Ðe viwòa ƒe ahɔhɔ̃ ƒe akpa ɖeka lolo akpaa? Mina míasrɔ̃ nu tso hemimegalencephaly ŋu!

Ðe nètsi dzi ɖe viwò sue ƒe ta ƒe nɔnɔme ŋu kpɔa, alo ɖe alesi wòtsia enu enuenu ŋua? Ɣeaɖewoɣi la, atikewɔwɔ me nɔnɔme aɖe si mebɔ kura o ate ŋu anɔ megbe na nusiawo. Hemimegalencephaly nye dɔléle mawo dometɔ ɖeka. Togbɔ be ŋkɔa aɖi nya sesẽ vie hã la, mina míaƒo nu tso eŋu bɔbɔe.

Nukae nye Hemimegalencephaly?

Kpuie ko la, hemimegalencephaly nye ne viwò ƒe ahɔhɔ̃ ƒe akpa ɖeka (cerebral hemisphere) lolo wu evelia le mɔ si mesɔ o nu. Bu míaƒe ahɔhɔ̃a be enye dɔdzẽ gã aɖe si ƒe afã eve, miame kple ɖusime. Le nɔnɔme sia me la, axa ɖeka koe lolona wu alesi wòle be wòanɔ. Ɣeaɖewoɣi la, ahɔhɔ̃a ƒe akpa ma ƒe akpa sue aɖe koe kekeɖenudɔ sia ate ŋu anɔ , alo axa bliboa ate ŋu alolo.

Ne ahɔhɔ̃a ƒe akpa ɖeka lolo alea la, tɔtrɔ bubuwo ate ŋu ava ahɔhɔ̃a me. Le kpɔɖeŋu me:

• Ahɔhɔ̃mekawo nye lãmenugbagbevi ƒomevi aɖe le míaƒe ahɔhɔ̃ me. Ðewohĩ womewɔ ɖoɖo ɖe wo ŋu nyuie o (cytoarchitecture) alo ahɔhɔ̃a ƒe akpa si le etame la mawɔ nyuie o (cortical dysplasia).
• Corpus callosum si bu alo gblẽ: Esia le abe tɔdzisasrã si ƒoa ahɔhɔ̃a ƒe akpa eveawo nu ƒu ene . Ne ebu alo gblẽ la, kuxiwo ate ŋu ado mo ɖa.
• Ahɔhɔ̃a ƒe do siwo me tsi yɔ fũu (ventricles) ƒe lolome le akpa si ŋu dɔlélea le.

Esi ahɔhɔ̃a ƒe akpa aɖewo metsi nyuie le mɔ sia nu o ta la, alɔ̃madɔmadɔ ate ŋu adzɔ enuenu. Míeyɔa esia hã be asrãdɔ . Zi geɖe la, esesẽna be woatsɔ atike dzro siwo tsia alɔ̃madɔmadɔ nu aɖu alɔ̃madɔmadɔ siawo dzi . Ne womate ŋu atsɔ atike aɖu alɔ̃madɔmadɔa dzi o la, ele be woawɔ dɔ na ɖevi geɖe le ahɔhɔ̃ me (amekoko na aɖuɖɔtoedɔ).

Ðe hemimegalencephaly ƒomevi vevi aɖewo lia?

Ẽ, nɔnɔme sia ƒomevi vevi geɖewo li:

• Isolated hemimegalencephaly: Esia gblẽa nu le ɖevia ƒe ahɔhɔ̃ ƒe akpa si le gota wu (cerebral cortex) ko ŋu. Ŋutilã alo ŋutinu bubuwo mekpɔa gome le eme o.
• Syndromic hemimegalencephaly: Menye ahɔhɔ̃a koe esia gblẽa nu le o, ke egblẽa nu le ɖevia ƒe ŋutigbalẽ kple ŋutinu bubu aɖewo hã ŋu. Gake laŊutilã ƒe dzesiwo ate ŋu adze le vidzidzi megbe ɣleti alo ƒe geɖe gɔ̃ hã. Azɔ hã, vidzĩa ƒe ŋutilã ƒe akpa ɖeka ate ŋu alolo wu evelia (hemihypertrophy).
• Total hemimegalencephaly: Le esia me la, ɖevia ƒe ahɔhɔ̃mekawo kple ɣeaɖewoɣi ahɔhɔ̃mekawo gɔ̃ hã lolona. Esia ate ŋu adzɔ le eɖokui si alo le dɔléle aɖe nu.

