Lennox-Gastaut Syndrome (LGS) ni iki? Reka tubiganireho!

Ese umwana wawe akunze kugira igicuri? Ese agira impinduka zikomeye mu myigire no mu myitwarire? Niba ari uko bimeze, icyo ngiye kuvuga gishobora kuba ingenzi cyane kuri wewe. Uyu munsi tugiye kuvuga kuri Lennox-Gastaut Syndrome , cyangwa (LGS) mu magambo ahinnye, indwara ikomeye kandi igoye kuvura igicuri. Ibi bishobora gutangira akiri muto. Ariko ntugahangayike, imiti mishya n'uburyo bwo kuvura ubu birahari.

Ese koko indwara ya Lennox-Gastaut syndrome (LGS) ni iki? Ni nde uyirwaye?

Muri make, LGS ni ubwoko bukomeye bw'igicuri gitangira mu bwana, akenshi mbere y'imyaka 10, cyane cyane hagati y'imyaka 3 na 5. Ikunze kugaragara gato ku bahungu. Ariko, si indwara ikunze kugaragara cyane. Ifata abantu bagera kuri umwe kugeza kuri babiri muri miliyoni. Hagati ya 1% na 2% by'abarwayi bose b'igicuri bafite LGS.

Ibi bigira ingaruka zite ku mibiri yacu?

Igicuri ni indwara aho ibimenyetso by'amashanyarazi cyangwa amakuru yo mu bwonko bikwirakwira hagati y'uturemangingo. (LGS) ni ubwoko bw'igicuri bukabije . Hari impamvu nyinshi:

• Muri LGS, havuka ubwoko burenze bumwe bw'indwara zo mu mutwe. Bumwe muri ubwo bwoko bw'indwara zo mu mutwe bushobora gutera umwana imvune ikomeye.
• Izi ndwara zishobora kwangiza ubwonko ubwabwo. Abana bafite LGS bashobora kwangirika cyane mu bwonko, bakagira ibibazo mu myigire, ndetse bagatinda mu mikurire. Ibi bivuze ko umwana ashobora gutakaza cyangwa kwibagirwa ibintu yamaze kumenya, nko kugenda no kuvuga. Iki ni ikibazo kibabaje cyane.
• (LGS) ubusanzwe irarwanya cyane kuvurwa. Ibi bivuze ko bigoye kuyigenzura ukoresheje imiti. Imiti ni yo nzira ya mbere yo kuvura, ariko akenshi ubwoko bwinshi bw'imiti butangwa icyarimwe. Nubwo yatangwa, imiti yonyine ntabwo ibasha guhagarika burundu indwara zo mu mutwe.

Ni ibihe bimenyetso bya (LGS)?

Umwana urwaye LGS ashobora kugaragaza ibimenyetso bitandukanye. Bimwe muri byo bigaragara mu buzima bwa buri munsi no mu bikorwa bye, mu gihe ibindi bigaragara gusa mu gihe cy'igicuri.

Ibibazo byo kwiga no kwitwara neza

Ni ibisanzwe ko abana bafite LGS bagira ibibazo byo kwiga no kugira ibibazo by’imyitwarire . Ibi bishobora kugaragara nyuma gato y’uko indwara y’igicuri itangiye, kandi bishobora kwiyongera uko indwara y’igicuri ikomeza. Ibi bibazo byo kwiga no mu bwenge akenshi bibaho burundu, bivuze ko bishobora gukomeza no mu gihe cy’ubukure.

Abana bafite LGS bagorwa no kwiga, kugira inshuti no kuvugana n'abandi. Bashobora kandi kugorwa no kugenzura amarangamutima yabo. Hari abana bashobora no kugaragaza ibimenyetso bisa n'iby'abana bafite Autism Spectrum Disorder .

Icyiciro cyo gufatwa n'indwara

Indwara y'igicuri ishobora kubaho mu byiciro bitatu:

1. Prodrome cyangwa aura: Iki ni ikintu bamwe bahura na cyo mbere yo kugira igicuri, nubwo atari buri gihe. Ibi bishobora kuba ibyiyumvo bibi. Impinduka mu byo umuntu abona, impinduka mu bindi bice by'umubiri, cyangwa impinduka mu byiyumvo cyangwa mu myitwarire zishobora kubaho muri iki gihe.

