Ðe viwòa ƒe ŋkume ʋuʋuna enuenu alo ɖe wòdzena abe ɖe wole ʋuʋum enea? Mina míasrɔ̃ nu tso Jeavons Dɔléle kple Asrãdɔ si me Ŋku ƒe Myoclonia Le ŋu!

Ðe viwò sue la te ŋkuʋuʋu kabakaba zi ɖekaa? Alo ɖe woƒe ŋkuwo te ʋuʋu yi megbe eye woƒe ta se le wo ɖokui me abe ɖe wole megbe yim enea? Ɣeaɖewoɣi la, alɔ̃madɔmadɔ sue aɖe ate ŋu akpe ɖe esia ŋu. Abe dzila ene la, esɔ be nàvɔ̃ ŋutɔ eye nàtsi dzimaɖi ne èkpɔ nane si le abe esia ene. Egbea míele nu ƒom tso asrãdɔ ƒomevi aɖe si mebɔ o si ɖea dzesi siawo fiana ŋu, gake míesea wo ŋu nya zi geɖe o. Woyɔa esia be Jeavons Dɔléle, alo Aɖuɖɔtoedɔ si me Ŋku ƒe Myoclonia Le.

Nukae nye Jeavons ƒe Dɔlélea?

Kpuie ko la, Jevons ƒe dɔlélea nye asrãdɔ si mebɔ o . Eƒe dzesi enye ŋkuʋuʋu kpata, kpata, alo ŋkuʋuʋu. Zi geɖe la , kekeli ƒe nɔnɔme ƒe tɔtrɔ kple kekeli ƒe seselelãme ƒe tɔtrɔ gbɔe nɔnɔme sia tsona . Zi geɖe la, nɔnɔmea dzea egɔme tso ɖevime .

Ànya se le ɖokuiwò me be vɔvɔ̃ ɖo wò ale gbegbe esime viwòa xɔ alɔ̃dɔdɔ zi gbãtɔ, eye ɣesiaɣi si wòxɔe, alo? Wotsia dzi ɖe woƒe dedienɔnɔ ŋu ɣesiaɣi. Togbɔ be viwòa di be yeafe, asrɔ̃ nu, ahakpɔ dzidzɔ abe ɖevi bubuwo ene hã la, esɔ be mi ame evea siaa miase le mia ɖokui me be yemate ŋu awɔ naneke le nɔnɔmea ŋu vie o.

Gake nya nyuiae nye be atikewɔmɔnu aɖewo li siwo dzi woato aɖu alɔ̃madɔmadɔ siawo dzi ahakpɔ ŋusẽ si woate ŋu akpɔ ɖe mia kple viwòa ƒe susu ƒe lãmesẽ dzi gbɔ . Jevons ƒe dɔlélea menye dɔléle si nu yina ne ɖevia le tsitsim o, ke boŋ enye dɔléle si nɔa anyi le eƒe agbemeŋkekewo katã me . Eyata ahiã be woawɔ atike na ɖevia nyuie ahalé ŋku ɖe eŋu le eƒe agbemeŋkekewo katã me.

Ðe Jeavons ƒe Dɔlélea nye afɔkua?

Ẽ, Jeavons Dɔlélea ate ŋu anye afɔku le go aɖewo me . Alesi alɔ̃madɔmadɔa le kple alesi wòsẽe ate ŋu akpɔ ŋusẽ gã aɖe ɖe ɖevia ƒe lãmesẽ bliboa dzi. Esi míenya ɣeyiɣi si me alɔ̃dɔdɔ adzɔ o ta la, mɔnukpɔkpɔ gã aɖe li be ɖevia nadze anyi kasia axɔ abi . Eyata ele vevie be nàdi atike tso ɖɔkta gbɔ eye nàwɔ ɖe mɔfiame siwo wona be ɖevia nanɔ dedie dzi.

Aleke gbegbee nɔnɔme sia mebɔ o?

Jeavons Syndrome dzea ame siwo wokpɔ be woxɔ asrãdɔ la dometɔ 1% va ɖo 2% . Wobu akɔnta be ame siwo ade miliɔn 50 ye kpea fu le xexea me godoo. Eyata bu alesi gbegbe nɔnɔme sia le wo dometɔ ʋɛ aɖewo ko ŋui ŋu kpɔ.

Nukae nye Jeavons Dɔléle ƒe dzesiwo?

