Dravet maʻi pale. Loa myoclonic epilepsy o kaʻuʻuku mai

Mawaena o na hoikeana likeʻole o ka noho 'ana kamaliʻi epilepsy Dravet maʻi pale mea he kūikawā wahi, a mea paha i ka loa ka wī a me ke ola-hooweliweli kāna hana o ke keiki. Kēia maʻi pale hoike mai ai ia ia iho i loko o ka makahiki mua o ke ola i ke keiki a me ka pinepine hiki aku ai i ka mea hoʻohuikau o kana psychomotor? Acaeoey, a ia ma kekahi hihia - a me ka make. He mea nō He pohihihi keia maʻi a me kona papa kuhikuhiE symptoms a me nā nohoʻana, a me mākou e kamaʻilio i loko o kēia 'atikala.

Ma kekahi mau hihia, hōʻike i ke alo o Dravet maʻi pale?

Dravet maʻi pale mea ole, he pono ole - kaulana i keia maʻi ka mea i 1 kanaka no 40 tausani nā pēpē i (a me nā keiki e i ma kahi o 66% o nā hihia) .. Akā, i kēia, ma ke alanui, hiki aku ai i ka mea i pololeiʻike, kauka i kekahi manawa loaʻa mea paakiki e hoʻouka, a, penei, ua nalowale waiwai manawa. A i ka wā i kapaʻia maʻi pale me ka malama Inc ke keiki ka ke ano ana worsens me ka elemakule.

Inā he mea kekahi kamaiki nana i ole hiki i ka makahiki, pinepine bouts me ka pūkaʻina 'ole ke kūlana i loko o (' o ia hoʻi, E., Ua hahai kekahi i kekahi, a me ka hoʻomanawanui i loko o nā wahi i wāhiʻia,ʻaʻole ia e hele mai i ka manao) a i hoʻopale ai i ka hana ana o anticonvulsant ai ', hiki ke kēia maʻi pale Drava.

Children me keia pinepine i mau ho'āhewa wale i ka lā, a me keia ano mau mea e pili ana i ka hebedoma. A laila, ua hai hou he hoohiamoe ia no ka mau o ka pule, a me na mea a pau.

Kūlana epilepticus me Dravet maʻi pale - he 'ano, he pono ole kŰia. He hiki e ukali e convulsions paha e ole-convulsive, he okoa ka nui o na kanaka haunaele o ka ike paka i me ka segmental myoclonia (hookeai Muscle twitching).

Dravet maʻi pale: mea, lalau mai

I ka papa kuhikuhiE kumu o ka maʻi pale i hoakakaia ma ka noiʻi kapa he aaiaoe predisposition, o - ke alo i loko o ka hoʻomanawanui ka paʻakai Kanal'ōewe mutations.

Ano hoomainoino aʻe no ka hoouka kaua ana o ke ano i hoakakaia ma na kamalii uuku kuni pinepine hanaʻia i loko o ka maʻi, e lawe i ka wela auau, overheating, No hoi, ka mea hiki e loa luhi ole stimulation malamalama (ʻoaka o na kukui, ke hoʻololi, mai ka pouli keena i loko o ka brightly ho'āʻia, a no laila, ma luna o. N.). It E e kaulana i keia a pau i loko o nā kēia mau makahiki o ka hoʻomanawanui i ka ola e e weliweli, ma o na la likeʻole ho'āhewa wale ma luna o ka mana no ka mea.

Dravet maʻi pale: Symptoms

I ka papa kuhikuhiE hoikeana o Dravet maʻi pale hiki ke wahi, a me ka mea nui, a generalized kāʻili 'ana. Mea nui ke kāʻili 'ana e wehewehe aku i loko o ia ka excitation māhele' āina, me ka hoonaukiuki aku i ko lakou hanana, He hoʻokahi wale hapa o ka lolo. Ma ka hihia o pathological haʻawina o neurons i loko o nā hemispheres ia mea he generalized 'ana.

Convulsions ma Dravet maʻi pale pinepine i polymorphic ma ka ano. Keiki i loko o ka makahiki e eʻike piʻi clonic (me ka loli ma ka Muscle leo), tonic (i nā 'ano lōʻihi Muscle spasm) a me ka generalized myoclonic kāʻili' ana.

Pinepine loaʻa nō hoʻi ko lākou nei i loko o ka palapala o ka atypical wa e kaawale aku - kumu ma i ke keiki o ka ike paka i mea ole hapa 'ia' ole loa pane i ke kaiapuni. Ke kao keiki ke numb i ka manawa, huli i kekahi wahi, uma hoʻi, he ulia kulu paha e ninini i loko o kona mau lima.

