Dravet syndrome. Zvakakomba myoclonic pfari kubva paucheche

Pakati kuratidzwa siyana uduku pfari Dravet chirwere inzvimbo chaiyo uye zvichida wakakura zvikuru uye upenyu-kutyisidzira zvinetso mwana. chirwere ichi kunooneka pacharo pagore upenyu mwana wokutanga uye kazhinji kunotungamirira zvakakomba nokuvhiringidzirwa ake psychomotor kukura, uye mune zvimwe zviitiko - uye rufu. Izvi chaizvo isingawanzowanikwi chirwere chacho chikuru zviratidzo uye marapiro, uye tichataura munyaya ino.

Mune zvimwe zviitiko, anoratidza kuvapo Dravet chirwere?

Dravet chirwere haisi zvakajairika - akacherechedza kuti chirwere ichi chiri pasi 1 munhu pa 40 zviuru vachangoberekwa (uye vakomana anoumba vanenge 66% of nyaya) .. Asi ichi, nenzira, zvinotungamirira chokwadi kuti zvakarurama nechirwere, vanachiremba dzimwe nguva zvakaoma kuisa uye, nokudaro, nguva inokosha akarasika. Zvino akadana chirwere pasina munyu kurapa hwomwana nemamiriro Kazhinji Zvinoipisisa nezera.

Kana pane mwana uyo haana gore, kugara tichibatwa vane Serial kana chinzvimbo mukati (kureva. E., Vanotevera mumwe uye murwere ari makwanza haapindi pfungwa) uyewo nemishonga kuitwa anticonvulsant zvinodhaka, anogona vakafungira urwere Drava.

Vana vane ichi vanowanzova kurwisa akawanda masikati, uye ichi pakunotora kweinenge vhiki. Ipapo takanga tisisanakidzwi kuti vaviri wamavhiki, uye zvose inodzokororwa zvakare.

Status epilepticus pamwe Dravet chirwere - a kumbonyanya inozivikanwa. Iye angaperekedzwa nokugwinha kana kuva dzisiri convulsive, yakasiyana zvikuru kuvhiringidza pfungwa pamwe segmental myoclonia (kutsanya tsandanyama twitching).

Dravet chirwere: zvinokonzera

The chikuru mhaka chirwere inotsanangurwa vaongorori vanoti nemagene kubatwa, zvinoti - kuvapo muna murwere yavaibvisa chiteshi Gene kunotouraya.

Sembura zvikonzero nokuti pakatanga mamiriro anorondedzerwa vacheche fivha kazhinji anoita pakurwara, kumbotura inopisa kugeza, overheating. Uyewo, zvinogona kwazvo kuneta kana kurudziro chiedza (kuvheneka nechiedza, kuchinja kubva mumba yerima kupinda zvakajeka yakavhenekerwa, uye zvichingodaro. N.). Zvinofanira kucherechedzwa kuti zvose izvi uye mumakore akatevera upenyu murwere achava nenjodzi, zvichiita kurwiswa-siyana simba nokuti.

Dravet chirwere: Zviratidzo

The chikuru kuratidzwa Dravet chirwere chinogona kunzi, uye ainyanya kukoshesa uye generalized pfari. Zvatichiri pfari dzinoratidzwa mukuti excitation nharaunda, afunge chiitiko kwavo, mumwe chete kuuropi. Kana pathological webasa Tutsinga tunofambisa mashoko utwu vari vaviri hemispheres chinhu generalized nepfari.

Nokugwinha muna Dravet chirwere kazhinji ndivo polymorphic zvakasikwa. Child pagore uinako clonic (pamwe kuchinja tsandanyama matauriro), tonic (izvo chaizvo refu tsandanyama spasm) uye generalized myoclonic pfari.

Kazhinji kune kurwisa muchimiro atypical tisipo - mamiriro umo hwomwana kuziva hakusi zvishomanana kana zvachose kuteerera kwezvakatipoteredza. The mbudzana anogona bindira panguva, akatarira imwe nguva, curved shure, pakarepo donhwe kana chete kusiya kuti mumaoko ake.

