge. (Don't blame me that this guy can't sing too well :)) 28 Page

29 To test your knowledge you may want to undertake this quiz for cranial nerves nerves quiz/ 3. Motor examination will comprise of: Muscle atrophy will be looked for (muscle atrophy can also be termed muscle wastage. If the person develops weakness in certain groups of muscles and they are not used properly, you will see that the person presents with a muscle that has shrunk in size and one limb (for example) can be thinner than the other. Muscle wastage occurs very quickly) Muscle tone (In those with MS the tone within a muscle can be stiff and it can be weak. In other conditions, such as Stroke, the muscle can be not only weak but also flaccid) Assess upper strength, asks the person to squeeze your hands, ask them to raise their arms in the air. There is a common assessment carried out by the Neurologist, they ask the patient to hold both arms 29 Page

30 out in front of them with their palms upward. If the person has a tendency for one of their arms to drift, then they have a weakness. This is called the pronator drift and is pictured here. 4. Sensory examination: The Neurologist will check if the person can feel light touch, pain, temperature, vibration and joint position sense. 5. Co ordination examination: Finger to nose testing is often carried out. The person will be asked to touch his or her finger to their nose and then to the examiners out stretched finger. There will be repetition of this as the examiner will move his finger about. Patients can also be asked to walk heel to toe in a straight line. There are 3 main investigations used to support the clinical diagnosis of MS. These are a Magnetic Resonance Imagery Scan (MRI), Evoked Potentials and a Lumbar Puncture (LP). MRI Scanning As time has gone on, it is the MRI that has become the major player in the confirmation of MS; the other 2 tests are generally only used if there is uncertainty about the diagnosis and to confirm Primary Progressive MS. 30 Page

31 Used routinely in the diagnosis of MS, can show demyelination, inflammation, axonal loss and brain atrophy) A contrast agent can be injected into the patient at the same time as their MRI. Normally this contrast would not pass into the brain and spinal cord (remember what was written earlier about the BBB), but if parts of the BBB have been compromised by MS activity, then it will pass through and will therefore demonstrate new lesions or those which are currently active Evoked Potentials MRI 31 Page

32 Simple and painless electrical tests that measure how long it takes for nerves to respond to stimulation, for example it will measure how long it takes when light is shone into the patient s eye to reach the brain. If this response is slow then this demonstrates demyelination and reduced nerve conduction. Lumbar Puncture (LP) A sample of cerebrospinal fluid (CSF) (go to: )can be obtained by performing a lumbar puncture (go to: ). Subsequent analysis of CSF allows detection of abnormal proteins, tiny fragments of myelin and specific white cells which can help in making a diagnosis of MS. It is also useful in excluding other diagnosis that can mimic MS. Here is a more detailed look at the diagnosis of MS; Go to: for a more detailed look at the different investigations used to diagnose MS. 32 Page

33 Category 2: ASSESSMENT AND INTERVENTION FOR CLINCAL PRACTICE: this area represents 42% of the overall mark. 1. Fatigue 2. Pain 3. Spasticity 4. Tremor 5. Cognition Impairment 6. Elimination Dysfunction (Bladder and Bowels) 7. Sexual Dysfunction 8. Weakness 9. Altered Mobility and Balance 10. Visual Impairment 11 Altered Speech and Swallowing 12. Altered Sensation 13. Other I haven t added anything about symptom management yet. This is an area I am sure you already excel in. But this website has lots of information to remind you, go to: 33 Page

34 A. Pharmacologic Therapies - steroids There is a useful section here on Projects of Knowledge with an audio clip too that you might want to take a look now: The use of steroids in the care of people with long term conditions: Steroid therapy is not, of course, just used in the treatment of MS. You will soon see that in everyday clinical practice patients are prescribed steroids to treat a range of conditions including asthma, arthritis, eczema and leukaemia; they are also used to prevent organ transplant rejection or to treat severe systemic allergic reactions. So, what are steroids? Steroids are naturally occurring hormones secreted by the adrenal glands at a time of stress to help aid recovery and repair. When treating some LTC such as MS, Rheumatoid Arthritis and SLE, synthetic steroids are administered. The dosage will vary according to the specific condition, but the action of steroids in all these conditions is twofold, to suppress inflammation as well as act as an immunosuppressent. When looking in more detail, synthetic steroids also lower white blood cells counts and reduces antibody formation. Immunosuppression with prednisolone occurs in individuals who take in excess of 20 mg per day. Here is a list of commonly prescribed steroids: Hydrocortisone Cortisone Prednisone Methylprednisolone Dexamethasone Here is a short video providing an overview of steroid therapy. Go to to take a look High dose corticosteroid (also known as glucocorticoid) therapy is the main treatment therapy for MS relapse because it shortens the duration of a relapse and 34 Page

