I naute thluak sir khat chu a lian lutuk em? Hemimegalencephaly chungchang hi i zir dawn teh ang!

I fa naupang lu pianzia te, a nih loh leh a fit fo thin dan te hi i ngaihtuah ngai em? A châng chuan, heng thilte hnuaiah hian damdawi lam thil awm lo tak a awm thei bawk. Hemimegalencephaly hi chutiang natna pakhat a ni. A hming hi a buaithlak deuhin a lang thei a, mahse a awlsam zawngin sawi ila.

Hemimegalencephaly hi eng nge ni?

A awlsam zawngin, hemimegalencephaly chu i fa thluak sir khat (cerebral hemisphere) chu a sir dang aiin a lian lo hle a ni. Kan thluak hi tumor lian tak, a chanve pahnih, veilam leh dinglam nei angin han ngaihtuah teh. Hetiang dinhmunah hian a sir pakhat chauh hi a lian tur aia lian a ni. A châng chuan he tihpunna hi chu thluak lam hmun tlemteah chauh a awm thei , a nih loh leh a sir zawng zawng chu a lian thei bawk.

Hetianga thluak sir khat a lo lian chuan thluak chhungah hian inthlak danglamna dang a thleng thei a ni. Entir nan:

• Neurons hi kan thluaka cell chi khat a ni. An inrual tawk lo (cytoarchitecture) emaw, thluak chunglam layer chu a lo insiam tha lo (cortical dysplasia) emaw a ni thei.
• Corpus callosum bo emaw, chhia emaw: Hei hi thluak kil hnih inzawmkhawmna kawngpui ang a ni . A bo emaw, a chhiat emaw chuan harsatna a awm thei.
• A natna vei lam thluaka fluid-a khat cavities (ventricles) te chu a lian chho zel.

Hetianga thluak peng thenkhat hi a lo thang tha lo a, seizure a awm fo thei a ni. Hei hi epilepsy kan ti bawk . Vawi tam tak chu, heng seizure te hi antiseizure damdawi pangngai hmanga control a harsa thin . Damdawi hmanga seizures control theih loh chuan naupang tam tak chu thluak zai (epilepsy surgery) an neih a ngai a ni.

Hemimegalencephaly chi hrang hrang a awm em?

Ni e, he natna hi chi hrang hrang lian tak tak a awm a:

• Isolated hemimegalencephaly: Hei hian naupang thluak pawn lam layer (cerebral cortex) chauh a nghawng a ni. Vun leh taksa peng dang inrawlhna a awm lo.
• Syndromic hemimegalencephaly: Hei hian thluak chauh ni lovin, naupang vun leh taksa peng dang thenkhat a nghawng a ni. EngpawhniseVun natna lan chhuah dan hi pian hnu thla emaw kum tam tak emaw pawh a ni thei. Tin, naute taksa sir khat chu a sir dang aiin a lian zawk thei bawk (hemihypertrophy).
• Total hemimegalencephaly: Hemiah hian naupang cerebellum leh a chang chuan thluak zung pawh a lian chho thin. Hei hi isolated emaw syndromic emaw pawhin a thleng thei.

Hetiang natna lan chhuah dan hi engte nge ni?

I hriat hmasak ber tur chu i naute hian ``infantile spasms or seizures '' a nei a ni. Hengte hi focal seizures a ni tlangpui. Chumi awmzia chu naute taksa sir khat chauh a inher thei tihna a ni.

Symptom dang hmuh theihte chu:

• Lu lian zawk (Macrocephaly).
• Lu pianzia inang lo tak.
• Kal leh tlan ang chi chetna lama harsatna.
• Taksa sir khatah rilru hah leh chak lohna.
• Hmasawnna tlai: Hei hian an kum nena inmil taka thusawi, inkhelh leh zir tlai tihna a ni.
• Mit lama harsatna: Entirnan, mit pahniha en hian hmuh theihna hmun chanve a bo (hemianopia).

Eng thilin nge hemimegalencephaly hi a thlen?

Dik tak chuan scientist-te chuan he natna hi eng nge a thlen tih chiang takin an la hre lo. Ṭhenkhat chuan genetic niin an ngai a, chu chu naupang DNA inthlak danglamna avanga lo awm tihna a ni . Mahse, thlahtu a ni lo. Chu chu condition i nei tihna a ni a, i fa hnena i pass thil a ni lo tihna a ni.

A nihna takah chuan naupai kar thumna vel hian thluak thanlenna atana pawimawh gene pakhatah random emaw, mahni insiamthatna emaw a awm thin. He natna hi naupai laia i thil tih avanga lo awm a ni lo tih hre reng ang che. Hei hi i sualna leh pa sualna a ni lo.