Nu vevi etɔ̃e le Jevons ƒe dɔlélea ŋu:

1. Ðevia ƒe ŋkuwo tsia alo nɔa ʋuʋum atraɖii le ŋkume ƒe lãmekawo ƒe tete kabakaba le eɖokui si ta (`Eyelid Myoclonia`).Le ɣeyiɣi sia me la, ɖevia ƒe ta kple to ate ŋu aɖiɖi ɖe megbe. Esia ateŋu adzɔ ne ame ƒe susu megabu kpata alo madzɔ o (`absence seizure`). Zi geɖe la, esia nɔa anyi sɛkɛnd ʋɛ aɖewo. Esia ate ŋu adzɔ zi geɖe gbesiagbe, gake zi geɖe la, wokpɔnɛ le ŋdi me ne wofɔ .

2. Ŋku ƒe ʋuʋu si hea alɔ̃madɔmadɔ vɛ (`Eyelid closure-elicited electroencephalographic paroxysms`) nye alɔ̃madɔmadɔ si tsoa kekeli ƒe tɔtrɔ alo alɔ̃madɔmadɔ gbɔ. Esia nye ne ɖevia miã ŋku eye wòkpɔ elektrikŋusẽ ƒe nɔnɔme siwo mesɔ o le ahɔhɔ̃a me (`Eyelid closure-elicited electroencephalographic paroxysms`). Ðɔkta ateŋu atsɔ ``EEG`` dodokpɔ adi kpɔɖeŋu siawo.

3. Kekeli ƒe seselelãme. Akaɖi siwo le keklẽm ate ŋu ahe alɔ̃dɔdɔ vɛ. Akaɖi siwo klẽna alo kekeli ƒe nɔnɔme siwo le tɔtrɔm (e.g., ɣe ƒe keklẽ si klẽna to atiwo me) ate ŋu ana ame nase le eɖokui me.

Bu eŋu kpɔ, esime viwòa le fefem la, kasia eƒe ŋkuwo te ŋeŋe kabakaba eye eƒe ta trɔ yi megbe. Le sɛkɛnd ʋɛ aɖewo megbe la, egatrɔna va nɔa nɔnɔme si me wònɔ tsã. Aleke gbegbe wòado dzikui nenye be esia dzɔna zi geɖe gbesiagbe?

Tsɔ kpe ɖe eŋu la, ɖevia ate ŋu akpɔ abɔwo kple afɔwo ƒe ʋuʋu kpata (`Myoclonic seizures`) alo ŋutilã bliboa ƒe ʋuʋu si dzi womate ŋu aɖu o (`Grand mal` alo `Tonic-clonic seizures`) . Ke hã, nusiawo mebɔ boo o.

Nukawoe hea Jeanvons Dɔlélea vɛ?

Akaɖi siwo le keklẽm, siwo le keklẽm alo esiwo le keklẽm koŋ gbɔe dzesi siawo tsona .

• Strobe-kaɖiwo abe disko-kaɖiwo ene
• Akaɖi si wòle be woatrɔ (si le keklẽm, tsii) .
• Ɣe si le dodom to alilikpowo me zi ɖeka
• Kekeli si le keklẽm le tsia tame
• Kekeli ƒe nɔnɔme si le tɔtrɔm ne ʋu le ʋu kum to atiwo me

Nu siawo ate ŋu ana ame naxɔ alɔ̃. Eyata , ne èƒo asa na kekelitsoƒe aɖewo alo kpɔ viwò ƒe ŋkuwo ta tso kekeli ƒe tɔtrɔ me ate ŋu akpe ɖe ŋuwò nàɖe dzesi siawo ƒe dzɔdzɔ zi gbɔ zi geɖe dzi akpɔtɔ.

Ƒe nenie Jeavons Dɔléle ƒe dzesiwo dzea egɔme tsoe?

Zi geɖe la, dzesi siawo dzea egɔme tso ɖevime . Ƒe si woxɔna hafi dzea dɔa gɔme tso ƒe 1 va ɖo ƒe 15 le mama dedie nu , gake wogblɔna be dɔlélea ƒe akpa gãtɔ xɔ tso ƒe 6 va ɖo ƒe 8 .

Nukae hea Jeavons ƒe Dɔlélea vɛ?