Nukae nye nɔnɔme sia ƒe dzesiwo?

Dzesi gbãtɔ si nàte ŋu ade dzesii enye be viwòa ƒe ``vidzĩ ƒe ʋuʋu alo alɔ̃madɔmadɔ ''. Zi geɖe la, esiawo nyea alɔ̃madɔmadɔ siwo wokpɔna le teƒe ɖeka. Esia fia be vidzĩa ƒe ŋutilã ƒe akpa ɖeka koe ate ŋu aʋuʋu.

Dzesi bubu siwo woate ŋu akpɔ dometɔ aɖewoe nye:

• Ta si lolo (Macrocephaly).
• Ta ƒe nɔnɔme si mesɔ o.
• Ʋuʋu abe azɔlizɔzɔ kple duƒuƒu ene sesẽ.
• Ŋutilã ƒe akpa ɖeka ƒe lãmetutudɔ kple gbɔdzɔgbɔdzɔ.
• Tsitsi ƒe megbetsitsi: Esia fia be woatsi megbe le nuƒoƒo, fefe, kple nusɔsrɔ̃ me le mɔ si sɔ ɖe woƒe ƒexɔxɔ nu nu.
• Nukpɔkpɔ ƒe gbɔdzɔgbɔdzɔ: Le kpɔɖeŋu me, afisi woate ŋu akpɔ nu le ƒe afã buna ne wotsɔ ŋku eveawo le ŋku lém ɖe eŋu (hemianopia).

Nukae hea hemimegalencephaly vɛ?

Le nyateƒe me la, dzɔdzɔmeŋutinunyalawo menya nusi tututu hea nɔnɔme sia vɛ haɖe o. Ame aɖewo xɔe se be domenyiŋusẽfianu mee wòtso, si fia be ɖevia ƒe DNA ƒe tɔtrɔ gbɔe wòtso . Gake menye domenyiŋusẽfianue wònye o. Ema fia be nɔnɔmea le ŋuwò eye menye nane si nètsɔna naa viwòe o.

Anɔ eme godoo be le fufɔfɔ ƒe kwasiɖa etɔ̃lia lɔƒo la, tɔtrɔ aɖe dzɔna le vome, alo le eɖokui si, le domenyiŋusẽfianu siwo le vevie na ahɔhɔ̃a ƒe tsitsi dometɔ ɖeka me. Ðo ŋku edzi be menye naneke si nèwɔ le fufɔɣi gbɔe nɔnɔme sia tso o. Esia menye wò vodada alo fofoa ƒe vodada o.

Nukawoe nye afɔku siwo gblẽa nu le nɔnɔme sia ŋu?

Hemimegalencephaly ate ŋu adzɔ kpe ɖe domenyiŋusẽfianu ƒe nɔnɔme bubuwo ŋu. Esia fia be ɖevi siwo ŋu dɔléle siawo le la le afɔku me be woaxɔ hemimegalencephaly:

• `Epidermal nevus ƒe dɔléle`
• `Klippel-Trenaunay ƒe dɔléle`
• `McCune-Albright ƒe dɔléle`
• ``Ahɔhɔ̃mekawo ƒe Dɔléle Ƒomevi 1 (NF1)''.
• `CLOVES ƒe dɔléle`
• `Proteus ƒe dɔléle`
• `Ito ƒe hypomelanosis`

Aleke wowɔa dea dzesi hemimegalencephaly?

