Multiple sclerosis

The multiple sclerosis (shortened in a current way in " SEP") is a neurological disease chronic and often invalidating. It is multifactorielle and its clinical demonstrations are related to a Démyélinisation nervous fibers of the white substance of the central Nervous system (Cerveau, Spinal-cord and Optical nerve).

It touches approximately 80  000 people in France (Prevalence), and approximately 2  000 people moreover per annum (incidence). 70% of the new patients are young adults between 20 and 40 years, of which nearly two thirds of women. It is an important cause of handicap.

History

The multiple sclerosis was diagnosed for the first time in 1868 by Dr. Jean-Martin Charcot.

Physiopathology

The causes of the multiple sclerosis are imperfectly known. They associate factors Génétique S (the family of a patient has more risk to be touched than the general population), factors environmental (the moderate countries are touched much than the tropical countries, whatever the ethnic origin) and a starting factor of the disease (probably infectious, by over-sensitiveness and birth of an auto-immune answer after a banal infection). For this reason, one finds among patients reached of SEP a very high rate of Anticorps directed against some Virus (in particular the Rougeole).

The multiple sclerosis would be thus a auto-immune Maladie (related to the abnormal activity of certain antibodies directed against the sheath of Myéline of nervous fibers) started after a probably viral event, at a subject genetically predisposed with the Maladie.

Since the Years 1970, genes associated in occurred with the disease were discovered little by little, in particular on the level of the group HLA. In July 2007, two variable of genes was identified, IL2RA and IL7RA , both coding Interleukine S intervening in the immunity and whose presence would increase (slightly) the risk to develop the disease.

Anatomical lesions

They consist of more or less wide plates of demyelinisation within the white Substance of the central nervous system (consisted of nervous fibers, i.e. of neuronal prolongations: the Axon S). These nervous fibers are normally surrounded by a sheath of myéline consisted the Oligodendrocyte S (they are the cells of Schwann which assumes this role in the peripheral Nervous system, which is not touched him by the SEP), which is destroyed at the time of a push of the disease: the sheath is destroyed, but the axon is intact. This demyelinisation involves a deterioration of electric conduction in the axon (information forwards less quickly), which leads to varied clinical signs, which appear in a few days. The characteristic of this disease is its evolution, marked by phases of thorough (at the time of the constitution of a new zone of demyelinisation) and of remission (when the plate heals, with remyelinisation partial) with sometimes spectacular improvement of the Symptôme S. Unfortunately, with time, the new pushes heal less better, and neurological deteriorations end up more not regressing, constituting final lesions. The rate/rhythm of the phases of thorough/remission is very variable from one individual to another, with the result that for some the disease remains very a long time without major impact apart from the pushes, whereas at others a fast deterioration of quality of life occurs in connection with frequent and not very resolvent pushes.

Diagnosis

The disease begins typically between 20 and 40 years and touches three women for a man. 3% of the cases are diagnosed at the 50 year old people and more. Certain characteristic elements must make suspecter a multiple sclerosis: repeated, regressive neurological accidents (at least at the beginning of disease), concerning variable functions (vision, motricity, sensitivity, etc): the pushes are disseminated in time and space. Among the neurological accidents most frequent, one finds:

pyramidal Syndrome

It is inaugural (revealing) of the disease in 20% of the cases, and appears by disorders of the Marche with an important fatigability, a spasticity, reflexes exaggerated on the level of the lower extremities, the presence of a Signe of Babinski, an abolition of the abdominal cutaneous reflexes. At the conclusion of the push, the sign of Babinski often remains like after-effect.

retro-bulbar optical Neuritis

Revealing in nearly a third of the cases, it is the most evocative Symptôme. It appears for the patient by a fall of progressive and major vision, pains orbital eyepiece and , a central Scotome and an eye trouble of the colors (Dyschromatopsie of the axis red-green). At the acute stage, the Fond of eye is normal (“the patient does not see anything, and the doctor either”), and it is only after one fifteen or so days that an atrophy of the papilla appears, witness of the attack of the Optical nerve and persisting sometimes like after-effect. Visual recovery is often good and is done in a few weeks.

Disorders of the sensitivity

They are primarily subjective: Paraesthesia S, swarmings, Sign of positive Lhermitte (feeling of electric shock along the rachis at the time of the inflection of the neck). One finds sometimes a posterior syndrome cordonnal with disorders of the major sensitivity, and more rarely a spinothalamic attack of the beam with a thermo-algesic anesthesia. Pains of the face (or on the other hand an anesthesia) are possible in the event of attack of the cranial nerve in its bulbar portion.

vestibular Syndrome

It associates rotatory Vertige, Nystagmus, and Ataxie.

cérébelleux Syndrome

The plates of demyelinisation are frequent in the Cervelet and the posterior pit in general, being able to give a cérébelleux syndrome with station upright unstable, ebrious walk, movement dysmetric, etc

Diplopia

Diplopia is a feeling of Vision duplicated by anomaly of the muscles oculomoteurs (generally side right innervé by the sixth pair of cranial nerves). An internuclear ophtalmoplégie is possible in the event of attack of the posterior longitudinal strips (which establish the link between the cores of the nerves oculomoteurs and ensure their harmonious operation) which appears in the side glance by a incomplete Adduction of an eye associated with a nystagmus of the eye in Abduction.

Disorders génito-sphincteriens

They are frequent and related to an attack of the spinal-cord. They appear by pressing micturitions (or a urinary retention), the Constipation, the Impuissance. These disorders cause acute retention of urines, urinary infections and psychological distress and are a therapeutic priority.

facial Paralysis

By attack of the facial Nerve in its protuberantial way (i.e. in the cerebral Trunk).