Womese nusi tututu gbɔ Jevons ƒe dɔlélea tso gɔme bliboe haɖe o , gake numekukuwo ɖee fia be domenyiŋusẽfianu ƒe tɔtrɔ aɖe li . Wokpɔe be tɔtrɔ aɖewo siwo va domenyiŋusẽfianu siwo gbɔna me ate ŋu ahe alɔ̃madɔmadɔ sia vɛ:

• `CHD2`
• `COL6A3`
• `KCNB1`
• `NAA10`
• `NEXMIF`
• `RORB`
• `SYNGAP1`

Womekpɔ de dzesi domenyiŋusẽfianu ƒe tɔtrɔ siwo hea nɔnɔme sia vɛ la dometɔ aɖewo haɖe o. Alesi domenyiŋusẽfianu ƒe tɔtrɔ ɖesiaɖe tso dzidzime yi dzidzime (domenyinu) ate ŋu ato vovo. Àte ŋu adi kpekpeɖeŋu tso domenyiŋusẽfianu ŋuti aɖaŋuɖola gbɔ be nàsrɔ̃ nu geɖe tso esia ŋu.

Amekae le afɔku me wu le nɔnɔme sia me?

Amesiame ate ŋu axɔ Jeanvons Syndrome. Gake afɔkua lolo wu ne asrãdɔ le wò ƒomea me tɔ aɖe ŋu . Ðevi siwo ŋu wokpɔ Jeavons Syndrome le la dometɔ siwo ade 80% ƒe ƒometɔ si ƒe ʋu me ʋu le si hã ŋu asrãdɔ si bɔ .

Azɔ hã, nɔnɔme sia bɔ ɖe nyɔnuviwo dome wu ŋutsuviwo .

Kuxi kawoe ate ŋu ado tso Jeanvons Dɔlélea me?

Ame siwo ade ɖeka le atɔ̃ ɖesiaɖe si ŋu wokpɔ Jevons ƒe dɔlélea le ate ŋu axɔ alɔ̃madɔmadɔ zi geɖe ɖe wo nɔewo yome, eye womagbɔ ɖe eme le wo dome o. Woyɔa esia be ŋkume ƒe lãkusi ƒe nɔnɔme si nye epilepticus .

Vevietɔ wu la, status epilepticus nye atikewɔwɔ kpata si ate ŋu ade ame ƒe agbe afɔku me. Ebia be woakpɔ egbɔ enumake le atikewɔƒe!

Ne viwòa ƒe alɔ̃madɔmadɔ xɔ wu miniti 5, alo ne exɔ alɔ̃ zi geɖe ɖe wo nɔewo yome evɔ womegbɔ ɖe eme le wo dome o la, ƒo ka na 911 enumake alo nàkplɔ viwòa ayi kɔdzi ƒe dɔwɔƒe si te ɖe ŋuwò kpata.

Asrã ate ŋu akpɔ ŋusẽ gã aɖe ɖe ɖevi ƒe susu ƒe lãmesẽ hã dzi . Ne ɖevia xɔ alɔ̃ vɔ la, ate ŋu ase le eɖokui me be vɔvɔ̃ le ye ŋu eye yemaɖe dzi ɖi o. Dzimaɖitsitsi kple blanuiléle hã bɔ ɖe Jevons ƒe dɔlélea me. Eyata ele vevie be nàlé be na ɖevia ƒe susu ƒe nyonyo eye ne ehiã la, nàdi kpekpeɖeŋu tso susumedɔléle ŋuti aɖaŋuɖola gbɔ .

Aleke wokpɔa Jeanvons ƒe Dɔlélea?

Ðɔkta aɖe tsɔa dodokpɔ kple nu siawo ŋu bubu dea dzesii be yeaxɔ Jeavons ƒe Dɔlélea:

• To ahɔhɔ̃mekawo ƒe dodokpɔ wɔwɔ me.
• To ɖevia ƒe lãmesẽ ƒe nɔnɔme kple ƒomea ƒe lãmesẽ ŋutinya nyanya me (vevietɔ asrãdɔ).
• To EEG dodokpɔ (Electroencephalogram - EEG) wɔwɔ me.

Le dodokpɔ siawo me la, ɖɔktaa adi Jevons ƒe dɔlélea ƒe dzesi vevi etɔ̃ siwo míeyɔ va yi. Aɖe nɔnɔme bubu siwo ate ŋu ahe dzesi mawo tɔgbe vɛ hã ɖa be woate ŋu akpɔ dɔlélea nyuie.

Aleke wodaa Jeanvons Dɔléleae?

Wotsɔa atike siwo wua alɔ̃madɔmadɔ daa Jevons ƒe dɔlélea.Tsɔ kpe ɖe eŋu la, wò ɖɔkta ate ŋu aɖo aɖaŋu na wò be nàdo ŋkumeɖonu tɔxɛwo atsɔ aɖu dzesi siwo kekeli hena vɛ dzi.

Atike ka ƒomevie wozãna na esia?