Be viwòa ƒe ɖɔkta nanya kakaɖedzitɔe nenye be aleae wòle la, ado viwòa kpɔ gbã eye wòabia viwòa ƒe lãmesẽ ŋutinya wò. Emegbe, woate ŋu awɔ dodokpɔ siwo gbɔna:

• Ahɔhɔ̃ ƒe MRI-dɔdamɔnu.
• EEG (Electroencephalogram) dodokpɔ. Esia dzidzea ahɔhɔ̃a ƒe elektrikŋusẽ ƒe dɔwɔwɔ.

Le egbegbe mɔ̃ɖaŋununya deŋgɔwo me la, woate ŋu akpɔ nɔnɔme sia ɣeaɖewoɣi esime vidzĩa gakpɔtɔ le vidzidɔ me ``le fufɔɣi.`` Wowɔa esia to MRI scan tɔxɛ aɖe wɔwɔ le vidzĩa ƒe ahɔhɔ̃ ŋu ``fetal MRI``.

Aleke wowɔa atike nɛ?

Taɖodzinu vevitɔ si le hemimegalencephaly dada ŋue nye be woaɖu alɔ̃madɔmadɔa dzi. Ɣeaɖewoɣi la, alɔ̃madɔmadɔ siawo ate ŋu adze egɔme tso woƒe ɖevime ŋutɔ eye zi geɖe la, esesẽna be woaɖu wo dzi to atikewo zazã me (atikewo mete ŋu nɔa te ɖe enu o).

Nɔnɔme sia ate ŋu ana ame nadze ame ŋu le mɔ vovovowo nu. Ele vevie be míade dzesii be lãmetutudɔ si dzɔna le ɖevime, vevietɔ ne edze egɔme tso vidzĩme, ate ŋu akpɔ ŋusẽ gã aɖe ɖe ɖevi ƒe tsitsi dzi. Ate ŋu agblẽ nu le ɖevi ƒe tsitsi kple tsitsi ŋu vevie, eye wòagblẽ nu le ahɔhɔ̃ si le tsitsim hã ŋu. Eyata ele vevie be nàtɔ te eƒe ƒoƒo kaba ale si nàte ŋui.

Wodzea alɔ̃madɔmadɔ dada gɔme kple atike siwo tsia alɔ̃madɔmadɔ nu. Ðevi aɖewo ate ŋu atsɔ atike siawo aɖu woƒe alɔ̃dɔdɔ dzi le woƒe agbemeŋkekewo katã me. Gake ame geɖe va xɔa asrãdɔ si atikewo mete ŋu ɖuna o. Emegbe wowɔa dɔ aɖe si woyɔna be hemispherectomy. Esia bia be woaɖe ɖevia ƒe ahɔhɔ̃ ƒe akpa si ŋu dɔlélea le (cerebral cortex) ɖa alo amae ɖa tso ahɔhɔ̃a ƒe akpa kemɛ.

Ne wowɔ dɔ sia la, woyɔnɛ ɣeaɖewoɣi be "functional hemispherectomy." Le amekoko sia me la, amekolaa lãa lãmeka kple lãmeka siwo doa ka kple ɖevia ƒe ahɔhɔ̃ ƒe akpa ɖeka kple akpa kemɛ la heɖea wo ɖa, gake woate ŋu agblẽ ahɔhɔ̃a ƒe akpa si lolo le mɔ si mesɔ o nu la ɖe taɖua me. Le "anatomical or complete hemispherectomy" me la, woɖea ahɔhɔ̃a ƒe akpa ɖeka ɖa tso akpa kemɛ gbɔ, eye woɖea akpa si mesɔ o la ɖa keŋkeŋ. Ele be ahɔhɔ̃ŋutinunyala si xɔ hehe tɔxɛ le asrãdɔ ƒe amekoko ŋu nawɔ amekoko sia.

Ðe amekoko be woaɖe ahɔhɔ̃a ƒe afã ɖa (Hemispherectomy) sesẽa?

Le nyateƒe me la, ahɔhɔ̃mekawo ɖeɖe nye ahɔhɔ̃mekawo ƒe dɔwɔwɔ sesẽ ŋutɔ. Susu geɖewo li siwo tae amekoko sia wɔwɔ sesẽna ɖe edzi le nɔnɔme si nye hemimegalencephaly ta.

