Multiple sclerosis: symptoms, diagnosis, therapy and course
The MS has many faces
Multiple sclerosis (MS) is an autoimmune disease that causes a chronic inflammatory process in the brain and spinal cord, typically manifesting at the age of 20-40 years. Since the disease manifests itself in very different ways and does not always run the same way, it is also referred to as the "1000 faces disease". Other synonyms are encephalomyelitis disseminata and polyslerosis.
The causes of multiple sclerosis are not yet known. In the context of the disease, however, there is a destruction of the myelin sheaths of nerve fibers in the cerebrum and cerebellum and in the spinal cord, which can lead to a variety of complaints.
Typical for the MS are:
- blurred vision
The diagnosis of multiple sclerosis is made by the synopsis of several typical findings. If your doctor has the suspicion that you have MS, he will first perform specific neurological tests such as reflex testing. The diagnosis is confirmed by a brain water examination, the examination of the brain waves in the EEG as well as by an imaging of the brain by means of MRI.
Depending on the course of the disease, multiple sclerosis is classified. There are three different forms:
- relapsing-remitting MS (RR-MS)
- Secondary progressive MS (SP-MS)
- primarily progressive MS (PP-MS)
Not (yet) curable
Although multiple sclerosis is not yet curable today, there are a number of medications that can relieve symptoms and slow the progression of the disease. Drug therapy should always be started early for these reasons.
Accompanying ergotherapy and physiotherapy as well as speech therapy and psychotherapy can be used, as these forms of therapy have a proven positive effect on the course of the disease and can make life easier with MS.
The body attacks itself
Multiple sclerosis (MS) is an autoimmune disease that leads to a chronic process of inflammation of nerve cells in the brain and spinal cord. A typical feature of autoimmune diseases is that the body's own defense system is directed against certain parts of the body, attacks and destroys them. This also happens in the context of MS, which leads to a gradual destruction of the myelin sheaths of the nerve fibers.
Myelin sheaths, also known as myelin sheaths, are extensions of certain cells (oligodendrocytes) whose task is to envelop and thus isolate the nerve cells. However, a nerve fiber is not continuously covered by a myelin sheath, but is interrupted by small constrictions (so-called Schnürringen). This is very important for the dissemination of information that can jump from lacing ring to lacing ring in the form of electrical excitement and so quickly traverse the nerve fiber.
In the context of multiple sclerosis, these isolations are further degraded and replaced by scar tissue (gray sclerosis). As a result, information along the nerve cells can only be passed on slowly, as evidenced by the classic symptoms of the disease. Particularly affected are the nerves of the brainstem, the cerebellum, certain parts of the spinal cord and the optic nerves.
Currently around 120,000 people in Germany suffer from multiple sclerosis. Especially young women between the ages of 20 and 40 are affected. It is interesting that the MS has a striking geographical distribution: the further the distance to the equator, the more often the disease occurs.
The causes of multiple sclerosis are unknown. It is believed that genetic factors and certain infections favor the development of the autoimmune process. On the other hand, there are risk factors that can promote a MS surge.
Anyone who has first-degree relatives (siblings, parents, children) with multiple sclerosis, in contrast to the general population, has a 10 to 30-fold increased risk of also developing MS, which corresponds to a risk of 3-4%. But also the ethnicity seems to play a role. In the USA, for example, a reduced disease risk was observed in the group of Hispania as well as among the African Americans.
Infections: dormant danger
How and which infections play a role in the development of MS, is still widely discussed. Responsible could be pathogens that linger for a lifetime in the body after an infection and can always lead to inflammatory inflammations. It is believed that in the course of this leads to the formation of antibodies to the myelin sheaths of the nerve cells and thus to the emergence of the autoimmune process or MS.
Risk factors in each other
Certain life situations may favor the onset of a recent MS episode. These risk factors are suspected to activate the immune system and thus feed the autoimmune process.
