So wo ba no aniwa no twitwiw mpɛn pii anaasɛ ɛte sɛ nea ɛrehuruhuruw? Momma yensua Jeavons Syndrome ne Epilepsy With Eyelid Myoclonia ho ade!

So wo ba ketewa no afi ase rebɔ n’ani ntɛmntɛm mpofirim? Anaasɛ wɔn ani afi ase resan akɔ akyi na wɔn ti ayɛ te sɛ nea wɔrekɔ akyi? Ɛtɔ mmere bi a, eyi betumi de akisikuru ketewaa bi aka ho. Sɛ́ ɔwofo no, ɛyɛ ade a ɛfata sɛ wuhu biribi a ɛte sɛɛ a, wobɛte ehu ne dadwen kɛse nka. Ɛnnɛ yɛbɛka akisikuru bi a ɛntaa nsi a ɛda saa nsɛnkyerɛnne yi adi ho asɛm, nanso yɛntaa nte ho asɛm. Wɔfrɛ eyi sɛ Jeavons Syndrome, anaasɛ Epilepsy a Ɛwɔ Aniwa mu Myoclonia.

Dɛn ne Jeavons Syndrome?

Sɛ yɛbɛka no tiawa a, Jevons syndrome yɛ akisikuru a ɛntaa nsi . Nea ɛda adi sɛ ɛyɛ n’ani a ɛbɔ mpofirim, mpofirim, anaa aniwa a ɛwosow. Mpɛn pii no hann nhyehyɛe a ɛsakra ne hann ho nkate a ɛsakra na ɛde tebea yi ba . Mpɛn pii no tebea no fi ase wɔ mmofraase .

Ɛbɛyɛ sɛ wotee ehu nka kɛse bere a wo ba no nyaa akisikuru a edi kan no, na bere biara a obenya bi no, ɛnte saa? Wɔn ahobammɔ ho dadwen wɔ hɔ bere nyinaa. Ɛwom mpo sɛ wo ba pɛ sɛ odi agoru, osua ade, na n’ani gye te sɛ mmofra afoforo de, nanso ɛyɛ ade a ɛfata sɛ mo baanu nyinaa bɛte nka sɛ wontumi nyɛ hwee kakra wɔ tebea no ho.

Nanso asɛmpa no ne sɛ ayaresa ahorow bi wɔ hɔ a wobetumi de adi saa akisikuru yi so na wɔadi nkɛntɛnso a ebetumi anya wɔ wo ne wo ba adwenemyare so no ho dwuma . Jevons syndrome nyɛ tebea a ɛkɔ bere a abofra no nyin no, ɛyɛ tebea a ɛba ne nkwa nna nyinaa mu . Enti, abofra no behia ayaresa a ɛfata na wɔhwɛ no wɔ n’asetra nyinaa mu.

So Jeavons Syndrome yɛ nea asiane wom?

Yiw, Jeavons Syndrome betumi ayɛ asiane wɔ tebea horow bi mu . Sɛnea akisikuru no te ne sɛnea emu yɛ den no betumi anya abofra no akwahosan nyinaa so nkɛntɛnso kɛse. Esiane sɛ yennim bere a akisikuru bɛba nti, hokwan kɛse wɔ hɔ sɛ abofra no bɛhwe ase mpofirim na wapira . Enti, ɛho hia sɛ wɔhwehwɛ ayaresa fi oduruyɛfo hɔ na wodi akwankyerɛ a wɔde ama abofra no so dwoodwoo no akyi.

Tebea yi ho yɛ na dɛn?

Jeavons Syndrome ka nnipa a wɔahu sɛ wɔwɔ akisikuru no mu 1% kosi 2% . Wobu akontaa sɛ nnipa bɛyɛ ɔpepem 50 na wɔyare akisikuru wɔ wiase nyinaa. Enti susuw sɛnea wɔn mu ɔha biara mu kakraa bi na wɔwɔ saa tebea yi ho hwɛ.

Dɛn ne Jeanvons Syndrome ho sɛnkyerɛnne ahorow?