Nu gbãtɔe nye be zi geɖe la, ʋukawo nɔa teƒe si mebɔ o. Eyata esesẽna be woake ɖe wo ŋu ahatso wo le amekoko me. Esia ate ŋu ana ʋu nado fũu akpa ɣeaɖewoɣi.

Tsɔ kpe ɖe eŋu la, esi ɖevi ƒe ahɔhɔ̃ bliboa ƒe nɔnɔme mesɔ o ta la, zi geɖe la, dzesi siwo amekola zãna tsɔ dea dzesi ahɔhɔ̃a ƒe akpa ɖesiaɖe la medzena o. Azɔ hã, vidzĩwo kple ɖevi suewo ate ŋu abu ʋu gbogbo aɖe le amekoko sia me. Ele be woalé ŋku ɖe esia ŋu nyuie ŋutɔ eye woaɖɔli ʋu ne ehiã.

Eyata ele vevie ŋutɔ be atikewɔlawo ƒe ƒuƒoƒo si le viwòa dam la nanye ƒuƒoƒo bibi siwo si nuteƒekpɔkpɔ geɖe le le amekoko sia me. Anyo wu be viwòa nawɔ dɔ sia le kɔdzi si ɖea vidzĩ siwo ŋu hemimegalencephaly le edziedzi eye nuteƒekpɔkpɔ geɖe le esi le dɔ sia wɔwɔ me.

Nukae nye nyagblɔɖi na ɖevi si ŋu dɔléle sia le?

Alesi viwòa ƒe nɔnɔmea ava nɔ te ɖe alesi woate ŋu akpɔ alesi wòate ŋu aɖui dzi nyuie kple ne ahɔhɔ̃a ƒe tsitsi si mesɔ o le akpa ɖeka alo akpa evea siaa dzi koŋ.

Hemimegalencephaly nye dɔléle si gblẽa nu le ɖevi ɖesiaɖe ŋu le mɔ vovovowo nu. Esia fia be emetsonuwo ate ŋu ato vovo ŋutɔ tso ɖevi ɖesiaɖe gbɔ.

• Ðevi geɖe ate ŋu anye nuwɔametɔ aɖewo le woƒe susu me .
• Ðevi aɖewo siwo ƒe lãmekawo ƒe lãme sẽ nyuie gɔ̃ hã ate ŋu anye esiwo ƒe tagbɔ le abe alesi wòle dzɔdzɔmee ene kloe.
• Gake le ɖevi bubu aɖewo gome la, nu siwo le abe azɔlizɔzɔ, nuƒoƒo, kple susu ƒe tsitsi ene ate ŋu agblẽ nu le wo ŋu vevie.

Ðevi siwo katã kloe ŋu hemimegalencephaly le la ƒe ŋutilã ƒe akpa ɖeka gbɔdzɔna (hemiparesis) . Esia nye ahɔhɔ̃mekawo ƒe dɔmawɔmawɔ nyuie ƒomevi aɖe. Ðevi aɖewo ate ŋu agbɔdzɔ vie, gake gbɔdzɔgbɔdzɔ sesẽ aɖe ate ŋu anɔ ɖevi aɖewo ŋu. Ðevi aɖewo ate ŋu axɔ dɔléle aɖe si woyɔna be homonymous hemianopsia, afisi woƒe ŋku ƒe afã buna le. Ðevi geɖe te ŋu zɔna heƒoa nu, gake menye ɖeviwo katãe tea ŋu o. Ðevi aɖewo hiã na nuɖuɖu tɔxɛwo, evɔ bubuwo ya te ŋu ɖua nu le mɔ si sɔ nu.