Typical risk factors are:
- mental stress situations such as separations or death of a close relative
- physical stress situations such as accidents or major operations
- Changes in hormone balance as the entry into puberty
- Viral infections (for example with herpes viruses or influenza)
- active vaccinations
- Medicines that affect the immune system (eg sun hat)
The risk factors individually have a very different influence on the course of the disease. For this reason, it is important that you recognize early on how you can protect yourself from negative factors and which risk factors are the most dangerous for you.
Questions about causes and MS risk
Since the MS can play in many different parts of the brain, the symptoms of the disease can be very different interindividuell. There is no specific indication that marks the onset of the disease or is typical of a particular course. The variety of possible complaints has also helped multiple sclerosis achieve its epithet "Disease of the 1000 Faces".
Early symptoms: usually start with blurred vision
Symptoms that can already occur in the early stages of the disease are mainly:
- blurred vision
Visual disturbances appear as an early symptom in approximately ¾ of all people with MS and in most cases are initially only one-sided. The cause is an inflammation of the nerve sheaths of the optic nerve (so-called optic neuritis), which leads to visual disturbances and painful eye movements.
Typical of optic neuritis is that everything is suddenly perceived as through a veil or thick fog. Depending on how severe the inflammation is, color vision may be compromised. In many cases, the inflammation is manifested by the appearance of light flashes or visual field defects. If the eye muscles are also affected by the inflammation, the eye movement can be restricted, which leads to the formation of double images. In most cases, the symptoms completely disappear within a few weeks to months.
Deafness on arms and legs
Also feeling or sensory disturbances have very versatile characteristics. Typical of MS are not only tingling and numbness of the arms and legs, but also tight joints and around the hips, as well as pain or changes in temperature perception. Often, the discomfort begins at the tips of the fingers or toes and gradually spreads to the arms and legs.
Characteristic of the beginning of MS is also the so-called Lhermitt'sche character (also positive neck flexion sign). It comes when bending over the head to electrifying sensations that descend along the spine from top to bottom.
Symptoms of paralysis appear rather unspecifically at the beginning of the disease and lead to a quick fatigue of the musculature. Occasionally spastic tension and unusual muscle stiffness can occur. Muscle weakness characteristically increases due to fever, heat or physical exertion (so-called Uthoff phenomenon).
Late symptoms: movement, balance and language
Late symptoms usually occur when a large number of nerve cells or myelin sheaths have already been destroyed by the inflammatory process. Similar to the early symptoms, the appearance of the disease is very heterogeneous. In the course of the disease, impairments of voluntary motor function, the cerebellum, and the autonomic nervous system are the main causes.
The infestation of certain parts of the spinal cord can lead to the loss of voluntary motor skills. It expresses itself by the inability to carry out certain movements deliberately and willingly. This is particularly psychologically very stressful, since foreign aid is needed and the quality of life is severely impaired. If individual cranial nerves are affected by the inflammatory process, it can lead to facial paralysis, but also to balance and taste disorders.
MS impairment of the cerebellum classically causes the triad of speech disorders, gait insecurity, and tremor. Speech disorders in particular are very stressful, as the language sounds increasingly indistinct and choppy.Individual syllables are uncoordinated and are emphasized indiscriminately but in a characteristic way. The trembling of the hands (tremor) typically occurs during target movements and increases with increasing proximity to the target (so-called goal or intention tremor).
Problems with sex
An impairment of the autonomic nervous system manifests itself above all in bladder and rectal deficiency disorders such as urinary and fecal incontinence, but also urinary retention. Furthermore, disturbances of the sexual function can become noticeable:
- in men typically in the form of erectile dysfunction
- in the woman in the form of a loss of libido and in a reduced sensitivity in the genital area
MS can continue to cause mental illness. Whether these are caused by the MS itself or are the result of the disease is still unclear. Typical are mood swings and depressive phases, which manifest themselves in the form of sadness, sleep disorders and lack of drive.
Even deficits of memory and concentration performance up to dementia can occur. But sometimes there is also a groundless euphoria, which is not appropriate for the disease situation and especially in combination with cerebellar disorders.
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Symptoms and complaints
Multiple sclerosis is usually a relapsing disease. Depending on the appearance, a distinction is made between relapsing remitting MS (RR-MS), secondary progressive MS (SP-MS) and primarily progressive MS (PP-MS).