Loa pinepine i hoike ia ke kāʻili 'hana ana i ala, e like me ka pono me ia ma ka Pa moku'āina (i loko o ka moe, ka mea i kākau 3 wale% o nā mea maʻi i kēiaʻike,).

E like me ka ulu 'lapaʻau hoikeana o Dravet maʻi pale?

E like me ka rula, Dravet maʻi pale ua wehewehe aku ma ka mea i na luna i oleloia symptoms ana i loko o ka kekahi kaʻina. Kauka maopopo ma waena o ekolu nui manawa o ka maʻi.

1. A kŘpa a mehana wā, a me keʻano o ka clonic kāʻili 'ana (e hoʻokēʻai ai twitch, kekahi ma hope o ka' ē aʻe, ma hope o ka pōkole wā o ka manawa). E like me ka hoʻonāukiuki kulana, e like me ka rula, ka mea kū i ke ana wela ala i loko o ke keiki, akā, i loko o ka wā e hiki mai ka mea hiki ke kū aʻe, a kū kaʻawale no ia.
2. E alaʻano pākīkē - me ka Hekikai o ka lehulehu myoclonic ke kāʻili 'ana. Ka mea, e febrile ( 'ole. E. ke kaumaha ma muli o ka mahana mea ulu) pinepine, a pili i ka kumu a me nāʻaoʻao. No ka myoclonic kāʻili 'hui atypical wa e kaawale aku ke kāʻili' ana a me ka luna 'mea nui ke kāʻili' ana.
3. Kūpaʻa manawa ma i ka nawaliwali ho'āhewa wale, oiai e waiho ana no ke keiki i ka nui a me ka neurological psychiatric kāna hana.

I ka papa kuhikuhiE symptoms o loa myoclonic epilepsy o ka noho 'ana kamaliʻi

Me ka makou i olelo mai, ma ka pili 'o Randy me ka mea Dravet maʻi pale - he pohihihi keia maʻi, o ka poe akamai i pinepine huli mea paakiki e diagnose ia. Nolaila, na makua mea nui e hoʻolako pololei 'ike e pili ana i ke kūpono' ana o ka pathological ana o ke keiki. hiki e kēia kapa maʻi pale ina he mea i loaʻa i ke kēia mau hiʻona:

• i ka maʻi i holo ai i kekahi makahiki o ka makahiki;
• polymorphous ke kāʻili (.. oa kā lākou nā hoikeana);
• maluna ua i pani? eaia typical anticonvulsants;
• i ka hanana o ke kāʻili 'ana i hīkiʻiʻia i ka mea ulu i loko o ke keiki ka kino mehana;
• ke kamaiki kekahi 'developmental' ole 'ana (i kēia hiʻona hiki ke hoike i ka loli iki ana degere);
• haʻi ataxia symptoms (raskoordinirovannosti neʻe);
• MRI hoike Aʻole e hoʻokūpaʻa i ke alo o ka maʻi (oi loa i ka hoʻomaka o ka maʻi);
• EEG - ka meheu o ke kāʻei kua pāpana a me ka multifocal aʻe 'ana i hoʻouna' ia spike in a lohi ale.

Ma waho aʻeo ia mau symptoms na keiki me Dravet maʻi pale ana wehewehe aku ma ke alo o ka hyperactivity a me ka hoʻolohe nele kāna hana.

Ei no? Acaeoey o Dravet maʻi pale

Ka olelo wanana a loa myoclonic epilepsy mea IeAUPIIe, IAa IO noho lakou malaila. All nā mea maʻi diagnosed me Dravet maʻi pale nānā noʻonoʻo retardation, a me ka hapalua o ka mea i kaumaha. Ma hope o ka makahiki o ka eha nā mea maʻi he mea kekahi e holumua ai mea hōʻemi, a me ka hooulu ana o ka 'ano' abnormalities, me psychosis.

Eia naʻe, lethal i ka ho'ākāka 'ia ma pathologies Ua hoi loa kiʻekiʻe - mai i 18%, a me ka ia ke kumu o ka loa pinepine he ulia pakalaki i ke kāʻili' ana a me ke kūlana epilepticus.

I mea e emi i ka pilikia o ka mea hopena i ke kāʻili 'ana i o ke keiki, na makua pono i ka naauao maikai o ana i ka hoʻolako' ana i mua kokua no ke kāʻili 'ana.

Pehea e hōʻike mua kōkua no ke kāʻili 'ana, kena ae la ma ka kiʻekiʻe mahana?

Inā i ke keiki i hopu ia ma muli o ia ke ala ma ka wela (a, e like me oe i keia iloko o, keia maʻi o kekahi o na kumu nui hiʻona), mālama i ke kēia mau rula:

• Kau i ka pēpē ma ka palahalaha ili;
• i hou lewa;
• hoʻomaʻemaʻe i ka pēpē ka waha o mucus;
• huli ke poo o ke keiki ka i kekahiʻaoʻao.
• Pana aku au i antipyretic hana.