35 accelerates recovery. The steroid of choice in this condition is Methylprednisolone. There is, however, no evidence that this treatment improves the overall degree of recovery or alters the natural course the disease is going to take. There is no clear evidence of the optimum regime, administration strategies alter between countries. Even within one country there can be different prescribing patterns for MS, go to : for a short article on relapse management in Scotland by Alan Izat. Since 2010 there has been a move to prescribing oral steroids that the patient can receive at home rather than admitting them for Intravenous therapy. A fairly recent Cochrane review said they were similar in efficacy. There are many adverse effects of steroids, it is essential that we, as nurses, are aware of these so we can educate our patients accordingly. It is important we discuss with the patient that they could experience some or all of the following: Alterations to sleep Anxiety, mania and depression Confusion GI Effects Metallic taste in the mouth High blood sugar levels Water retention Infections Increased BP Ankle edema and weight gain Long term effects on bone density For a more detailed discussion of the side effects of steroids, go to: and take a read The capacity for cortico steroids to cause the individual to experience adverse effects, means that the duration of treatment should be kept to a minimum. 35 Page

36 Despite the fact that steroids are effective in relapse management, the individual also needs support in terms of equipment and personal care. They will also often require input from the rehabilitation team such as the Physiotherapist. Discontinuation of steroids IMPORTANT In MS relapse management, we give what is described as pulse steroids, what this means is that we give high dose period of steroids over a short period of time. Therefore, we are able to stop this treatment abruptly. BUT in other patients who are on steroids for other conditions IT IS ESSENTIAL that the steroid dose is reduced gradually over the course of a few days. You might ask yourself, why? Well, you may recall that the natural production of corticosteroids in an individual is controlled by a "feedback mechanism," involving the adrenal glands, the pituitary gland, and brain. But if a patient is prescribed synthetic steroids, this will lead to the inhibition or reduction of the working of this feedback mechanism as synthetic steroids will cause the feedback mechanism to "hibernate." Thus, steroid use cannot be stopped abruptly. Reducing the drugs slowly gives the adrenal glands time to return to their normal patterns of secretion and therefore is an essential part of steroid management. Overview of Disease Modifying Therapies Steroids have long been the only treatment that has been prescribed for MS that has a positive influence on the disease itself, but this impact is short term only and as already stated does not overall influence the long term outcome of the disease. The 1990 s literally revolutionised the treatment and management of MS. There became the emergence of injectable products known as Disease Modifying Treatments (DMTs)that were the first ever drug to have proven effect on MS and were able to modify its course and reduce relapses that an individual could be expected to experience. There are 3 main groups 1. Injectables: beta interferon & copaxone 2. Monoclonal antibodies 3. Oral therapies There are 2 major compounds that are included in the classification of Injectable DMTs. These are: 36 Page

37 Beta Interferon (products are: Rebif, Avonex, Betaferon and Extavia) how does interferon work? Go to: to find out. You will be led to a 10 minute video that introduces the concept of MS in picture form, before discussing Rebif which is one of the Beta Interferons used. Glatiramer Acetate (Copaxone) how does it work? Go to: to find out. All these drugs have similar efficacy, this to reduce relapse rate in any individual by 30% a year, so if the individual has 3 relapses over a 2 year period, this would be reduced to 2, often patients experience even less than this. Unfortunately their ability to reduce disease progression is not significant. There are a wide abundance of articles on the treatment of MS, you may want to read more about the treatments in more detail. This article is free to view, Go to: therapies multiple sclerosis for the link Click here to read more about the treatments: Second Line Treatments: Monoclonal Antibodies The development of targeted therapies (Monoclonal antibodies) is progressing rapidly with hundreds of new treatments being formulated each year. It is essential that nurses of today have some understanding of these drugs and how they work for specific long term conditions. Nurses are expected to provide information to their patients and to be able to answer patients questions and help them make sense of the treatments they are on. The side effects of these treatments can be life limiting, therefore nurses need to be able to assess their patients and pick up early side effects so they can be addressed and treated. So, What are Monoclonal Antibodies (MABs)? MABs are produced in a laboratory and classed as a type of biological therapy. They can also be called targeted therapy or magic bullets as they are selective in the specific response that they generate. 37 Page