A push indicates the onset of new symptoms or a significant worsening of pre-existing discomforts that last at least 24 hours or continue to increase over time. By definition, there must be more than 30 days between two individual bursts. If two "relapses" occur within 30 days, this is considered a single thrust.
Shear-remitting MS (RR-MS)
The relapsing-remitting MS is characterized by the appearance of individual thrusts, which regress more or less completely. At 80%, RR-MS is the most common form of initial diagnosis, but about half of the cases progress to secondary progressive MS.
Secondary progressive MS (SP-MS)
The secondary progressive MS is also characterized by a relapsing course. However, the symptoms no longer completely recede between episodes, so that the disease progresses overall progressively. By definition, the SP-MS originates from the RR-MS.
Primary progressive MS (PP-MS)
The rather rare primary progressive MS (20% of all MS forms) is entirely without relapses, but progresses steadily. It is associated with a worse prognosis and is less responsive to medication.
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Questions and answers about the course forms
If MS-typical symptoms are present, it is essential to go to the doctor. In a first interview (anamnesis), he asks for the entire complex of complaints and also inquires about first-degree relatives who may also have MS. This is followed by a complete neurological examination, in which, among other things, the function of the cranial nerves, reflexes, muscle strength and sensitivity are checked.
Particular attention is paid to the examination of the two cranial nerves, the facial nerve and the trigeminal nerve, which are responsible for the mimic muscles (facial nerve) and for the sensations of the face (trigeminal nerve). The functions of these two nerves are particularly often limited in MS.
A typical finding for MS is also an extinct abdominal skin reflex. To test this reflex, the doctor asks you to lie down and clear your stomach. Using a mouth spatula or similar, he then gently strokes the abdomen below the costal arch, at the level of the navel or above the inguinal region. Normally it comes to a reflex tension of the abdominal muscles, but not in the context of a MS. There the abdominal skin reflex is extinguished.
The sensitivity is checked above all by the Lhermittian sign. But also vibration, temperature and touch measurements must be made.
In order to finally diagnose multiple sclerosis, further tests and examinations are necessary, which usually necessitate hospitalization in hospital. Important investigations include cerebral water diagnostics, encephalographic procedures and imaging of the brain.
The brain is under the microscope
The examination of the cerebrospinal fluid (liquor diagnostics) can show inflammatory changes of the brain and spinal cord. In this study, certain antibodies and immune cells are detected in the Liqour, which are not found there in the healthy state, but there in the context of various inflammatory diseases of the nervous system but there accumulate.Only the synopsis with other typical findings can prove a MS.
These include, among others, the EEG (electroencephalogram) examination, which measures reaction times to specific stimuli. For example, you will be presented with an optical or acoustic stimulus while you are connected to the EEG. Based on certain changes in the EEG, the examiner can see how long it took for the set stimulus to reach your brain. In MS, the reaction times are usually prolonged, since the nerve conduction velocity is reduced by the degradation of myelin sheaths.
Very important for the diagnosis of multiple sclerosis is still magnetic resonance imaging (MRI). Early on, inflammatory foci can be detected in the brain in typical locations, often before the first symptoms appear.
All about diagnostics and examinations
A complete cure for multiple sclerosis is not yet possible according to the current state of science. Nevertheless, therapy should be started as early as possible to reduce the chronic inflammatory process in the brain and spinal cord and alleviate the symptoms.
The treatment of MS consists of different pillars. In addition to drug therapy supportive and symptom-reducing measures such as physiotherapy and ergotherapy, but also speech therapy and psychotherapy are of crucial importance for the disease process.
1. Drug therapy
The medical treatment of MS depends on the course of the disease, the symptoms in the foreground and the severity of the disease.
According to the guidelines of the German Society of Neurology, the following types of therapy are distinguished:
- thrust therapy
- Course-modifying therapy consisting of basic therapy and escalation therapy
a) Shock therapy: short and intense
The Schubtherapie is always used only for a short time to contain the acute episode of illness. Essentially, it serves to reduce the activity of the immune system and thus to reduce the symptoms or shorten the duration of the thrust.