Viwò ƒe ɖɔkta ate ŋu aŋlɔ atike siwo gbɔna siwo wozãna tsɔ tsia alɔ̃dɔdɔ nu la dometɔ ɖeka alo esi wu nenema (siwo woŋlɔ ɖe ɖoɖo si nu ŋɔŋlɔdzesiwo le nu):

• Brivaracetam `(Brivaracetam)`
• Clobazam ƒe ŋkɔ
• Ethosuximide ƒe atike
• Lacosamide ƒe atike
• Lamotrigine si woyɔna be Lamotrigine
• Levetiracetam `(Levetiracetam)`
• Perampanel `(Perampanel)`
• Topiramate ƒe atike
• Valproic acid `(Valproic acid)`

Amesiame wɔa nu ɖe ​​alɔ̃dɔdɔ ƒe atikewo ŋu le mɔ vovovowo nu . Ðewohĩ axɔ ɣeyiɣi hafi nàdi atike nyuitɔ alo atike siwo wotsɔ ƒo ƒui na viwòa, eye ahiã be nàte atike vovovo geɖe kpɔ . Esia ate ŋu anye nuteƒekpɔkpɔ si tea ɖeɖi ame ŋu eye ɣeaɖewoɣi la, exɔa ɣeyiɣi didi. Le ɣeyiɣi sia me la, ahiã be nàyi viwò ƒe ɖɔkta gbɔ edziedzi akpɔ alesi viwò ƒe ŋutilã le nu wɔm ɖe atikeawo ŋui.

Ðɔktaa ana nànya nu tso atike ɖesiaɖe si woana viwòa ƒe nugbegblẽ le ame ŋu hã ŋu. Ne nya aɖe le asiwò ku ɖe atikewɔwɔ si ɖɔktaa kafu na wò ŋu la, biae.

Ðe woate ŋu ada nɔnɔme sia to ɖevia ƒe nuɖuɖu tɔtrɔ mea?

Nuɖuɖu aɖeke meli si wokafu koŋ na Jeanvons Dɔlélea o. Fifia nyatakaka aɖeke meli si aɖo kpe ketogenic nuɖuɖu zazã abe dɔléle sia dada ene dzi o.

Nukae nye ɖevi si ŋu Jeavons Dɔlélea le ƒe etsɔme? (Nyagblɔɖi) .

Ðevi si ŋu dɔléle sia le ƒe etsɔme toa vovo le ɖevi ɖesiaɖe ƒe nɔnɔme tɔxɛ nu . Viwò ƒe ɖɔktae nye ame nyuitɔ kekeake si nànya tso esia ŋu.

Atike aɖeke meli na Jevons ƒe dɔlélea o, gake wole numekuku geɖe wɔm. Togbɔ be enye dɔléle si nɔa ame ŋu le eƒe agbemeŋkekewo katã me hã la, atike aɖewo li siwo dzi woato aɖu alɔ̃madɔmadɔ dzi .

Ðe Jeavons ƒe Dɔlélea gblẽa nu le ɖevi ƒe nunya ŋua?

Alɔ̃madɔmadɔ kple ŋkuʋuʋu ate ŋu ado kplamatse ɖevi ƒe ŋutete be wòasrɔ̃ nu le sukuxɔ me . Ne womete ŋu na woƒe ŋkuwo ʋu nyuie o la, ate ŋu asesẽ be woakpɔ gome le klass me bliboe. Ðevi geɖe siwo ŋu Jevons ƒe dɔlélea le la ƒe nusɔsrɔ̃ sesẽna vie le suku eye woate ŋu ahiã sukudede ƒe kpekpeɖeŋu bubuwo . Gake nɔnɔme sia mekpɔa ŋusẽ ɖe ɖevi ƒe nunya dzi tẽ o .

Ne ɖevia ƒe tsitsi tsi megbe la, ate ŋu anye dzesi be domenyiŋusẽfianu aɖe li si gbɔ Jevons ƒe dɔlélea tso.

Ðe Jeavons Dɔlélea kpɔa ŋusẽ ɖe agbenɔƒe dzia?

Jeavons Dɔlélea mekpɔa ŋusẽ ɖe ɖevi ƒe agbenɔƒe dzi tẽ o . Agbenɔƒe si sɔ ate ŋu adzɔ. Gake alesi alɔ̃madɔmadɔa nu sẽe kple alesi ɖevia ƒe ŋutilã wɔa nui ne wowɔ atike nɛ ate ŋu akpɔ ŋusẽ ɖe ɖevia ƒe lãmesẽ bliboa dzi. Ðɔkta ate ŋu akpe asi ɖe eŋu be wòalé ŋku ɖe nɔnɔmea ŋu ahakpɔ edzi le ɖevia ƒe agbemeŋkekewo katã me.

