Ingabe uhlangothi olulodwa lobuchopho bomntwana wakho lukhulu kakhulu? Ake sifunde nge-hemimegalencephaly!

Wake wakhathazeka ngesimo sekhanda lengane yakho, noma ngokuguguleka kwayo njalo? Ngezinye izikhathi, kungase kube nesifo esingavamile kakhulu ngemuva kwalezi zinto. I-Hemimegalencephaly ingenye yalezi zimo. Nakuba igama lingase lizwakale liyinkimbinkimbi kancane, ake sixoxe ngakho kalula.

Kuyini i-Hemimegalencephaly?

Kalula nje, i-hemimegalencephaly yilapho uhlangothi olulodwa lobuchopho bengane yakho (i-cerebral hemisphere) lukhulu ngokungavamile kunolunye. Cabanga ngobuchopho bethu njengesimila esikhulu esinezingxenye ezimbili, uhlangothi lwesobunxele nolwesokudla. Kulesi simo, uhlangothi olulodwa kuphela olukhula lube lukhulu kunalokho okufanele lube yikho. Ngezinye izikhathi lokhu kukhula kungakhawulelwa engxenyeni encane yalolo hlangothi lobuchopho , noma lonke uhlangothi lungakhula.

Uma uhlangothi olulodwa lobuchopho lukhula ngale ndlela, ezinye izinguquko zingavela ngaphakathi kobuchopho. Isibonelo:

• Ama-neurons awuhlobo lweseli ebuchosheni bethu. Angase angahlelwanga kahle (cytoarchitecture) noma ungqimba oluphezulu lobuchopho lungase lungakhiwanga kahle (cortical dysplasia).
• I-corpus callosum elahlekile noma eyonakele: Lokhu kufana nebhuloho elixhumanisa izinhlangothi ezimbili zobuchopho . Uma ilahlekile noma yonakele, kungavela izinkinga.
• Ukwanda kwemigodi egcwele uketshezi (ama-ventricle) yobuchopho ohlangothini oluthintekile.

Ngenxa yokuthi izingxenye zobuchopho azikhuli kahle ngale ndlela, ukuxhuzula kungavela njalo. Siphinde sikubize lokhu ngokuthi ukuxhuzula . Ngokuvamile, lokhu kuxhuzula kunzima ukukulawula ngemithi ejwayelekile yokulwa nokuxhuzula. Uma ukuxhuzula kungenakulawuleka ngemithi, izingane eziningi kufanele zihlinzwe ubuchopho (ukuhlinzwa kokuxhuzula).

Ingabe zikhona izinhlobo eziyinhloko ze-hemimegalencephaly?

Yebo, kunezinhlobo eziningana eziyinhloko zalesi simo:

• I-hemimegalencephaly ehlukanisiwe: Lokhu kuthinta kuphela ungqimba lwangaphandle lobuchopho bengane (i-cerebral cortex). Akukho ukubandakanyeka kwesikhumba noma ezinye izitho zomzimba.
• I-syndromic hemimegalencephaly: Lokhu akuthinti nje ubuchopho kuphela, kodwa futhi nesikhumba sengane kanye nezinye izitho zomzimba.Izimpawu zesikhumba zingase zivele ezinyangeni noma ngisho neminyaka ngemva kokuzalwa. Futhi, uhlangothi olulodwa lomzimba womntwana lungase lube lukhulu kunolunye (hemihypertrophy).
• I-hemimegalencephaly ephelele: Kulokhu, i-cerebellum yengane futhi ngezinye izikhathi ngisho ne-brainstem iyakhula. Lokhu kungenzeka kungakhathaliseki ukuthi kuhlukanisiwe noma kuhambisane nezimpawu.

Ziyini izimpawu zalesi simo?

Uphawu lokuqala ongase ulubone ukuthi ingane yakho ine-"spasms noma i-seizures " ezinsaneni. Lokhu kuvame ukuba yi-focal seizures. Lokhu kusho ukuthi uhlangothi olulodwa kuphela lomzimba womntwana olunganyakazisa.