High-dose glucocorticoids (500-1000 mg / day methylprednisolone (Advantan®, Methylpred®, Metysolone®, M-Predni®) over 3-5 days. With good regression of the symptoms, the shock therapy can be ended gradually. In case of poor regression, the therapy can be extended to 10 days and a short-term dose increase to double the dose.
Unlike the long-term use of cortisone, short-term administration results in significantly fewer side effects. However, food cravings, restlessness, sleep disturbances and palpitations may occur.
Not always does the acute boost from the administration of glucocorticoids decrease satisfactorily, in some cases the symptoms even progress. Then there is the possibility to perform a plasmapheresis. In this method, the blood is separated into its liquid (plasma) and solid components. The plasma is exchanged for a replacement solution, the solid components are returned to the body. The aim of this method is to filter antibodies from the blood and to reduce the inflammatory activity.
b) Course-modifying therapy
Course-modifying therapy is a long-lasting medication designed to slow the progression of MS and maintain quality of life. Depending on the severity, course and stage of the disease, a distinction is made between a basic therapy, which is used for mild to moderate disease activity, and an escalation therapy for highly active MS.
Basic therapy: mostly as an injection
For the treatment of mild and moderate forms of pregnancy, the following drugs are preferably used:
- Interferon-beta (IFN-β) (Avonex®, Rebif®, Betaferon®, Extavis®)
- Glatiramer acetate (Copaxone®)
- Dimethyl fumarate (Tecfidera®)
- Mitoxantrone (Haemato-tron®Novatron®, Oncotrone®, Ralenova®)
Interferon beta: every few days in the stomach
The drug of first choice for the basic therapy of multiple sclerosis is interferon-beta (IFN-β). IFN-β has an immunomodulatory effect. This means that the drug can regulate the formation of certain inflammatory messengers and thus influence the immune system.
IFN-β must be injected into the subcutaneous fatty tissue as it would not reach its site of action when administered orally. With a little practice, you can easily inject the remedy yourself every two to three days.
The list of side effects that can occur with IFN-β is long. Especially at the beginning of the treatment, it can lead to flu symptoms such as fever, headache and body aches or fatigue and fatigue. The puncture site at which the drug was injected into the subcutaneous fatty tissue can swell or redden, especially after the first doses. The side effects usually decrease over time, so the benefits of the drug are more prominent.
Glatirameracetet: side effects less common but more severe
An alternative to IFN-β is the drug glatiramer acetate, which is also considered the drug of first choice for the treatment of MS. Glatiramer acetate must be injected daily into the subcutaneous fatty tissue.
The exact mechanism of action of this drug is not fully understood. However, it is believed that the immune system is "distracted" and focuses more on the elimination of the drug than on the destruction of myelin sheaths.
Unlike IFN-β, glatiramer acetate is usually well tolerated from the beginning. In some cases, however, it also comes to side effects, which are often difficult. In addition to shortness of breath, anxiety and anxiety, tachycardia and sweating also occur, which often disappear after a few minutes.
Alternative in tablet form
Dimethyl fumarate is an immune modulator that has anti-inflammatory effects and has a protective effect on the nerve cells. Advantage of the drug over the above two is that it can be taken as a tablet and does not need to be injected.
However, especially at the beginning of dimethyl fumarate can lead to gastrointestinal complaints and allergic reactions of the skin. These side effects are usually over time. However, dimethyl fumarate has a particularly dreaded side effect: progressive multifocal leukoencephalopathy (PML), which can severely limit sensitivity, muscle power, and cognition.
Because the immune system is severely limited under dimethyl fumarate therapy, blood counts should be performed every four to eight weeks.
Mitoxantrone is rarely used, but often helps, especially when a shift from relapsing MS to secondary progressive form has occurred. The drug is a potent immunosuppressant that can reduce the breakdown of myelin sheaths.