Nneɛma atitiriw abiɛsa na ɛwɔ Jevons yare no mu:

1. Abofra no ani to anaasɛ ɛbɔ bere nyinaa esiane aniwa no ntini a ɛtwetwe ntɛmntɛm a ɛnyɛ ne pɛ (`Eyelid Myoclonia`) nti.Saa bere yi, abofra no ti ne n’aso betumi ahwe ase. Eyi betumi aba bere a obi nhu nhumu mpofirim anaasɛ ɛnte saa (`absence seizure`). Eyi taa di sikani kakraa bi. Eyi betumi asi mpɛn pii da biara, nanso wɔtaa hu no anɔpa bere a wɔanyan no .

2. Akisikuru a ɛma aniwa a wɔto mu (`Eyelid closure-elicited electroencephalographic paroxysms`) yɛ akisikuru a ɛnam nsakrae a ɛba hann mu anaasɛ nna a wonnya so na ɛba. Eyi ne bere a abofra no mu n’ani na onya anyinam ahoɔden nhyehyɛe a ɛnteɛ wɔ amemene no mu (`Eyelid closure-elicited electroencephalographic paroxysms`). Oduruyɛfo betumi de ``EEG`` sɔhwɛ ahwehwɛ saa nhwɛso ahorow yi.

3. Nneɛma a ɛma hann no te nka. Akanea a ɛhyerɛn betumi ama obi anya akisikuru. Kanea a ɛhyerɛn anaa hann nhyehyɛe a ɛsakra (e.g., owia hann a ɛhyerɛn fa nnua mu) betumi ama obi ayɛ basaa.

Fa no sɛ, bere a wo ba no redi agoru no, mpofirim ara n’ani fi ase twitwiw ntɛmntɛm na ne ti san n’akyi. Wɔ sikɔne kakraa bi akyi no, ɔsan ba sɛnea na ɔte kan no. Sɛ eyi si mpɛn pii da biara a, anka ɛbɛyɛ ahometew dɛn?

Bio nso, abofra no betumi anya nsa ne ne nan a ɛrewosow mpofirim (`Myoclonic seizures`) anaasɛ nipadua no nyinaa a ɛrewosow a wontumi nni so (`Grand mal` anaa `Tonic-clonic seizures`) . Nanso, eyinom nyɛ nea abu so kɛse.

Dɛn ne nneɛma a ɛkanyan Jeanvons Syndrome?

Kanea a ɛhyerɛn, ɛhyerɛn anaa ɛrehyerɛn titiriw na ɛde saa sɛnkyerɛnne ahorow yi ba .

• Strobe akanea te sɛ disko akanea
• Kanea a ɛsɛ sɛ wɔsesa (sɔ, dum) .
• Owia a ɛrepue mpofirim wɔ mununkum no mu
• Hann a ɛhyerɛn wɔ nsu no ani
• Hann nhyehyɛe a ɛsakra bere a kar bi fa nnua mu no

Saa nneɛma yi betumi ama obi anya akisikuru. Enti , hann fibea ahorow bi a wobɛkwati anaasɛ wobɛbɔ wo ba ani ho ban afi hann mu nsakrae ho no betumi aboa ma sɛnkyerɛnne ahorow yi a ɛba mpɛn pii no so atew.

Mfe bɛn na Jeavons Syndrome ho sɛnkyerɛnne fi ase?

Saa sɛnkyerɛnne ahorow yi taa fi ase wɔ mmofraase . Sɛ wɔkyekyem pɛpɛɛpɛ a , mfe a obi adi fi ase no yɛ mfe 1 kosi 15 , nanso wɔbɔ amanneɛ sɛ nnipa dodow no ara di mfe 6 kosi 8 .

Dɛn na ɛde Jeavons Syndrome ba?