2. Gufatwa n'indwara yo mu mutwe: Aha niho umuntu afatwa n'indwara yo mu mutwe. Ibi bishobora gutandukana bitewe n'ubwoko bw'indwara yo mu mutwe (turaza kuganira ku bwoko butandukanye bw'indwara yo mu mutwe hepfo).

3. Gukira nyuma yo gufatwa n'icyorezo: Iki ni cyo gihe umuntu asubirana ubwenge bwe bwose nyuma yo gufatwa n'icyorezo.

Ubwoko bw'indwara zo mu mutwe zigaragara muri LGS

Hari ubwoko bwinshi bw'ingenzi bw'indwara zo mu mutwe zigaragara muri LGS. Ubwoko busanzwe bw'indwara zo mu mutwe n'ingaruka zazo ni ubu bukurikira:

• Indwara zo mu mutwe ziterwa n'indwara ya atonic:
• Kubura ubushobozi bwo kugenzura imitsi/gucika intege: Ibi byitwa kandi "igitero cyo kugwa" kuko bijyana no kubura ubushobozi bwo kugenzura no kugwa hasi, nk'igipupe gifite imigozi yacitse. Ibi ni bibi cyane kuko umwana nta bubasha afite bwo kugenzura iyo aguye kandi ashobora gukomereka bikomeye, ndetse no mu kaga, ku mutwe, mu ijosi, mu gituza no mu mugongo.
• Gutakaza igice cy'ubwenge: Iyi ndwara ishobora kudatuma umuntu atakaza ubwenge burundu.
• Igihe gito: Izi ndwara zimara igihe gito cyane, nk'amasegonda make.

• Kurwara indwara zo mu mutwe:
• Gukomera k'umubiri wose/gukonja: Iki ni cyo gihe imitsi yose yo mu mubiri ihita yifata, bigatuma umubiri ukomera rwose nk'aho wahindutse ibuye.
• Uburyo bwihariye bwo kwifata: Iyo bari hasi, amaguru n'amatugu byabo biri hasi, imigongo yabo ishobora gupfunyika. Amaboko ashobora kuramburirwa hejuru y'imitwe yabo, cyangwa amaboko agapfunyika kandi inkokora zerekeza ku mpande, kandi ibipfunsi bishobora kuba hafi y'ikibuno.
• Akenshi bibaho mu gihe cyo gusinzira: Ibi bikunze kubaho mu gihe cyo gusinzira. Ariko bishobora no kubaho ku manywa. Ibi bibaye umwana akiri maso, hari ibyago byo gukomereka kuko umwana ashobora kugwa.
• Igihe gito: Ibi kandi bimara amasegonda make kugeza ku munota umwe.

• Indwara zidasanzwe zo kubura ubumuga:
• Amaso arafunguka areba nta cyo atekereza: Muri uku gucika intege, ubwenge burabura, ariko amaso agakomeza kuba maso. Kubwibyo, ibi akenshi bishobora kwitiranywa no kurota ku manywa, kutagira icyo umuntu yibandaho, no kumva ko atari ahantu heza.
• Ingendo zisubiramo: Muri iki gihe cyo gukubita, ushobora kubona ingendo z'amaso n'umunwa. Hashobora kandi kubaho ingendo z'amaboko.
• Igihe gito: Ibi kandi ntibimara amasegonda make.

Icy'ingenzi: Abana barwaye LGS bashobora kugira ibisebe mu "matsinda," bivuze ko ibisebe byinshi bibaho mu gihe gito. Abana barenga bibiri bya gatatuIndwara yitwa "Status Epilepticus" ibaho. Iyi ni indwara isaba ubuvuzi bwihutirwa!

Indwara ya Status Epilepticus ni iki?

Ibi bivuze kimwe mu bintu bibiri:

• Indwara imwe, imara igihe kirekire, imara hagati y'iminota 5 na 30.
• Indwara zirenze imwe mu minota 30, kandi umuntu ntagarura ubwenge bwuzuye muri iyo ndwara.

Indwara y’igicuri ni ikibazo cyihutirwa cy’ubuvuzi. Ni ikibazo gikomeye cyane kuko gishobora kwangiza ubwonko burundu ndetse no gupfa.