Kakawanda kutaurwa pfari munguva wakasvinura, uyewo ari kutsinzina mamiriro (muchiroto, ivo akanyorwa 3 chete% varwere nechirwere ichi).

Sei kuva zvekuchipatara kuratidzwa Dravet chirwere?

Kazhinji, Dravet chirwere kunoratidzwa ndechokuti yekunwa zviratidzo kuitika mune imwe kutevedzana. Doctors kusiyanisa matatu enguva huru dzehupenyu chirwere.

1. A pfupi munyoro nguva, pamwe zvinenge clonic pfari (kutsanya pfura, mumwe pashure pomumwe, pashure pfupi nguva). Sezvo inotokonya ezvinhu, sezvo mutemo inomirira tembiricha kuwedzera mwana, asi munguva yemberi vanogona simuka uye tisingatungamirirwi payo.
2. Achifumira hasha - pamwe veMubatanidzwa dzakawanda myoclonic pfari. kazhinji vane febrile (t. E. Zvichienderana tembiricha kuwedzera) uye zvinoshanda hunde uye migumo. For myoclonic pfari vanobatana atypical asipo pfari uye kunzwisisa zvatichiri pfari.
3. Kunofambira mberi nguva apo simba nokurwisa, apo mwana chinoramba chakakomba chetsinga uye vairwara nepfungwa.

The chikuru zviratidzo zvikuru myoclonic pfari muuduku

Sezvatakamboreva, tichifunga nezvokuti kuti Dravet chirwere - isingawanzowanikwi chirwere, penyanzvi kazhinji zviomere diagnosing nayo. Saka, vabereki kunokosha kupa ruzivo yakarurama pamusoro kukura pathological mamiriro mwana. Ainzi chirwere anogona vanofungidzirwa kana pane zvinowanikwa anotevera zvinhu:

• chirwere vafambira mberi gore rimwe zera;
• polymorphous pfari (.. kureva kuratidzwa dzavo siyana);
• Kurwisa haina kumborega kugamuchirwa zvemo anticonvulsants;
• panowanikwa pfari akasungirirwa kuwedzera hwomwana muviri tembiricha;
• mwana kuoneka zvokukura sokunonoka (chikamu ichi kungaratidzwa pakawanda);
• iziviswe ataxia zviratidzo (raskoordinirovannosti masangano);
• MRI uchapupu hazvirevi kuzosimbisa kuvapo chirwere (kunyanya pakutanga chirwere);
• Eeg - tava kuderera of kumashure mutinhimira uye multifocal kutyorwa akabvuma yakatesvera panyanga dzacho uye anononoka nemafungu.

Kuwedzera kune zviratidzo izvi vana vane Dravet chirwere kazhinji dzinoratidzwa pamberi wokusagadzikana uye kutarisirwa dhefisiti nyonganyonga.

Achatanda kuti pave Dravet urwere

Kufembera zvakakomba myoclonic pfari rinowanzova zvisingafadzi.. Varwere All nechirwere Dravet chirwere vakachengeta retardation mupfungwa, uye hafu chinoremedza. Pashure pemakore wevarwere mana pane mberi kudzikira, pamwe pave maitiro abnormalities, kusanganisira psychosis.

Zvinosuruvarisa ngozi panguva zvinorondedzerwa pathologies ndiyewo yakakwirira chaizvo - kusvika 18%, uye rinoita kuti tsaona dzakawanda kakawanda munguva pfari kana chinzvimbo epilepticus.

Kuti yekuzvidzivirira mutsekwende panguva kupambwa mwana, vabereki vanofanira kunzwisisa sei kupa rubatsiro wokutanga pfari zvakanaka.

How kupa ruyamuro kuti pfari inokonzerwa dzakakwirira tembiricha rokutanga?

Kana mwana ane pfari papiwa kuwedzera kupisa (iyo, sezvo ungayeuka, chirwere ichi ndicho chimwe chezvikonzero zvikuru zvinhu), chengetai mitemo zvinotevera:

• Kuisa mwana pamusoro sandara penyika;
• zvinopa mhepo yakachena;
• kuchenesa muromo mwana wacho madzihwa;
• adzorere mwana aruise rimwe divi;
• Shandisa antipyretic mabasa.