Ɣekaɣie wòle be makpɔ ɖɔkta?

Ne viwòa xɔ alɔ̃dɔdɔ zi gbãtɔ la, ƒo ka na dɔwɔƒe siwo kpɔa nudzɔdzɔ kpatawo gbɔ enumake alo nàkplɔ wo ayi kɔdzi.

Ne wokpɔ Jeavons Syndrome le ŋuwò alo viwòa, ne alɔ̃madɔmadɔ alo dzesi siwo dze abe ɖe wole sesẽm ɖe edzi ene, alo ne atikewɔwɔa ƒe nugbegblẽ le ame ŋu le fu ɖem na wò la, yi ɖɔkta gbɔ enumake . Wò ɖɔkta ate ŋu akpe ɖe mia kple viwòa ŋu miakpɔ nɔnɔmea gbɔ ahaɖo miaƒe biabiawo ŋu.

Nya kawoe wòle be mabia ɖɔktaa?

• "Ðɔkta/Aƒenɔ, atikewɔwɔ ka ƒomevie nyo na vinyea?"
• "Nukae nye atike siawo ƒe nugbegblẽ le ame ŋu siwo ate ŋu ado tso eme?"
• "Èɖo aɖaŋu be nàzã ŋkumeɖobolo tɔxɛ aɖe hena kekeli ƒe seselelãmea?"
• "Aleke mawɔ akpe ɖe vinye ŋu le suku?"

Ðe Sunflower Dɔléle do ƒome kple Jeanvons Dɔlélea?

Sunflower Syndrome kple Jeavons Syndrome nye dɔléle eve siwo ɖi wo nɔewo . Sunflower Syndrome hã nye asrãdɔ ƒomevi aɖe si ŋkume ƒe ʋuʋudedi kpena ɖe eŋu. Le esia me la, ame ate ŋu atrɔ ɖe kekelitsoƒe si klẽna (abe ɣe ƒe keklẽ ene) ŋu eye wòaʋuʋu asi le ŋkume. Esia ate ŋu ana kekeli ƒe nɔnɔme natrɔ eye wòahe alɔ̃dɔdɔ vɛ.

Mlɔeba la, nusiwo dzi wòle be woaɖo ŋkui (Take-Home Message) .

Míeŋlɔa alesi kekeli le vevie na míaƒe gbesiagbegbenɔnɔ be ɣeaɖewoɣi. Le kpɔɖeŋu me, kekeli kpena ɖe mía ŋu míekpɔa nu le viviti me eye míetsina numiemiewo. Gake le amesi ŋu Jeavons Syndrome le gome la, kekeli ate ŋu ana wòadze alɔ̃madɔmadɔ . Ðɔkta ate ŋu akpe ɖe ŋuwò nàkpɔ alesi kekeli kpɔa ŋusẽ ɖe viwòa dzii. Woate ŋu aŋlɔ atike kple ŋkumeɖonu tɔxɛwo be woatsɔ atsyɔ ŋkua dzi.

Ɖo ŋku edzi:

• Jeavons Dɔléle nye asrãdɔ si mebɔ o gake woate ŋu akpɔ egbɔ .
• Dzesi vevitɔwoe nye ŋkuʋuʋu kpata, kekeli ƒe seselelãme, kple fotovɔvɔ̃ .
• Togbɔ be esia nye agbemeŋkekewo katã ƒe nɔnɔme hã la, woate ŋu atsɔ atikewo kple agbenɔnɔ ƒe tɔtrɔwo aɖu edzi .
• Lé ŋku ɖe viwòa ƒe susu ƒe lãmesẽ hã ŋu . Di kpekpeɖeŋu tso eŋutinunyalawo gbɔ ne ehiã.
• Alɔ̃madɔmadɔ si xɔa wu miniti 5 alo alɔ̃madɔmadɔ si kplɔ wo nɔewo ɖo nye nusi dzɔ kpata! Di atikewɔƒe enumake.

Mele mɔ kpɔm be nyatakaka sia kpe ɖe ŋuwò nèse nɔnɔme sia gɔme vie. Medi be viwòa nahaya kaba!

` Jevons ƒe dɔléle, asrã, alɔ̃madɔmadɔ, ŋkume ƒe myoclonus, ɖeviwo ƒe dɔléle, keklẽŋusẽ, EEG