Ibindi bimenyetso n'ubwoko bw'indwara z'icyorezo bifitanye isano

• Ibisebe bya Tonic-clonic: Ibisebe bya Tonic-clonic (byahoze byitwa "grand mal") bitangira kimwe n'ibisebe bya tonic, ariko bifite ikindi cyiciro. Icyiciro cya clonic ni igihe umubiri uhuha, ari na cyo kintu gikunze kugaragara muri ibyo bisebe. Ibisebe bya Tonic na Tonic-clonic akenshi bifata iminota ibiri.
• Ingendo zihuse, ngufi kandi zihuta: Ingendo z'imitsi ni ingendo zitunguranye kandi zihuta. Zimara amasegonda make gusa, ariko rimwe na rimwe zishobora kumara igihe kirekire. Abantu badafite inzendo bashobora no kugira ubwo bwoko bwa "myoclonic jerks." Urugero, zishobora gutera gucika intege cyangwa zikanguka mu buryo butunguranye bivuye mu bitotsi.
• Ibimenyetso by'amarangamutima, ibyo mu mutwe, cyangwa ibindi bimenyetso: Indwara z'ibice bimwe cyangwa iz'ibice byibasira igice gito cy'umubiri gusa.

Ni izihe mpamvu zitera LGS?

Hari impamvu nyinshi zishobora gutera Lennox-Gastaut syndrome. Zimwe mu mpamvu ziba mbere y'uko umwana avuka. Izindi ziterwa n'ibintu bibaho mu bwana. Mu bantu bagera kuri kimwe cya kane barwaye ``(LGS)``, nta mpamvu isobanutse neza ishobora kumenyekana.

Dore zimwe mu mpamvu zagaragajwe:

• Ibibazo bijyanye n'iterambere ry'ubwonko mbere yo kuvuka.
• Ibikomere bikomeye byo mu mutwe (ibikomere byo mu bwonko cyangwa kuribwa mu bwonko).
• Indwara zangiza ubwonko, nka meningite cyangwa encephalitis.
• Indwara z'imikorere y'umubiri zarazwe, urugero `(indwara za Mitochondria)`.
• Kwangirika k'ubwonko guterwa no kubura umwuka wa ogisijeni mbere cyangwa mu gihe cyo kuvuka (hypoxia yo mu bwonko).
• Indwara ya sclerosis y'ibibyimba.
• Ibibyimba byo mu bwonko.
• Indwara y'Uburengerazuba (iyi ni indi ndwara y'igicuri, ishobora kuvamo indwara ya LGS).

Abashakashatsi basanze "mutations de novo" ikunze kugaragara mu bantu bafite LGS.De novo bisobanura "kuva mu ntangiriro." Izi ni inenge ziri muri `(DNA)` y'umuntu zibaho mu buryo butunguranye, ku buryo butunguranye. Ni ukuvuga ko iyo miterere iterwa n'inenge iri muri `(DNA)` y'intanga cyangwa igi ryakoze uwo muntu, ariko si inenge iri muri `(DNA)` y'umwe mu babyeyi bombi.

Ariko, hashobora kuba hari ingaruka ku miterere y’umubiri kuri iyo ndwara. Abantu bagera kuri 30% barwaye LGS bafite amateka yo mu muryango wabo yo kurwara igicuri. Ariko, hakenewe ubushakashatsi bwinshi kugira ngo hamenyekane neza ingano y’iyi ndwara.

Ese ibi byandura?

Oya, Lennox-Gastaut syndrome ntabwo yandura. Ntabwo yandurira mu muntu umwe ijya ku wundi. Ariko, zimwe mu ndwara zandura zishobora kwangiza ubwonko no gutera "LGS". Ariko, si buri wese ufite izo ndwara uzarwara "LGS".

Ni gute indwara ya Lennox-Gastaut syndrome (LGS) ipimwa?

Ubusanzwe hakorwa ibizamini byinshi kugira ngo hamenyekane indwara ya LGS, kandi muganga azasuzuma indwara z’imitsi. Umuganga w’abana ashobora gukeka ko iyo ndwara ishingiye ku byo ababyeyi bavuga ku bimenyetso by’umwana wabo, cyane cyane iyo umwana afite ubumuga bwo kwiga cyangwa ibibazo by’imyitwarire.