Numekuku aɖe si wowɔ nyitsɔ laa ɖee fia be le amekoko megbe la:

• Dɔlélea nu tso keŋkeŋ le ɖevi 68% me.
• Ðeviawo ƒe 43% ƒe tagbɔ gblẽ le mama dedie nu alo eƒe tagbɔ gblẽ vie.
• Ðevi 26% te ŋu ƒo nu le mɔ si sɔ ɖe woƒe ƒexɔxɔ nu nu.
• Ðevi 21% te ŋu xlẽ nu le agbɔsɔsɔ si sɔ me.

Ðe woate ŋu axe mɔ ɖe hemimegalencephaly nua?

Esi dzɔdzɔmeŋutinunyalawo menya nusi tututu hea nɔnɔme sia vɛ haɖe o ta la, mɔ aɖeke meli si dzi woato axe mɔ ɖe enu o.

Aleke nèléa be na ɖevi si ŋu dɔléle sia le?

Ne dɔléle si nye Hemimegalencephaly le viwò ŋu la, ke ewɔa dɔAtikewɔlawo ƒe ƒuƒoƒo gã aɖe hiã. Mia kple viwòa ƒe atikewɔlawo ƒe ƒuƒoƒoa miate ŋu awɔ ɖoɖo si akpɔ viwòa ƒe nuhiahiãwo gbɔ. Viwò ƒe atikewɔlawo ƒe ƒuƒoƒoa ate ŋu anye:

• Ðeviwo ƒe dɔdalawo
• Ahɔhɔ̃ŋutinunyalawo
• Ahɔhɔ̃mekaŋutinunyalawo
• Ŋgɔyiyi ŋuti nunyala bibiwo
• Nuƒoƒo ƒe dɔdamɔnufialawo, dɔdamɔnufialawo, kple ŋutilãmedɔdalawo
• Lãmesẽdɔwɔla bubuwo.

Nya kawoe wòle be nàbia viwòa ƒe ɖɔkta?

Ne wokpɔe be hemimegalencephaly le viwòa ŋu la, àte ŋu abia nya siawo ɖɔktaa:

• Hemimegalencephaly ka ƒomevie le vinyea ŋu?
• Ðe atikewɔwɔ ƒe nɔnɔme bubu aɖewo le vinyea ŋua?
• Nukae nye atikewɔwɔ nyuitɔ kekeake?
• Nukae nye vinye ƒe nukpɔsusu?
• Nukae míate ŋu awɔ atsɔ aɖe eƒe dzesiwo dzi akpɔtɔ?

Dada kple Papa, dzi nenɔ mia ƒo vie! (Gbedasi si Wotsɔ Yi Aƒeme)

Míenya be viwòa kpɔkpɔ be eƒe lãme sẽ, alo nyanya be dɔléle aɖe abe Hemimegalencephaly ene le ye ŋu la, ate ŋu anye nusi awɔ nuku na ame ŋutɔ eye wòagbã dzi na ame. Gake ɖo ŋku edzi be menye wò ɖeka koe o. Atikewɔlawo ƒe ƒuƒoƒo si le viwòa dam la le kpli wò le mɔa ƒe afɔɖeɖe ɖesiaɖe me.

Ne èle agbagba dzem be yeanɔ te ɖe nɔnɔme sia nu la, bia wò ɖɔkta tso kpekpeɖeŋunaha siwo kpena ɖe ɖevi siwo ŋu hemimegalencephaly le ƒe ƒomewo ŋu. Nuƒoƒo na ɖevi siwo ŋu dɔléle sia le dzilawo ate ŋu akpe ɖe ŋuwò nàɖe wò vɔvɔ̃ dzi akpɔtɔ eye nàva lɔ̃ ɖe viwòa ƒe nɔnɔmea dzi. Woate ŋu anye ŋusẽtsoƒe gã aɖe na wò esi viwòa le alesi wòanɔ agbe ɖe nɔnɔmea nu srɔ̃m.

` Hemimegalencephaly, ahɔhɔ̃ ƒe tsitsi si mesɔ o, vidzĩ ƒe asrã, ahɔhɔ̃ ƒe dɔwɔwɔ, tsitsi ƒe megbetsitsi, domenyiŋusẽfianu ƒe nɔnɔmewo, ahɔhɔ̃medɔlélewo