Escalation therapy in severe cases
A highly active form of multiple sclerosis requires a more intensive regimen of other immunomodulatory drugs. An MS is classified as highly active if the base therapy does not reduce the number of episodes or make the condition even worse. Even a severe form of MS from the beginning can be considered highly active. The following medications are preferred:
- Alemtuzumab (Lemtrada®)
- Fingolimod (Gilenya®)
- Natalizumab (Tysabri®)
- Mitoxantrone (Haemato-tron®, Novatron®, Oncotrone®, Ralenova®)
An anti-inflammatory antibody
Alemtuzumab, in addition to fingolimod and natalizumab, is used as a first-line treatment for highly active MS. Alemtuzumab is an artificially produced antibody that targets the disease-causing immune cells. If the antibody comes in contact with these cells, it binds them and makes them harmless, which significantly reduces the symptoms of MS.
The side effects include:
- flu-like symptoms
- Changes in the blood picture
- allergic reaction
- Increase in liver enzymes
Variant as a capsule
Fingolimod has a similar mechanism of action. The drug prevents disease-causing cells from entering the brain and destroying the nerve cells. As a result, fewer myelin sheaths are broken down and cell function is retained longer. Fingolimod, unlike alemtuzumab, which needs to be injected, can be taken as a capsule. Side effects can occur in large numbers, which is a significant disadvantage of the drug.
- Gastrointestinal problems
- a slowing down of the heartbeat (bradycardia)
- depressive moods
- blurred vision
Natalizumab must inject the doctor
Natalizumab is another antibody approved for the treatment of highly active MS. He also prevents penetration of the disease-causing cells from the blood into the brain and thus severe inflammatory reactions. Natalizumab must be given directly into the blood via the vein every four weeks.
One advantage is that side effects are rare. Now and then there are headaches, urinary or mild respiratory infections and joint pain. A disadvantage is that natalizumab, when used over a long period of time, can lead to the dreaded side effect of progressive multifocal leukoencephalopathy (PML). It is a reoccurring viral infection that can cause serious damage to the nervous system and is associated with muscle weakness and sensory disturbances.
2. Supportive therapy
Depending on which complaints are the focus of treatment, more drugs must be used. For example, symptoms such as tremor, discomfort to the arms and legs, bladder emptying disorders and disorders of sexual function can be treated well with medication.
It is important that you name all complaints and especially those that affect your quality of life the most, clarify and treat early.
Although MS therapy without medication does not work, general measures for the treatment of MS should not be missed. For once destroyed nerve tissue can not regenerate, so that only by training lost body functions can be compensated.
Exercise and physiotherapy can help you maintain your flexibility, endurance, and fine motor skills for a long time. Furthermore, the early handling of tools can be learned.Logopedics aims to train communication, to maintain language and expressiveness.
Alternative therapies such as acupuncture and homeopathy may prove useful in the treatment of MS but in no case replace drug therapy.
Do not forget the psyche
Psychological support through the disease and therapy is always useful. Living with multiple sclerosis raises questions that you did not have to face before and that can be very stressful. With a trained therapist at your side, you can more easily rearrange your life goals and deal with the overwhelming burden of disease.
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Everything important to the treatment
Multiple sclerosis is a chronic progressive disease that in most cases is fatal. The course can be predicted for the individual case, however, with difficulty because the disease is very different.
Factors associated with a favorable prognosis are:
- a young age of onset (under the age of 35)
- only a symptom or only sensitive symptoms
- short push duration
- complete regression of the thrust
- long-term walking ability
In general, the MS has a much better prognosis than generally assumed. Studies have shown that, after a median duration of illness of 15-17 years, almost half of all people with MS are still fully employed or able to self-medicate themselves.
Author: Lisa Wunsch
R.M. Schmidt et al .; Multiple Sclerosis, Urban & Fischer, 2015.
K.F. Masuhr et. al .: Dual Series Neurology, Thieme Verlag, 2013.
Guidelines of the German Society of Neurology: Diagnosis and Treatment of Multiple Sclerosis, http://www.awmf.org/uploads/tx_szleitlinien/030-050l_S2e_Multiple_Sklerose_Diagnostik_Therapie_2014-08_verlaengert.pdf, last accessed on 24.03.2017.