Ezinye izimpawu ezingase zibonakale zifaka:

• Ikhanda elikhulu (i-Macrocephaly).
• Isimo sekhanda esingalingani.
• Ubunzima bokunyakaza okufana nokuhamba nokugijima.
• Ukuba ndikindiki nobuthakathaka ohlangothini olulodwa lomzimba.
• Ukulibaziseka kokukhula: Lokhu kusho ukuphuza ukukhuluma, ukudlala, nokufunda ngendlela efanele iminyaka yabo.
• Ukukhubazeka kokubona: Isibonelo, ingxenye yobubanzi obubonakalayo iyalahleka uma ubheka ngamehlo womabili (i-hemianopia).

Yini ebangela i-hemimegalencephaly?

Eqinisweni, ososayensi abakakazi kahle ukuthi yini ebangela lesi simo. Abanye bakholelwa ukuthi sibangelwa ufuzo, okusho ukuthi sibangelwa ushintsho ku -DNA yengane. Kodwa akulona ufuzo. Lokho kusho ukuthi unalesi simo futhi akuyona into oyidlulisela enganeni yakho.

Cishe, cishe ngesonto lesithathu lokukhulelwa, kwenzeka ushintsho olungahleliwe, noma oluzenzakalelayo, kwenye yezakhi zofuzo ezibalulekile ekukhuleni kobuchopho. Khumbula, lesi simo asibangelwa yinoma yini oyenzile ngesikhathi sokukhulelwa. Lokhu akulona iphutha lakho noma likababa.

Yiziphi izinto eziyingozi ezithinta lesi simo?

I-hemimegalencephaly ingenzeka kanye nezinye izimo zofuzo. Lokhu kusho ukuthi izingane ezinezimo ezilandelayo zisengozini enkulu yokuthola i-hemimegalencephaly:

• 'I-Epidermal nevus syndrome'
• Isifo i-Klippel-Trenaunay
• 'I-McCune-Albright syndrome'
• "I-Neurofibromatosis Uhlobo 1 (NF1)"
• `Isifo se-CLOVES`
• 'I-Proteus syndrome'
• `I-Hypomelanosis ye-Ito`

Itholakala kanjani i-hemimegalencephaly?

Ukuze athole ukuthi lokhu kunjalo yini, udokotela wengane yakho uzoqala ahlole ingane yakho bese ekubuza ngomlando wempilo yengane yakho. Ngemuva kwalokho, bangenza izivivinyo ezilandelayo:

• Iskeni se-MRI yobuchopho.
• Ukuhlolwa kwe-EEG (i-Electroencephalogram). Lokhu kulinganisa umsebenzi kagesi wobuchopho.

Ngobuchwepheshe obuthuthukisiwe banamuhla, ngezinye izikhathi kungenzeka ukuthola lesi simo ngenkathi ingane isesesibelethweni "ngesikhathi sokukhulelwa." Lokhu kwenziwa ngokwenza i-MRI scan ekhethekile yobuchopho bomntwana ethi "fetal MRI".

Iphathwa kanjani?

Umgomo oyinhloko wokwelapha i-hemimegalencephaly ukulawula ukuquleka. Lokhu kuquleka ngezinye izikhathi kungaqala esemncane kakhulu futhi kuvame ukuba nzima ukukulawula ngemithi (engazweli emithini).

Lesi simo singabangela ukuguguleka okuhlukahlukene. Kubalulekile ukuqaphela ukuthi ukuguguleka okwenzeka ebuntwaneni, ikakhulukazi uma kuqala ebuntwaneni, kungaba nomthelela omkhulu ekukhuleni kwengane. Ukuguguleka okungalawulwa kungalimaza kakhulu ukukhula nokukhula kwengane, kanye nokulimaza ubuchopho obusakhula. Ngakho-ke, kubalulekile ukuyeka ukuguguleka ngokushesha okukhulu.