Inā i ke keiki i olelo i ka mai kuni, i mea, i kona lae wela, i kona maka flushed, a laila, e e pahuna mua kōkua no ka hopu ma hoemi i ka mahana (anu pulu oOeEIAOO ma ka lae, anu i ka armpits, a ma ka groin kahi, holoi i ke kino me ka wai me ka vīnega i loko o ka lākiō o ka 1 1, antipyretics).

Inā i ke kao keiki ma ka kāʻei kua o kiʻekiʻe ka mahana ili o kona maka, bluish lehelehe a me fingernails, ole, anu na wawae, a he mau pama, ka rubdown a anu compresses e ole e hana. Mehana hoʻi i hāʻawe E haawi antipyretics, a me nā papa "No-Spa" a me "Papaverine" i ka uku o ka 1 mg no kg 1 kino kaupaona no ka vasodilatation.

Kōkua ka wā ua kauohaʻia epileptic ke kāʻili

Ma ka hanana o ka ua kauohaʻia epileptic 'ana me ka generalized clonic a me ka tonic convulsions o ke keiki pono e:

• waiho ma luna o kekahi i lalo ili;
• ma lalo o ke poo, e apo ai i kekahi mea palupalu, i ka pēpē i i kaua ia;
• i ulu eaʻai ana;
• hoʻomaʻemaʻe i ka waha a me ka'ā'ī o nā mucus;
• huli i kou poo i kekahiʻaoʻao;
• hui i kekahi apana o ka lole i loko o ka knot, a pili ana ia ma waena i nā niho, e pale ai biting elelo a me nā lehelehe, e like me ke keiki o ka makahiki i ia i paa mea (i ka puna, he lāʻau), e uhaʻi au i kona mau niho;
• holoi iʻikeʻia waha hua pepa;
• i ka eʻoiaʻiʻo ia i ka hoouka i ke kamaiki, aole ia i ku ma kekahi mea.

Inā convulsions lawe i ka palapala kūlana, eʻoiaʻiʻo, e kapa aku "ambulance,"

Walaʻauʻana me nā loina o ka hana maikaʻi o nā keiki a me Dravet maʻi pale

maʻi keiki lapaʻau o ka maʻi i hoakakaia deceleration ua hoemi ia i ka pale aku i ka ulu ana o ke kāʻili 'ana a me kā lākou kūlana ke ano.

A hiki i kaʻike, o ka iapaau ana "Dravet maʻi pale" nele ka neʻe aku i ka hoʻohana 'ana i lohe nuiʻia antiepileptic ai': "Carbamazepine", "Finlepsin", "Phenytoin" a me ka "lamotrigine" no ka mea, worsen wale i ke ahonui o ka kanawai, compounding i loko o nei ano o ke kāʻili 'ana.

Ma waho aʻeo ia OAXA lāʻau lapaʻau, ia mea nui, e halihali i ka manao o ka Kāohiʻana o ke kuni, no ka mea, o ka moku'āina pakahi aku la ia pilikia i ka hoʻomanawanui. E hookaawale ia hoonaukiuki ho'āhewa wale o ka malamalama stimulation i kaumaha aku e komo nā aniani kaulona makaaniani me ka uliuli a me kekahi lipine aniani.

Dravet maʻi pale Uluwehi O Inc

Inā i kaʻike, ua hookupaa iho la ia, lapaau hoʻomaka i ka hoʻomaka 'ana o ka lāʻau "Topiramate". Ua Ua hoakaka ia mahele lāʻau o 12,5 mg / lā., Ua mahuahua no 3-10 mg / kg / lā. (Medicine ia i nā manawaʻelua i ka lā). Kēia mea paahana mea oi loa aku hoi pono ma ka hihia kahi keiki i ho'ākāka 'ia epilepsyʻikea generalized ke kāʻili a paroxysms o convulsions me ka hoʻololiʻana, mai kekahi hapa o ke kino a hiki i kekahi (gemikonvulsiyami),

ʻO kēia mau mea haʻilula keimikala no monotherapy - he derivative o valproicʻakika (ka malakeke "Konvuleks", "Konvulsofin", a pela aku ...) - mea pakahi aku la ia mana i loko o atypical wa e kaawale aku, a myoclonus, a me barbituricʻakika ( "Phenobarbital") hoʻohana 'ia i loko o generalized convulsions me ka propensity a hiki i ke kūlanaʻikena. Incidentally, ma keia hihia, a ua kaulana kiʻekiʻe ka hoʻokōʻana o bromides.

Inā pono, e hoʻohana i ka huihui 'o ai'. Ka loa mana o ia mea he huihui 'ana o ka valproate meʻano "topiramate".