Ni ibihe bipimo bikorwa kugira ngo hamenyekane ibi?

Ibizamini bikunze gukoreshwa cyane ni ibi bikurikira:

• Electroencephalogram (EEG): Ibi bikubiyemo gushyira electrode nyinshi ku mutwe hamwe na gel iyobora kugira ngo ipime imikorere y'amashanyarazi y'ubwonko. EEG ni ingenzi mu gusuzuma LGS kuko igaragaza imiterere yihariye y'amashanyarazi mu miraba y'ubwonko.
• Ishusho ya Magnetic Resonance (MRI): Iyi shusho ikoresha rukuruzi ikomeye na mudasobwa kugira ngo ifate amashusho meza cyane y’imbere mu mubiri, cyane cyane ubwonko. Ibi bifasha cyane mu gutahura ibibazo biri mu mikurire y’ubwonko.
• Gupima muri laboratwari: Ibi bizamini bisuzuma amaraso n'inkari kugira ngo harebwe niba hari izindi mpamvu zishobora gutera iyi ndwara. Bireba ibintu nk'imiti y'amaraso, enzymes z'umwijima, n'imikorere y'impyiko. Nubwo ibi bizamini bidashobora gusuzuma LGS burundu, bishobora gufasha gukuraho izindi ndwara zishobora kuba zisa na LGS.
• Gupima imiterere y’uturemangingo: Ibi bishobora gufasha kumenya niba hari indwara umuntu avukana zishobora gutera LGS, cyangwa niba hari impinduka zitunguranye.

Byongeye kandi, hari ibindi bizamini bishobora gukorwa kugira ngo hatabaho indwara nk'izo. Muganga wawe azakubwira byinshi kuri ibi.

Ibi bishobora kuvurwa gute? Ese bishobora kuvurwa?

Hari uburyo bwinshi bwo kuvura LGS. Ariko, iyi ndwara iragoye kuyivura. Kenshi na kenshi, bumwe cyangwa uruvange rw'ubu buryo bwo kuvura rushobora gukoreshwa:

• Imiti
• Uburyo bwo kuvura imirire mibi
• Kubaga ubwonko cyangwa ibikoresho byatewe

Imiti

Indwara ya Lennox-Gastaut ikunze kugorana cyane n'imiti. Niyo mpamvu bigoye kuyivura. Kenshi na kenshi, hakenewe imiti myinshi. Ikindi kintu kigoye ni uko imwe mu miti igabanya ubukana bw'indwara zo mu mutwe, igenzura ubwoko bumwe na bumwe bw'indwara zo mu mutwe, ishobora kongera inshuro nyinshi z'izindi ndwara zo mu mutwe.

Iyo indwara idakira kuvurwa, abaganga bashobora kugerageza gukumira ubwoko bumwe na bumwe bw'indwara zo mu mutwe cyangwa kugabanya inshuro zikunze kugaragara. Urugero, indwara zo mu mutwe ni ingenzi cyane mu kuzirinda, kuko zifitanye isano n'ibyago byinshi byo gukomereka bitewe no kugwa.

Uburyo bwo kuvura imirire mibi

Ku bantu benshi bafite igicuri gikomeye, guhindura imirire bishobora gufasha kugabanya inshuro zo gufatwa n’igicuri cyangwa kubihagarika burundu. Indyo ya ketogenic ku igicuri - indyo irimo poroteyine nyinshi n’ibinure n’ibinure bike - ikunze kugirwa inama n’abaganga. Ishobora kuba amahitamo meza iyo imiti itagize icyo ikora.

Nubwo indyo ya ketogenic ari uburyo buzwi bwo kuvura igicuri gikomeye, ifite ibibazo bike. Iyi ndyo ikora gusa iyo uyikurikije neza. Ibi biterwa nuko kurya karubohidrati nyinshi, nk'isukari n'ibiryo birimo ibinyampeke, bishobora gutera indwara yo kurwara. Ariko, izindi ndyo zahinduwe nazo zishobora gufasha. Niba ubyifuza, muganga wawe ashobora kugufasha kugerageza imwe muri izi ndyo.

