ʻO West Syndrome, i kapa ʻia hoʻi ʻo infantile spasms, he ʻano kakaikahi o ka epilepsy i nā pēpē a me nā keiki e hoʻomaka ana i ka makahiki mua o ke ola, maʻamau ma waena o 4 a me 8 mau mahina. Hōʻike ʻia ia e ka spasms, hopu a i ʻole ka hoʻihoʻi ʻana o ka ulu ʻana o ka psychomotor o ka pēpē a me ka hana o ka lolo. He loli loa ka prognosis a pili i nā kumu kumu o ka spasms, hiki ke nui. Hiki iā ia ke hoʻoulu i ka moʻo koʻikoʻi a me ka naʻauao sequelae a holomua i nā ʻano epilepsy ʻē aʻe.

ʻO nā spasms ka hōʻike mua loa o ka maʻi, ʻoiai ua hiki i ka pēpē i hoʻololi ʻia ma mua o lākou. Loaʻa maʻamau lākou ma waena o 3 a me 8 mahina, akā i nā hihia liʻiliʻi hiki ke maʻi ma mua a ma hope paha. ʻO nā ʻokiʻoki pōkole loa (hoʻokahi a ʻelua kekona) i hoʻokaʻawale ʻia, ʻoi loa ma ke ala ʻana a i ʻole ma hope o ka ʻai ʻana, e hāʻawi mālie i ka poha o ka spasms e hiki ke hoʻomau no 20 mau minuke. I kekahi manawa, ʻōwili ʻia nā maka i ka manawa o ka hopu ʻana.

ʻO nā spasms wale nō nā hōʻailona ʻike ʻia o ka hana mau ʻana i ka hana lolo e hōʻino ai iā ia, e hopena i ka hoʻomohala ʻana o ka psychomotor. No laila, hele pū ʻia ke ʻano o ka spasms me ka stagnation a i ʻole ka regression o nā mana psychomotor i loaʻa mua: nā pilina e like me ka minoʻaka, ka hopu ʻana a me ka hoʻopunipuni ʻana i nā mea ... Hōʻike ka electroencephalography i nā nalu o ka lolo i kapa ʻia ʻo hypsarrhythmia.

ʻO nā spasms ma muli o ka hana hewa o nā neurons e hoʻokuʻu ana i ka hoʻokuʻu ʻana i ka uila. Hiki ke ʻike ʻia nā maʻi he nui i ke kumu o West syndrome a hiki ke ʻike ʻia i loko o ʻekolu hapaha o nā keiki i hoʻopilikia ʻia: ka ʻeha hānau, ka lolo, ka maʻi, ka maʻi metabolic, genetic defect ( Down syndrome, no ka laʻana), nā maʻi neuro-cutaneous ( ʻO ka maʻi o Bourneville). ʻO ka hope ka maʻi maʻamau e pili ana i ka West syndrome. ʻO nā hihia i koe e ʻōlelo ʻia he "idiopathic" no ka mea ʻaʻohe kumu ʻike ʻia, a i ʻole "cryptogenic", ʻo ia hoʻi e pili ana i kahi anomaly ʻaʻole mākou i ʻike pehea e hoʻoholo ai.

ʻAʻole lele ʻo West's syndrome. Hoʻopilikia pinepine ia i nā keikikāne ma mua o nā kaikamahine. ʻO kēia no ka mea pili kekahi o nā kumu o ka maʻi i kahi kīnā genetic i pili i ka chromosome X e pili pinepine ana i nā kāne ma mua o nā wahine.

ʻAʻole hiki ke ʻike ʻia ka maʻi ma mua o ka puka ʻana o nā hōʻailona mua. ʻO ka lāʻau lapaʻau maʻamau, ʻo ia ka lawe ʻana i ka lāʻau anti-epileptic ma ka waha i kēlā me kēia lā (ʻo Vigabatrin ka mea maʻamau i kuhikuhi ʻia). Hiki ke hui pū me nā corticosteroids. Hiki ke hoʻopili ʻia ke ʻoki ʻana, akā ʻokoʻa loa, ke hoʻopili ʻia ka maʻi maʻi i nā ʻeha lolo kūloko, hiki i kā lākou wehe ʻana ke hoʻomaikaʻi i ke kūlana o ke keiki.

He loli loa ka prognosis a pili i nā kumu kumu o ka maʻi. ʻOi aku ka maikaʻi i ka wā ʻelemakule o ke pēpē i ka manawa o ka hoʻomaka ʻana o nā spasms mua, ʻoi aku ka maʻi o ka mālama ʻana a ʻo ka maʻi idiopathic a cryptogenic paha. ʻO 80% o nā keiki i hoʻopilikia ʻia he sequelae i hiki ʻole ke hoʻololi ʻia i kekahi manawa a ʻoi aku ka koʻikoʻi: nā maʻi psychomotor (lohi i ka ʻōlelo ʻana, hele wāwae, a me nā mea ʻē aʻe) a me ke ʻano (ka hoʻihoʻi ʻana iā ia iho, hyperactivity, deficit attention, etc.). (1) Hiki pinepine nā keiki me West syndrome i ka maʻi epileptic ma hope, e like me Lennox-Gastaut syndrome (SLG).