Hetiang dinhmun tichhe thei risk factors te chu engte nge ni?

Hemimegalencephaly hi genetic condition dang nen a inzawm thei a ni. Hei hian a hnuaia natna nei naupangte hi hemimegalencephaly vei theihna a sang zawk tihna a ni:

• `Epidermal nevus syndrome` tih a ni
• `Klippel-Trenaunay natna a ni` tih hi a ni
• `McCune-Albright natna a ni` tih hi a ni
• ``Neurofibromatosis chi khat (NF1)'' tih hi a ni.
• `CLOVES syndrome` tih a ni
• `Proteus syndrome` tih a ni
• `Ito-a hypomelanosis a awm`

Engtin nge hemimegalencephaly hi an hriat theih?

Chutiang chu a nih leh nih loh chiang taka hriat theih nan i fa doctor chuan i fa chu a enfiah hmasa ang a, i fa hriselna chanchin a zawt hmasa ang che. Tichuan, a hnuaia test te hi an ti thei ang:

• Thluak MRI scan a ni.
• EEG (Electroencephalogram) hmanga enfiah a ni. Hei hian thluak electrical activity a teh a ni.

Tunlai technology hmasawn tak hmang hian naute chu nausen pum chhunga a la awm laiin ``naupai laiin`` he natna hi hriat theih a ni fo thin.`` Hei hi naute thluak MRI scan bik ``fetal MRI`` tih hmanga tih a ni.

Engtin nge enkawl a nih?

Hemimegalencephaly enkawlna tum ber chu seizures control hi a ni. Heng seizures te hi a chang chuan kum naupang tak atangin a intan thei a, damdawi (drug-resistant) hmanga control a harsa fo bawk.

Hetiang dinhmun hian fit chi hrang hrang a thlen thei a ni. Naupan laia fit lo awm, a bik takin nausen atanga tan a nih chuan naupang hmasawnna kawngah nghawng lian tak a nei thei tih hriat a pawimawh. Fit thunun loh hian naupang thanlenna leh thanlenna nasa takin a tichhe thei a, chubakah thluak lo thanglian zel chu a tichhe thei bawk. Chuvangin a fit hi a rang thei ang bera tihtawp a pawimawh.

Seizure enkawlna hi anti-seizure damdawi hmanga tan a ni. Naupang thenkhat chuan heng damdawi hmang hian an dam chhungin an seizure an control thei a ni. Mahse mi tam tak chuan damdawi-resistant epilepsy an nei a. Tichuan hemispherectomy an tih surgery an ti leh a. Hei hian naupang thluak (cerebral cortex) a natna hmun chu lakchhuah emaw, thluak sir lehlamah then hran emaw a huam a ni.

He surgery hi an tih hian "functional hemispherectomy" an ti \hin. He surgery-ah hian surgeon chuan naupang thluak sir khat leh a sir lehlamah thlunzawmtu tissue leh nerve te chu a tikehsawm a, a then a, mahse a thluak lam lian lo tak chu luruh chhungah a awmtir thei a ni. "Anatomical or complete hemispherectomy"-ah chuan thluak sir khat chu a sir lehlamah a inthen a, a lam pangngai lo chu a chhuak vek a ni. He surgery hi neurosurgeon, epilepsy surgery lama training bik neiin a tih tur a ni.

Thluak chanve lakchhuahna operation (hemispherectomy) hi a harsa em?

Dik tak chuan hemispherectomy hi thluak surgery harsa tak a ni. Hemimegalencephaly natna avang hian he surgery tih hi a harsa zawk chhan hrang hrang a awm.

A hmasa ber chu thisen kalna kawng hrang hrangte hi a danglam bik takin a awm fo thin. Chuvangin surgery lai hian hmuh leh cut harsa tak an ni. Hei hian a chang chuan thisen chhuah tam lutuk a thlen thei.

Chu bâkah, naupang thluak pum pui chu a pianzia inang lo tak a nih avângin, surgeon-in thluak pêng hrang hrang hriat theihna tûra a hman landmark-te chu hmuh theih a ni lo fo ṭhîn. Tin, naute leh naupang te te pawhin he surgery neih chhung hian thisen tam tak an hloh thei bawk. Hei hi uluk taka enfiah a ngai a, a tul angin thisen thlak a ngai bawk.