Kubaga ubwonko n'ibikoresho byatewe mu bwonko

Hari ubwo, cyane cyane iyo ubundi buryo bwo kuvura butagize icyo bugeraho, kubaga ni bwo buryo bwiza kurusha ubundi. Dore ubwoko bumwe na bumwe bwo kubaga:

• Gukangura imitsi yo mu nda (VNS): Ibi bikubiyemo gushyira igikoresho mu mubiri gitanga umuriro woroheje w’amashanyarazi ku mutsi wa 10 w’umutwe, umutsi wo mu nda - ufitanye isano itaziguye n’ubwonko. Uyu mutsi ushobora kugabanya inshuro n’ubukana bw’indwara zo mu nda. Uko igihe kigenda gihita, ingaruka zabyo ziyongera. Ibi bivuze ko indwara zo mu nda zigabanuka kandi indwara zo mu nda zigacika cyane. Gutera iki gikoresho ni uburyo bushobora gusubizwa inyuma, bufite ingaruka nke kandi bugakira igihe gito, bityo akenshi butekerezwaho mbere yo kubagwa cyane.
• Corpus callosotomy: Corpus callosum ni igice cy'ubwonko gikora nk'ikiraro gihuza ibice by'ibumoso n'iburyo. Iyo corpus callosum icitse, ibimenyetso bidasanzwe bitera igicuri birahagarara. Ibi bishobora kugabanya inshuro zo gukubita, cyangwa se bigahagarika bikomeye, bigatuma igicuri kidakora neza burundu.
• Hemispherectomy `(Hemispherectomy)`:Ibi bikubiyemo kubagwa uruhande rumwe rw'ubwonko. Nubwo ibi bishobora gusa n'ibikabije, abahanga bashobora kubikoresha kugira ngo bahagarike indwara zo mu bwonko zidakira. Nyuma, ubuvuzi bw'umubiri bushobora gufasha ubwonko kumenyera impinduka. Ubwonko burongera bukagena, hanyuma igice gisigaye kigakora imirimo y'igice cyakuweho.
• Gucamo ibice: Hari ubwo indwara zishobora gutera LGS. Niba abaganga basanze hari ikibazo nk'icyo, bashobora gukuraho ingingo z'ubwonko zangiritse cyangwa zirwaye no guhagarika indwara zo gucika intege. Ariko, kubera ko muri LGS, ibikorwa byo gucika intege biba mu bice byinshi by'ubwonko, ubusanzwe iyi si yo nzira nziza cyane kuri LGS.

Ingaruka mbi/ingaruka mbi zo kuvurwa

Ingorane zo kuvurwa ziba zihariye kuri buri buryo bwo kuvurwa. Kubwibyo, ni byiza kuganira na muganga wawe ku ngorane n'ingaruka mbi zo kuvurwa wowe cyangwa umwana wawe muhabwa. Hanyuma muganga ashobora kuguha amakuru ajyanye n'uburwayi bwawe, imimerere yawe, n'izindi ndwara.

Nzumva meze neza nyuma y'igihe kingana iki? Bizatwara igihe kingana iki kugira ngo ngire gukira?

Igihe cyo gukira nyuma yo kuvurwa gitandukana cyane bitewe n'uburyo bwo kuvura bwakoreshejwe ndetse n'uko umerewe muri rusange. Muganga wawe ni we muntu mwiza wo kukubwira neza icyo ugomba kwitega n'igihe bizatwara kugira ngo uruhuke.

Ese indwara ya Lennox-Gastaut syndrome (LGS) ishobora kwirindwa?

Kenshi na kenshi, LGS si indwara ishobora kwirindwa. Nta n'ubwo ibyago byo kuyirwara bishobora kugabanuka. Ikintu kimwe cyonyine kidashoboka ni ukwirinda imvune zo mu mutwe n'ubwonko zishobora guterwa na LGS. Uburyo bwonyine bwo kwirinda iyi ndwara ni ukwambara ingofero zemewe kandi zemewe.

Ni iki nkwiye kwitega mu gihe umwana wanjye cyangwa njye mfite iyi ndwara?

