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Index arrow Roztroušená skleróza arrow O nemoci arrow Rozbor roztroušené sklerózy

Rozbor roztroušené sklerózy Tisk E-mail
Pondělí, 14 květen 2007

Roztroušená skleróza je onemocnění, postihující mnohočetnými ložisky bílou hmotu centrálního nervového systému (CNS), tedy mozku a míchy. Bílá hmota je tvořena nervovými drahami (výběžky nervových buněk, po nichž jsou přenášeny nervové vzruchy) a jejich obalem, myelinem, který slouží jako izolační hmota a umožňuje rychlé vedení vzruchu po nervových vláknech. Nervová vlákna, která mají tento obal, tzv. myelinovou pochvu, nazýváme myelinizovaná. Ta onemocnění, u nichž dochází k poruše a úbytku této myelinové pochvy, nazýváme souhrnně demyelinizacemi.

Důležité je uvědomit si, že nervové vlákno, tzv. axon, spolu s nervovou buňkou jsou nositeli funkce v nervovém systému. Nervový vzruch v nervové buňce vzniká a je posílán k další buňce nebo k výkonnému orgánu např. svalu po nervovém vláknu. Řada vláken myelinový obal nemá. To jsou vlákna, která vedou vzruch pomalu a v jejich funkci to nevadí – předávají informace např. vnitřním orgánům či žlázám. Myelin je velmi prostorově náročný. Proto jsou jím obalena jen vlákna, která potřebujeme k tomu, aby vedla vzruch rychle, např. ke svalům. Vzruch se nepohybuje po jejich povrchu kousek po kousku, ale přeskakuje po zářezech v myelinu, které jsou od sebe vzdáleny asi 2 mm. Rychlost vedení vzruchu je proto mnohonásobně vyšší než u nemyelinizovaného vlákna.

myelin3.jpg Šedá hmota CNS je naopak tvořena těly nervových buněk. V mozku je to mozková kůra a podkorová šeď (velká podkorová jádra, tedy velké shluky nervových buněk). V míše je šeď v okolí centrálního kanálku, který je uprostřed míchy, bílá hmota je naopak v míše na povrchu.

U RS dochází z neznámých příčin k tvorbě zánětlivých demyelinizačních ložisek kolem drobných cév v bílé hmotě CNS. Ložiska mohou mít velikost od 1 mm do několika cm. V důsledku zánětlivých změn dochází k rozpadu myelinové pochvy v místě ložiska, a tím k poruše vedení vzruchu obnaženým nervovým vláknem. Vlákno, kterému se rozpadne myelin, není schopno po dobu několika dnů vést vzruch vůbec, dochází k bloku vedení. To proto, že na obnaženém vlákně nejsou iontové kanály, kterými vzruch běží. Ty jsou u myelinizovaného vlákna nashromážděny právě v zářezech, oddělujících „buřtíky“ myelinu. Trvá několik dnů, než se rozprostřou po obnaženém vláknu a obnoví funkci vlákna, tedy vedení vzruchu. Po několika týdnech, když zánětlivá reakce odezní, může být myelin dotvořen, pokud nebyly zničeny buňky, které ho jako jediné v těle vytvářejí. Není již nikdy tak silný a zářezy v něm jsou blíže u sebe, ale nervové vlákno již není obnažené, i když vedení vzruchu je přece jen o něco pomalejší než u zdravého vlákna.

myelin1.jpgV poslední době bylo zjištěno, že v akutním zánětlivém ložisku dochází i k trhání nervových vláken samých, což ve svém důsledku znamená při jejich velké ztrátě i poškození funkce. Není zatím známo, na čem je míra ztráty nervových vláken závislá, jak to, že u některých pacientů k ní dochází velmi pozvolna a u jiných masivně, takže se roztrhne v akutním zánětlivém ložisku až několik tisíc vláken v jednom mm3. Všechny nervové dráhy v těle jsou jištěny větším množstvím vláken, než je pro normální funkci potřeba. Proto až po jejich významné ztrátě zůstanou po atakách trvalé následky. Ztráta nervových vláken je tedy zodpovědná za trvalý výpadek funkce, za neurologickou invaliditu.

Pokud je myelin porušen na dráhách, které vedou pokyny pro pohyb, projeví se akutní příznaky poruchami hybnosti, pokud je myelin porušen na dráhách, vedoucích k CNS informace o tom, co cítíme dotekem, projeví se to poruchami citlivosti apod. (viz kapitola „Příznaky“).

Schopnost nahradit rozpadlý myelin novým je v CNS u člověka omezená. Myelin je totiž stočený výběžek buňky, která jej vytvořila, tedy její dvojitá membrána. Rozpadá-li se myelin opakovaně, znamená to, že buňka ztrácí opakovaně část sebe sama – své výběžky – a tento opakovaný stres, kdy musí znovu dotvářet část svého těla, vede k vyčerpání jejích regeneračních schopností. Opakuje-li se zánět několikrát na jednom místě, není myelin už nahrazen a v místě zánětlivého postižení přetrvává výrazně zpomalené vedení vzruchu, odpovědné za zhoršení funkce a zřejmě zčásti za unavitelnost svalu touto dráhou zásobeného, zraku, ap.

 Je-li nervová dráha (která může měřit až několik desítek cm) postižena rozpadem myelinu na několika místech, dochází časem k výrazné poruše funkce, protože obnažené nervové vlákno trpí a nakonec zaniká. Tento zánik degenerací je znám již dlouho a uplatňuje se kromě akutní ztráty vláken především ve fázi chronické progrese, kdy neurologická invalidita postupně narůstá. Regenerace nervových vláken v centrálním nervovém systému u člověka není možná, pouze v periferním nervu mohou vlákna dorůstat, a obnovit tak funkci ztracenou např. poraněním, ale ani tato regenerace není úplná a dokonalá.

 Dokud nejsou postiženy nervové dráhy přístupné neurologickému vyšetření, nelze prostým vyšetřením pacienta onemocnění ani jeho rozsah klinicky rozpoznat. K tomu jsou nutné pomocné vyšetřovací metody (viz příslušná kapitola).

Často se ataky onemocnění – vznik neurologických příznaků – váží na předcházející infekci, často lehkou, jak virovou, tak bakteriální, nebo na stres, ať psychický, či fyzický.

 doc. MUDr. Eva Havrdová, CSc.

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