Wonnya nte nea ɛde Jevons yare no ba no ase yiye , nanso nhwehwɛmu kyerɛ sɛ awosu mu nsakrae bi wɔ hɔ . Wɔahu sɛ nsakrae bi a ɛba awosu mu nkwaadɔm a edidi so yi mu no betumi de saa akisikuru yi aba:

• `CHD2`
• `KƆL6A3`
• `KCNB1`
• `NAA10`
• `NEXMIF`
• `RORB`
• `SYNGAP1`

Wonnya nhuu awosu mu nsakrae a ɛde tebea yi ba no bi. Ɔkwan a wɔfa so de awosu mu nsakrae biara fi awo ntoatoaso so kɔ awo ntoatoaso so (agyapade) betumi ayɛ soronko. Wubetumi ahwehwɛ mmoa afi awosu ho ɔfotufo hɔ na ama woasua eyi ho ade pii.

Hena na ɔwɔ asiane kɛse mu ma tebea yi?

Obiara betumi anya Jeavons Syndrome. Nanso, asiane no yɛ kɛse sɛ obi a ɔwɔ w’abusua mu wɔ akisikuru a . Mmofra a wɔahu sɛ wɔwɔ Jeavons Syndrome no mu bɛyɛ 80% wɔ mogya busuani bi a ɔno nso wɔ akisikuru a ɛkɔ so daa .

Afei nso, tebea yi abu so wɔ mmeawa mu sen mmarimaa .

Dɛn ne nsɛnnennen a ebetumi aba wɔ Jeavons Syndrome mu?

Ebia nnipa baanum biara mu biako a wohu sɛ ɔwɔ Jevons yare no benya akisikuru mpɛn pii a ɛtoatoa so, a wɔrennya ahomegye biara wɔ ntam. Wɔfrɛ eyi aniwa anim myoclonic status epilepticus .

Nea ɛho hia sen biara no, status epilepticus yɛ aduruyɛ mu ntɛmpɛ a ɛde nkwa to asiane mu. Ɛhwehwɛ sɛ wonya ayaresa ntɛm ara!

Sɛ wo ba no nya akisikuru a ɛkyɛ boro simma 5, anaasɛ sɛ onya akisikuru mpɛn pii a ɛtoatoa so a ahomegye biara nni ntam a, frɛ 911 ntɛm ara anaa fa wo ba no kɔ ayaresabea dwumadibea a ɛbɛn no a wɔhwɛ ntɛmpɛ so.

Akisikuru nso betumi anya abofra adwenemyare so nkɛntɛnso kɛse . Sɛ abofra no nya akisikuru wie a, ebetumi ate ehu nka na ne ho atɔ no. Dadwen ne adwenemhaw nso abu so wɔ Jevons yare no ho. Enti, ɛho hia sɛ wohwɛ abofra no adwene mu yiyedi so na sɛ ɛho hia a, wohwehwɛ mmoa fi adwenemyare ho ɔfotufo hɔ .

Ɔkwan bɛn so na wohu sɛ obi anya Jeavons Syndrome?

Oduruyɛfo bi nam nhwehwɛmu ne nneɛma yi a osusuw ho no so hu sɛ ɔwɔ Jeavons Syndrome:

• Ɛdenam ntini mu nhwehwɛmu a wɔbɛyɛ so.
• Ɛdenam abofra no akwahosan tebea ne abusua akwahosan ho abakɔsɛm a wobehu so (titiriw akisikuru).
• Ɛdenam EEG sɔhwɛ (Electroencephalogram - EEG) a wɔbɛyɛ so.

Wɔ nhwehwɛmu ahorow yi mu no, oduruyɛfo no bɛhwehwɛ Jevons syndrome ho sɛnkyerɛnne atitiriw abiɛsa a yɛadi kan aka ho asɛm no. Ɔbɛpow tebea afoforo a ebetumi de sɛnkyerɛnne a ɛte saa ara aba nso na ama wɔahu yare no pɛpɛɛpɛ.

Ɔkwan bɛn so na wɔsa Jeavons Syndrome?