Kana mwana akaratidza fivha, ndiko kuti, inopisa pahuma yake, chiso chake Chiso chinopenya, ipapo rubatsiro kuti pfari yokutanga inofanira zvainanga achidzikisa tembiricha (chando nyoro tsonyera pahuma, inotonhorera muhapwa uye muhudyu, muviri rubdown nemvura uye vhiniga in nhamba 1 1, antipyretics).

Kana mbudzana pamusoro yaitwa yakakwirira tembiricha ganda riri rakachenuruka, miromo rinokwanisa kucheneruka, uye nzara, chills, tsoka inotonhora uye nezvanza, kuti rubdown uye kuchitonhora anorumanya hazvifaniri kuitwa. Warm kukambaira ngaape antipyretics, uye mahwendefa "No-Spa" kana "Papaverine" mwero 1 MG pa 1 kg muviri uremu vasodilatation.

Batsirai kana muAtlantic zvipusha pfari

Panguva chiitiko ane muAtlantic zvipusha nepfari pamwe generalized clonic uye tonic nokugwinha mwana anofanira kuva:

• akaisa pamusoro sandara penyika;
• pasi musoro komberedza chinhu dzakapfavirira, mwana haana kurwa naro;
• zvinopa manyoro mhepo yaunodya;
• kuchenesa muromo uye mitsipa madzihwa;
• dzokai musoro wako mumwe rutivi;
• kusonganira chero nhindi jira kuva fundo uye dzinoisa nayo pakati pameno kudzivirira dzichiruma rurimi uye miromo, sezvo mwana mumwe gore kungava hwakasimba chinhu (a chipunu, rutanda) kutyora meno ake;
• pukuta akazviratidza muromo furo tauro;
• nechokwadi chokuti munguva kurwisa mwana asingadi yakarova pane chinhu.

Kana nokugwinha kutora chimiro chinzvimbo, iva nechokwadi kudana "amburenzi."

Basic misimboti kurapwa vana vane Dravet kuti urwere

sick kurapwa mwana zvirwere zvinorondedzerwa deceleration ipfupi kudzivirira pave pfari vavo mamiriro fomu.

Apo chirwere kurapa "Dravet chirwere" inosiya kushandiswa kuzivikanwa antiepileptic zvinodhaka: "Carbamazepine", "Finlepsin", "Phenytoin" uye "lamotrigine" sezvo ivo chete achiipa murwere ezvinhu, Chinowedzera mukati yaivapo siyana pfari.

Kuwedzera kurapwa fanira kwezvinodhaka, zvakakosha kuyeuka kudzivisa fivha, sezvo mamiriro kunyanya ngozi kumurwere. To siya kutsamwisa kurwisana chiedza dzishande rakapiwa kupfeka magirazi mitema lenses kana mumwe taped rinoonekera.

Dravet urwere Mishonga Yokurapa

Kana nechirwere akatsinhira, kurapwa rinotanga kutanga zvinodhaka "Topiramate". Zvinonzi ushandiswe dose kuti 12,5 MG / zuva., Zvishoma nezvishoma vachiwedzera kuti 3-10 MG / makirogiramu / zuva. (Medicine akatorwa kaviri pazuva). chokushandisa Izvi kunyanya anobatsira Muzviitiko apo vana akatsanangura pfari rinowanika generalized pfari uye paroxysms pakati nokugwinha pamwe nokuchinja kubva hafu muviri mumwe (gemikonvulsiyami).

Zvinotevera Formulations kuti monotherapy - rinobva pane valproic asidhi (manyuchi "Konvuleks", "Konvulsofin", etc ...) - iri abudirire pane atypical tisipo uye myoclonus, uye barbituric asidhi ( "Phenobarbital") rakashandiswa generalized nokugwinha pamwe propensity kuti chinzvimbo ano. Sezvineiwo, muchiitiko ichi, uye kuzivikanwa yakakwirira kunyatsoshanda kuti bromides.

Kana zvakakodzera, shandisa mubatanidzwa zvinodhaka. Inonyatsobudirira vavo kunosanganisira valproate ne rinoreva "topiramate".