Ukwelashwa kwesifo sokuwa kuqala ngemithi yokulwa nokuwa. Ezinye izingane zingalawula ukuwa kwazo impilo yazo yonke ngale mithi. Kodwa eziningi ziba nesifo sokuwa esingamelani nemithi. Bese kwenziwa ukuhlinzwa okubizwa ngokuthi i-hemispherectomy. Lokhu kuhilela ukususa ingxenye yobuchopho bomntwana ethintekile (i-cerebral cortex) noma ukuyihlukanisa nolunye uhlangothi lobuchopho.

Uma kwenziwa lokhu kuhlinzwa, ngezinye izikhathi kubizwa ngokuthi "i-functional hemispherectomy." Kulokhu kuhlinzwa, udokotela ohlinzayo usika futhi ahlukanise izicubu nezinzwa ezixhumanisa uhlangothi olulodwa lobuchopho bengane kolunye, kodwa uhlangothi lobuchopho olukhuliswe ngendlela engavamile lungashiywa ngaphakathi kwekhanda. Ku-"anatomical noma i-hemispherectomy ephelele," uhlangothi olulodwa lobuchopho luhlukaniswe kolunye uhlangothi, lususe uhlangothi olungavamile ngokuphelele. Lokhu kuhlinzwa kufanele kwenziwe udokotela ohlinzayo wezinzwa oqeqeshwe ngokukhethekile ekuhlinzeni isithuthwane.

Ingabe ukuhlinzwa kokususa ingxenye yobuchopho (i-hemispherectomy) kunzima?

Eqinisweni, i-hemispherectomy iwukuhlinzwa kobuchopho okunzima kakhulu. Kunezizathu eziningana zokuthi kungani ukwenza lokhu kuhlinzwa kunzima nakakhulu ngenxa yesimo se-hemimegalencephaly.

Into yokuqala ukuthi imithambo yegazi ivame ukutholakala ngendlela engavamile. Ngakho-ke, kunzima ukuyithola nokuyisika ngesikhathi sokuhlinzwa. Lokhu ngezinye izikhathi kungabangela ukopha ngokweqile.

Ngaphezu kwalokho, ngenxa yokuthi ubuchopho bonke bengane buyakheke ngendlela engajwayelekile, izimpawu ezisetshenziswa udokotela ohlinzayo ukuhlonza izingxenye zobuchopho ngokuvamile azibonakali. Futhi, izinsana nezingane ezincane zingalahlekelwa yigazi eliningi phakathi nalokhu kuhlinzwa. Lokhu kumele kuqashwe ngokucophelela futhi igazi kumele lishintshwe njengoba kudingeka.

Ngakho-ke, kubaluleke kakhulu ukuthi ithimba lezokwelapha elilapha ingane yakho libe yithimba elinekhono nelinolwazi kulokhu kuhlinzwa. Kuphephile ukuthi ingane yakho yenze lokhu kuhlinzwa esibhedlela esenza njalo i-hemispherectomy yezingane ezine-hemimegalencephaly futhi esinolwazi oluningi kule nqubo.

Iyini inkomba yokubikezela kwengane enalesi simo?

Indlela isimo sengane yakho esizokhula ngayo incike kakhulu ekutheni ingalawulwa kahle kangakanani indlela efanelekile nokuthi ukukhula kobuchopho okungavamile kuhlangothini olulodwa noma zombili.

I-Hemimegalencephaly yisimo se-spectrum esithinta ingane ngayinye ngendlela ehlukile. Lokhu kusho ukuthi imiphumela ingahluka kakhulu kuye ngengane.

• Izingane eziningi zingase zibe nokukhubazeka kwengqondo okulinganiselwe.
• Ezinye izingane ezinezifo zokuwa ezilawulwa kahle zingase zibe nokuhlakanipha okucishe kube okuvamile.
• Kodwa kwabanye abantwana, izinto ezifana nokuhamba, ukukhuluma, kanye nokukhula kwengqondo kungathinteka kakhulu.