Wɔde nnuru a ekum akisikuru na ɛsa Jevons yare no.Nea ɛka ho no, ebia wo duruyɛfo bɛkamfo akyerɛ sɛ wohyɛ ahwehwɛ soronko na ama woatumi asiw sɛnkyerɛnne ahorow a hann de ba no so.

Nnuru bɛn na wɔde yɛ eyi?

Wo ba no oduruyɛfo betumi akyerɛw nnuru a edidi so yi a ekum akisikuru (a wɔahyehyɛ no nnidiso nnidiso sɛnea nkyerɛwde no te):

• Brivaracetam `(Brivaracetam)`
• Clobazam a wɔfrɛ no Clobazam
• Ethosuximide a wɔde yɛ aduru
• Lacosamide a wɔde yɛ nnuru
• Lamotrigine na ɛyɛ adwuma
• Levetiracetam `(Nneɛma a ɛma obi nya ahoɔden)`
• Perampanel `(Perampanel)`
• Topiramate a wɔde yɛ aduru
• Valproic acid `(Valproic acid)`

Everyone responds differently to seizure medications . Ebia ebegye bere ansa na woanya nnuru a ɛfata anaa nnuru a wɔaka abom ama wo ba, na ebia ebehia sɛ wosɔ nnuru ahorow pii hwɛ . Eyi betumi ayɛ abasamtu na ɛtɔ mmere bi a ɛkyɛ. Saa bere yi mu no, ebehia sɛ wokɔ wo ba no oduruyɛfo nkyɛn daa na woahu sɛnea wo ba no nipadua reyɛ n’ade wɔ nnuru no ho.

Oduruyɛfo no nso bɛbɔ wo amanneɛ wɔ nsunsuanso bɔne a ebetumi afi nnuru biara a wɔde bɛma wo ba no mu aba no ho. Sɛ wowɔ nsɛmmisa bi fa ayaresa a oduruyɛfo no kamfo kyerɛ ho a, bisa.

So wobetumi asa tebea yi denam abofra no aduan a wɔbɛsakra no so?

Aduan pɔtee biara nni hɔ a wɔkamfo kyerɛ sɛ wɔmfa nni dwuma wɔ Jeavons Syndrome ho. Mprempren nsɛm biara nni hɔ a ɛbɛfoa ketogenic aduan a wɔde di dwuma sɛ tebea yi ano aduru.

Dɛn ne abofra a ɔwɔ Jeavons Syndrome daakye? (Nkɔmhyɛ) .

Ɛsono abofra a ɔwɔ saa tebea yi daakye a egyina abofra biara tebea pɔtee so . Wo ba no oduruyɛfo ne onipa a oye sen biara a obehu eyi ho asɛm.

Wonnya Jevons yare no ano aduru biara , nanso wɔreyɛ nhwehwɛmu pii. Ɛwom sɛ ɛyɛ tebea a ɛkɔ so wɔ nkwa nna nyinaa mu de, nanso ayaresa ahorow wɔ hɔ a wɔde siw akisikuru ano .

So Jeavons Syndrome ka abofra nyansa?

Akisikuru ne n’ani a ɛyɛ mmerɛw betumi asiw abofra no kwan sɛ obesua ade wɔ sukuu dan mu . Sɛ wɔantumi mmue wɔn ani yiye a, ebetumi ayɛ den sɛ wɔde wɔn ho bɛhyɛ adesua mu koraa. Mmofra pii a wɔwɔ Jevons syndrome no yɛ den kakra sɛ wobesua ade wɔ sukuu mu na ebia wobehia nhomasua mu mmoa foforo . Nanso, tebea yi nka abofra nyansa tẽẽ .

Sɛ abofra no nyin kyɛ a, ebetumi ayɛ sɛnkyerɛnne a ɛkyerɛ sɛ awosu bi wɔ hɔ a ɛde Jevons yare no ba.

So Jeavons Syndrome ka nkwa nna?