Mental health disorders

The depression is frequent during the multiple sclerosis, at the same time because of the painful demonstrations of the disease, but also because of the cerebral lesions characteristic of the disease and their effects on mood. A psychic excitation (Syndrome maniac) is rarer but possible.

Complementary examinations

IRM cerebral and medullary

It represents the best examination to visualize the lesions characteristic of the SEP: one finds in T2 sequence of the zones of hypersignal (i.e. very white) of variable size, of round form, without effect of mass on the other structures, laid out preferentially around the ventricles cerebral, in marrow, and the posterior pit. In sequence T1, these zones can be in Iso or hyposignal.

The visualized lesions are often old and asymptomatic. The young lesions, responsible for the push in progress, are noticed because they take contrast during the gadolinium injection (they are raised). These signs are not specific multiple sclerosis, and are frequently seen at the normal old subject.

Thus, in the event of dubious diagnosis, a IRM can wrongly describe as patient a patient who in is unscathed: it is primarily the temporal conjunction between neurological accident and young plates which makes the value of these images IRM. The clinical evolution remains however the element most useful for the diagnosis: 2 pushes distinct with more than 30 days from interval or two lesions of different localizations.

Lumbar puncture

The lumbar Ponction is not always essential to the diagnosis. It has an interesting diagnostic value when it finds a céphalo-rachidian Liquide inflammatory, with a rate of Protéine S high (< 1 g/l, the normal being of less than 0,4 g/l) and within these proteins a too high proportion of Immunoglobuline S (more than 14%), that a electrophoresis will show being of nature oligoclonale (i.e. composed of several types of immunoglobulins). Lastly, a lymphocytose moderate lower than 50 lymphocytes per millimetre cubes céphalo-rachidian Liquide.

Evoked potentials

Their study frequently finds a lengthening of the central latency time.

Criteria of McDonald of the diagnosis

Forms of multiple sclerosis

The traditional form of the multiple sclerosis can have three evolutionary modes:

  • the recurring form

  • the form progressive primary elections
  • the progressive form secondary

There are also the Frontières of the multiple sclerosis where one has the cases which one cannot classify in or out of the multiple sclerosis. They are:

  • the optical Neuromyélite of Eugene Devic

  • Three pathological forms (concentric Sclérose of Balo, the Maladie of Schilder and the malignant form, that one calls the Sclérose of Marburg)
  • Other diseases (autoimmune peripheral Neuropathie and Encephalomielitis disseminate acute, ADEM)

Treatment

Push

The pushes evolve naturally to the spontaneous remission. If they are invalidating, they can receive a treatment by corticosteroids, which make it possible to shorten their evolution, to hasten the cure, but not to prevent another push. The hospitalization in service of Neurologie essential, but is not strongly advised because of the symptoms generated by cortisone, particularly at the time of the stop of the treatment (suicidal impulse, pains.). The corticothérapie is done with raised amount, from 3 to 5 days, by intravenous way. One prescribes sometimes corticosteroids out of relay by oral way for approximately 3 weeks, associated with preventive measures of the side effects of the corticosteroids (désodé mode, supplementation in Calcium, Potassium, Vitamine D, monitoring of the weight, blood Pressure, mood, cutaneous state). According to the attack, meetings of Kinésithérapie are proposed.

Basic

The “ interféron beta and the acetate of glatiramère were the proof of their effectiveness in the multiple sclerosis (pushes fewer and less severe, improvement of the visible lesions in IRM, sometimes less evolutionarity of the handicap).
  • There exists Interféron beta-1b (managed by subcutaneous injections), and the Interféron beta-1a (managed by subcutaneous or intramuscular injections). The indications of the treatment by Interféron are the remittent SEP with at least two pushed out of both or three previous years, or the SEP secondarily progressive with persistence of thorough (continuous and progressive aggravation, without remission between the acute phases). The indications currently extend for an early beginning from the treatment as of first pushed under certain conditions. The interféron against is indicated during the Grossesse and the Allaitement, at the not stabilized epileptics, and during the severe depressions.
  • the acetate of glatiramère, as for him, is a copolymer made up of several Amino-acid advised to reduce the pushes among ambulatory patients (still being able to only go) reached multiple sclerosis evolving/moving by pushed of recurring type/rémitente characterized by at least two pushed during the two previous years.

The Natalizumab, a monoclonal antibody directed against the chain alpha of the Intégrine of the leucocytes, is used with a certain success. It can be proposed in the remittent SEP, either in first intention in the severe cases (two pushed in one year with after-effects), or after failure of let us interfere (a push in one year in spite of the treatment).

In the severe forms it can be proposed to use immunosupresseurs among whom the mitoxantrone .

  • Taken in social contribution and psychological: by integration with groups of patients, maintenance of an use and with the adaptation need of the work station, psychotherapy, treatment of a depression or an anxious state.
  • driving Rehabilitation, fight against the spasticity
  • Treatment of the disorders génito-sphinctériens

Research and development

According to a study directed by Jorge Correale of the Neurological Institute of Buenos Aires, an infection by intestinal worms would make it possible to divert the auto-immune action of the white globules against the parasites and either against the clean nervous system.

Efforts of fight

In France, many associations take part in the fight against the multiple sclerosis, and gathered their efforts by creating UNISEP.

Personalities reached of the multiple sclerosis

See too

Refer

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