Cishe bonke abantwana abane-hemimegalencephaly baba nobuthakathaka ohlangothini olulodwa lomzimba (hemiparesis) . Lolu uhlobo lwe-cerebral palsy. Abanye abantwana bangaba nobuthakathaka obuncane, kanti abanye bangaba nobuthakathaka obukhulu. Abanye abantwana bangaba nesimo esibizwa ngokuthi i-homonymous hemianopsia, lapho ingxenye yensimu yabo yokubona ilahleka khona. Izingane eziningi ziyakwazi ukuhamba nokukhuluma, kodwa akuzona zonke izingane ezingakwazi. Ezinye izingane zidinga izinsiza zokudla ezikhethekile, kanti ezinye zingadla ngendlela evamile.

Ucwaningo lwamuva nje luthole ukuthi ngemva kokuhlinzwa:

• Ukulingana sekume ngokuphelele ezinganeni ezingama-68%.
• Ama-43% ezingane ayenobuhlakani obujwayelekile noma obungasebenzi kahle.
• Izingane ezingama-26% zikwazile ukukhuluma ngendlela efanele iminyaka yazo.
• Izingane ezingama-21% zikwazile ukufunda ngezinga elanele.

Ingabe i-hemimegalencephaly ingavinjelwa?

Njengoba ososayensi bengakazi kahle ukuthi yini ebangela lesi simo, ayikho indlela yokusivimbela.

Unakekela kanjani ingane enalesi simo?

Uma ingane yakho inesifo se-Hemimegalencephaly, yona/yonaKudingeka ithimba elikhulu lezokwelapha. Ngokubambisana nethimba lezokwelapha lengane yakho, ningakha uhlelo oluhlangabezana nezidingo zengane yakho. Ithimba lezokwelapha lengane yakho lingafaka:

• Odokotela bezingane
• Odokotela bezinzwa
• Odokotela abahlinza izinzwa
• Ochwepheshe bentuthuko
• Abelaphi bokukhuluma, abelaphi bomsebenzi, kanye nabelaphi bomzimba
• Abanye abahlinzeki bezempilo.

Yimiphi imibuzo okufanele uyibuze udokotela wengane yakho?

Uma ingane yakho itholakale ine-hemimegalencephaly, ungabuza udokotela le mibuzo:

• Hlobo luni lwe-hemimegalencephaly ingane yami enalo?
• Ingabe ingane yami inezinye izimo zezokwelapha?
• Yikuphi ukwelashwa okungcono kakhulu?
• Uyini umbono wengane yami?
• Yini esingayenza ukuze sinciphise izimpawu zakhe?

Mama noBaba, yibani nesibindi esincane! (Umyalezo Wokuya Ekhaya)

Siyazi ukuthi ukubona ingane yakho inesifo sokuwa, noma ukuzwa ukuthi inesifo esifana ne-Hemimegalencephaly, kungaba yinto eshaqisayo kakhulu futhi ebuhlungu. Kodwa khumbula, awuwedwa. Ithimba lezokwelapha elilapha ingane yakho linawe kuzo zonke izinyathelo.

Uma ubhekene nobunzima bokubhekana nalesi simo, buza udokotela wakho ngamaqembu okusekela asiza imindeni yezingane ezine-hemimegalencephaly. Ukukhuluma nabazali bezingane ezinalesi simo kungakusiza ukunciphisa ukwesaba kwakho futhi uvumelane nesimo sengane yakho. Bangaba umthombo omkhulu wamandla kuwe njengoba ingane yakho ifunda ukuphila nalesi simo.

` I-Hemimegalencephaly, ukukhula kobuchopho okungavamile, isifo sokuwa kwezinsana, ukuhlinzwa kobuchopho, ukubambezeleka kokukhula, izimo zofuzo, izifo zemizwa