Jeavons Syndrome nka abofra nkwa nna tẽẽ . Nkwa nna a ɛfata betumi aba. Nanso, sɛnea akisikuru no mu yɛ den ne sɛnea abofra no nipadua yɛ n’ade wɔ ayaresa ho no betumi aka abofra no akwahosan nyinaa. Oduruyɛfo betumi aboa ma wɔahwɛ tebea no so na wɔadi ho dwuma wɔ abofra no nkwa nna nyinaa mu.

Bere bɛn na ɛsɛ sɛ mekɔ oduruyɛfo nkyɛn?

Sɛ wo ba no nya akisikuru nea edi kan a, frɛ wɔn a wɔhwɛ ntɛmpɛ so ntɛm ara anaa fa wɔn kɔ ayaresabea.

Sɛ wɔahu sɛ wo anaa wo ba anya Jeavons Syndrome, sɛ wowɔ akisikuru anaa sɛnkyerɛnne ahorow a ɛte sɛ nea ɛreyɛ kɛse, anaasɛ sɛ worenya ayaresa no mu nsunsuanso bɔne a, kɔ oduruyɛfo nkyɛn ntɛm ara . Wo duruyɛfo betumi aboa wo ne wo ba no ma woadi tebea no ho dwuma na woabua wo nsɛmmisa.

Nsɛm bɛn na ɛsɛ sɛ mibisa oduruyɛfo no?

• "Oduruyɛfo/Madam, ayaresa bɛn na eye ma me ba?"
• "Dɛn ne nneɛma bɔne a ebetumi afi saa ayaresa ahorow yi mu aba?"
• "So wokamfo kyerɛ sɛ fa ahwehwɛ soronko bi di dwuma na ama hann ate nka?"
• "Mɛyɛ dɛn aboa me ba wɔ sukuu mu?"

So Sunflower Syndrome ne Jeavons Syndrome wɔ abusuabɔ?

Sunflower Syndrome ne Jeavons Syndrome yɛ tebea abien a ɛte saa ara . Sunflower Syndrome nso yɛ akisikuru bi a aniwa mu ntini a ɛyɛ mmerɛw ka ho. Wɔ eyi mu no, obi betumi adan akɔ hann fibea bi a ɛhyerɛn (te sɛ owia hann) so na wawosow ne nsa wɔ n’ani anim. Eyi betumi ama hann no asakra na ama obi anya akisikuru.

Nea etwa to no, nneɛma a ɛsɛ sɛ wokae (Take-Home Message) .

Ɛtɔ da bi a yɛn werɛ fi sɛnea hann ho hia ma yɛn da biara da asetra. Sɛ nhwɛso no, hann boa yɛn ma yehu ade wɔ sum mu na yenyin afifide. Nanso wɔ obi a ɔwɔ Jeavons Syndrome fam no, hann betumi ama wanya akisikuru . Oduruyɛfo betumi aboa ma woadi sɛnea hann ka wo ba no ho dwuma. Ebia wɔbɛkyerɛw nnuru ne ahwehwɛ soronko a wɔde kata aniwa no so.

Kae:

• Jeavons Syndrome yɛ akisikuru tebea a ɛntaa nsi nanso wotumi di ho dwuma .
• Nsɛnkyerɛnne atitiriw ne n’ani a ɛbɔ mpofirim, hann a wɔte nka, ne hann a ɛyɛ hu .
• Ɛwom sɛ eyi yɛ asetra nyinaa tebea de, nanso wobetumi de nnuru ne asetra mu nsakrae adi so .
• Ma w’adwene nsi wo ba no adwenemyare nso so . Sɛ ɛho hia a, hwehwɛ mmoa a efi animdefo mu.
• Akisikuru a ɛkyɛ bɛboro simma 5 anaasɛ akisikuru a ɛtoatoa so no yɛ ade a egye ntɛmpɛ! Hwehwɛ ayaresa ntɛm ara.

Mewɔ anidaso sɛ saa nsɛm yi aboa wo ma woanya tebea yi ho ntease bi. Mepɛ sɛ wo ba no ho tɔ no ntɛm!

` Jevons syndrome, akisikuru, akisikuru, aniwa anim myoclonus, mmofra ayaresa, hann ho nkate, EEG