Chuvangin, i fa enkawltu medical team te hi he surgery-a team thiam leh tawnhriat ngah tak an nih a pawimawh hle. Hemimegalencephaly vei naute hemispherectomy tih fo thin leh he procedure lama tawnhriat ngah tak nei damdawi inah he surgery hi tihsak hi i fa tan a him zawk.

Hetiang natna vei naupang tan hian eng prognosis nge ni?

I fa dinhmun a lo thanlen dan tur chu a fit control theih dan leh thluak lo thanglian dan pangngai lo chu a sir khatah emaw, a sir lehlamah emaw a awm leh awm lohah a innghat nasa hle.

Hemimegalencephaly hi spectrum condition a ni a, naupang tinte chu a inang lo vek a ni. Chumi awmzia chu naupang tinah result a inang lo thei hle tihna a ni.

• Naupang tam tak chuan rilru lama harsatna engemaw chen an nei thei .
• Naupang thenkhat, fits control tha tak nei te chuan an finna pangngai hnaih tak an nei thei.
• Mahse naupang dang thenkhat tan chuan kea kal te, thusawi te, finna lama hmasawnna te hi nasa takin a nghawng thei a ni.

Hemimegalencephaly vei naupang zawng zawng deuhthaw hian taksa sir khatah chak lohna an nei (hemiparesis) . Hei hi cerebral palsy chi khat a ni. Naupang \henkhat chuan chak lohna tenau an nei thei a, \henkhat erawh chuan chak lohna nasa tak an nei thei thung. Naupang thenkhat chuan homonymous hemianopsia tih natna an nei thei a, chutah chuan an mit hmuhna hmun chanve chu a bo vek a ni. Naupang tam tak chu an kal thei a, an tawng thei a, mahse naupang zawng zawngin an ti thei vek lo. Naupang thenkhat chuan chaw pekna tur bik an mamawh a, thenkhat erawh chuan a pangngaiin an ei thei thung.

Tun hnaia zirchianna pakhatah chuan operation hnuah:

• Naupang 68% ah fit hi a tawp vek tawh a ni.
• Naupang 43% chuan an finna chu a vaiin emaw, a tlahniam lo emaw a ni.
• Naupang 26% chuan an kum nena inmil takin thu an sawi thei tawh a ni.
• Naupang 21% chuan lehkha chhiar an thiam tawk tawh a ni.

Hemimegalencephaly hi ven theih a ni em?

Scientist te hian he natna hi eng nge a thlen tih chiang taka an la hriat loh avangin ven theih dan tur a awm lo.

Hetiang natna nei naupang hi engtin nge i enkawl ang?

I fain Hemimegalencephaly natna a neih chuan aMedical team lian tak mamawh a ni. I fa medical team te nen tangkawpin i fa mamawh phuhruk thei tur ruahmanna i siam thei ang. I fate medical team-ah hian:

• Naupang enkawltu doctor te
• Neurologist te an ni
• Neurosurgeon te an ni
• Hmasawnna lama mithiamte
• Speech therapist te, occupational therapist te, leh physical therapist te an ni
• Hriselna lama thawktu dangte.

I fa doctor hnenah eng zawhna nge i zawh tur?

I fa chu hemimegalencephaly a vei tih hmuhchhuah a nih chuan doctor hnenah heng zawhnate hi i zawt thei ang:

• Ka fa hian eng ang hemimegalencephaly nge a neih?
• Ka fa hian damdawi lam harsatna dang a nei em?
• Enkawlna tha ber chu eng nge ni?
• Ka fate thlirna chu eng nge ni?
• A symptoms tihziaawmna turin eng nge kan tih theih ang?

Nu leh pa, huaisenna tlem tal nei rawh u! (In lama hruai tur thuchah)

I fain seizure a neih hmuh emaw, Hemimegalencephaly ang natna an neih thu hriat emaw chu thil mak tak leh thinlung ti na thei tak a ni tih kan hria. Mahse, nangmah chauh i ni lo tih hre reng ang che. I fa enkawltu medical team chu i kalna apiangah i bulah an awm reng a ni.

Hetiang dinhmun hi i hmachhawn harsat a nih chuan, i doctor hnenah hemimegalencephaly vei naupang chhungkaw tanpuitu support group chungchang zawt rawh. Hetiang natna vei naupang nu leh pate biak hian i hlauhthawnna tihziaawmna leh i fate dinhmun chu i pawm theih nan a pui thei che a ni. I fain chu natna nena nun a zir chhoh zel chuan i tan chakna ropui tak an ni thei a ni.

` Hemimegalencephaly, thluak than dan pangngai lo, nausen epilepsy, thluak surgery, hmasawnna tlai, genetic condition, neurological diseases