Lennox-Gastaut syndrome ni indwara ikunze kugira ingaruka mbi cyane. Abana benshi bafite iyi ndwara bagira ubumuga bwo kwiga no kwangirika burundu k'ubwonko bigabanya ubushobozi bwabo bwo gutekereza. Nanone, kubera ko indwara zo mu mutwe zikunze kugaragara, kandi akenshi zigatera kugwa, abana bafite iyi ndwara baba bafite ibyago byinshi byo gukomereka.

Iyi ndwara nayo iragoye kuyivura. Abana benshi bayirwaye bakeneye kwitabwaho n’inzobere ubuzima bwabo bwose kuko badashobora kwiyitaho. Ariko, hari abantu bafite iyi ndwara bagira ikibazo cyoroheje mu bwonko, bivuze ko bashobora kubaho mu buryo bwihariye.

(LGS) imara igihe kingana iki?

Keretse hatanzwe umuti wo guhagarika burundu indwara yo gucika intege (ibi bikaba bidakunze kubaho), LGS ni indwara ibaho ubuzima bwose.

Ni iki giteganyijwe kuri iki kibazo?

Ingaruka zikomeye zo kurwara Lennox-Gastaut syndrome ni ukwangirika k'ubwonko bitewe n'igicuri kidacunzwe, cyangwa kugwa bitewe n'igicuri. Kubera izo ngaruka, igipimo cy'impfu ku bantu barwaye "LGS" mu myaka 10 nyuma yo kumenyekana kiri hagati ya 3% na 7%.

Hari ubwo uburyo bwo kwa muganga bushobora guhagarika indwara yo gucika intege iterwa na LGS. Ibi bihagarika iyo ndwara, ariko ntibishobora gusana ibyangiritse mu bwonko byatewe n’indwara zo gucika intege zabanje.

Nikwitaho nte? / Ni gute nakwitaho umwana wanjye?

Niba wowe cyangwa uwo ukunda afite LGS, ni ngombwa cyane gukurikiza amabwiriza ya muganga wawe neza. Ibi birimo:

• Gufata imiti nk'uko byategetswe.
• Kujya kwa muganga nk'uko byasabwe.
• Gukurikirana ibicurane no kwirinda ibintu bishobora gutera ibicurane.

Ni ryari ngomba kubonana na muganga?

Muganga wawe cyangwa uw'umwana wawe azakubwira kuza gusura muganga wawe uko bikenewe. Ugomba kandi kubonana na muganga wawe niba ibimenyetso by'igicuri bihindutse cyane cyangwa niba bigira ingaruka ku buzima bwawe bwa buri munsi n'imirimo yawe.

Ni ryari nkwiye kujya mu Ishami ry’Ubuvuzi bw’Ingoboka (ETU) ?

Niba wowe cyangwa umwana wawe mufite isereri cyangwa indwara nyinshi zujuje ibisabwa kugira ngo habeho indwara yihutirwa yitwa status epilepticus , mugomba kujya mu cyumba cy’ubutabazi bw’ibanze ku bitaro biri hafi yanyu. Ibi bivuze ko mu bihe bikurikira:

• Iyo icyorezo kimwe kimaze hagati y'iminota 5 na 30.
• Iyo umuntu afashwe n'indwara ebyiri cyangwa nyinshi mu minota 30, ntagarure ubwenge mu gihe cy'indwara.

Ubutumwa bwo kujyana mu rugo

Lennox-Gastaut syndrome (LGS) ni ubwoko bukomeye bw'igicuri gitangira hakiri kare. Akenshi gishobora kugira ingaruka zikomeye, rimwe na rimwe mu buzima bw'umwana. Nubwo bigoye kuvura, imiti mishya, kubagwa, n'uburyo bwo kuvura bishobora gufasha kugabanya ubukana bw'indwara no kugabanya ikwirakwira ry'indwara. Ni gake cyane, ubuvuzi bushobora gukiza burundu iyo ndwara. Ariko, hakenewe ubushakashatsi bwinshi kugira ngo hamenyekane niba iyi ndwara ishobora kuvurwa cyangwa igakira. Niba umwana wawe afite kimwe muri ibi bimenyetso, ni byiza kujya kwa muganga vuba bishoboka.

Lennox -Gastaut syndrome, LGS, igicuri, ibibyimba, abana, indwara zo mu bwonko, indwara z'imitsi