ʻO Lambert-Eaton Myasthenic Syndrome

Anter

• He aha nā hōʻailona o Lambert-Eaton Myasthenic Syndrome?
• He aha ke kumu o Lambert-Eaton Myasthenic Syndrome?
• Kuhi ʻia ʻo Lambert-Eaton Myasthenic Syndrome
• Ke mālama nei iā Lambert-Eaton Myasthenic Syndrome
• He aha ka hiʻohiʻona lōʻihi?

He aha ka Lambert-Eaton Myasthenic Syndrome?

ʻO Lambert-Eaton myasthenic syndrome (LEMS) kahi maʻi autoimmune laha ʻole e hoʻopili i kou hiki ke neʻe. Hoʻouka kāu ʻōnaehana pale ʻole i nāʻiʻo o ke kino e hiki ai i ka hele wāwae paʻakikī a me nā pilikia muscular ʻē aʻe.

ʻAʻole hiki ke hoʻōla ʻia ka maʻi, akā hiki i nā ʻōuli ke hōʻemi iki inā hoʻoikaika ʻoe iā ʻoe iho. Hiki iā ʻoe ke hoʻokele i ke ʻano me ka lāʻau.

He aha nā hōʻailona o Lambert-Eaton Myasthenic Syndrome?

ʻO nā ʻōuli kumu o ka LEMS ka nāwaliwali o ka wāwae a me ka paʻakikī e hele wāwae. Ke holo nei ka maʻi, e ʻike ʻoe:

• nāwaliwali i nā mākala maka
• ʻōuli muscular ʻole
• kōpaʻa
• waha maloo
• maʻiʻole
• nā pilikia pākī

Hoʻonui pinepine ka nāwaliwali o ka wāwae i ka manawa pōkole ma ka hoʻoikaika ʻana. I kou hoʻoikaika kino ʻana, kūkulu ʻia ka acetylcholine i ka nui e ʻae ai i ka ikaika e hoʻomaikaʻi ai no kahi manawa pōkole.

Nui nā pilikia e pili ana me LEMS. Pākuʻi ʻia kēia mau mea:

• pilikia i ka hanu ʻana a me ka ale ʻana
• nā maʻi maʻi
• nā ʻeha ma muli o ka hina ʻana a i ʻole nā ​​pilikia me ka hoʻohui ʻana

He aha ke kumu o Lambert-Eaton Myasthenic Syndrome?

I kahi maʻi autoimmune, kuhi hewa ka ʻōnaehana pale o kou kino i kou kino ponoʻī no kahi mea haole. Hoʻopuka kāu ʻōnaehana pale pale i nā mea ʻino e hoʻouka i kou kino.

I ka LEMS, hōʻeha kou kino i nā hopena nerve e kaohi i ka nui o ka acetylcholineyour e hoʻokuʻu i kou kino. ʻO ka Acetylcholine kahi neurotransmitter e hoʻonāuki i nā kuʻikuʻi mākala. ʻO nā kuʻikuʻi Muscle e ʻae iā ʻoe e hana i nā neʻe manawaleʻa e like me ka hele wāwae ʻana, ka wili ʻana i kou mau manamana lima, a me ke kau ʻana i kou poʻohiwi.

Kūlike, hoʻouka kou kino i kahi protein i kapa ʻia voltage kanal gated calcium channel (VGCC). Koi ʻia ʻo VGCC no ka hoʻokuʻu ʻia o acetylcholine. ʻAʻole hana ʻoe i ka acetylcholine lawa ke hoʻouka ʻia ʻo VGCC, no laila ʻaʻole hiki i kou mauʻiʻo ke hana pono.

Pili nā hihia he nui o ka LEMS i ka maʻi ʻaʻai pūhaka. Manaʻo nā kānaka noiʻi hoʻopuka nā hunaola maʻi ʻaʻai i ka protein VGCC. ʻO kēia ke kumu o kāu ʻōnaehana pale pale e hana i nā mea pale kūʻē iā VGCC. Hoʻouka kēia mau mea ʻino i nā hunaola maʻi ʻaʻai a me nā hunaola mākala. Hiki i kēlā me kēia mea ke hoʻomohala iā LEMS i ko lākou ola, akā hiki i ka maʻi ʻaʻai māmā ke hoʻonui i kou makaʻu i ka hoʻomohala ʻana i kēia ʻano. Inā loaʻa kahi mōʻaukala o nā maʻi autoimmune i kou ʻohana, hiki paha iā ʻoe ke makaʻu kiʻekiʻe i ka hoʻomohala ʻana iā LEMS.

Kuhi ʻia ʻo Lambert-Eaton Myasthenic Syndrome

No ka hōʻoia ʻana iā LEMS, e lawe kāu kauka i kahi mōʻaukala kikoʻī a hana i kahi hoʻokolokolo kino. E nānā ana kāu kauka:

• hoʻemi i nā reflexes
• nalowale ʻaʻa puʻupuʻu
• nāwaliwali a i ʻole nā ​​pilikia e neʻe ana e ʻoi aku ka maikaʻi me ka hana

Kauoha paha kāu kauka i kekahi mau hoʻokolohua e hōʻoia i ke ʻano. E nānā kahi hōʻike koko i nā mea ʻino e kūʻē iā VGCC (anti-VGCC antibodies). ʻO kahi electromyography (EMG) e hoʻāʻo i kou mau olonā i ka ʻike ʻana pehea lākou e hana ai i ka wā e hoʻonāukiuki ai. Hoʻokomo ʻia kahi nila liʻiliʻi i ka mākala a pili i ka mika. E noi ʻia ʻoe e ʻaelike i kēlā mākala, a e heluhelu ka mika i ka maikaʻi o ka pane ʻana o kou mau mākala.

ʻO kahi hoʻāʻo hou paha ka hoʻāʻo o ka wikiwiki conduction nerve (NCV). No kēia hōʻoia, kau kau kauka i nā uila i ka ʻili o kou ʻili e uhi ana i kahi mākala nui. Hāʻawi nā pākuʻi i kahi hōʻailona uila e hoʻonāukiuki i nā aʻalolo a me nā mākala. ʻO ka hana i hopena ʻia mai nā aʻalolo i hoʻopaʻa ʻia e nā uila uila ʻē aʻe a hoʻohana ʻia e ʻike ai i ka wikiwiki o ka hopena o nā aʻalolo i ka hoʻonāukiuki.

Ke mālama nei iā Lambert-Eaton Myasthenic Syndrome

ʻAʻole hiki ke hoʻōla ʻia kēia ʻano. E hana pū ʻoe me kāu kauka e hoʻokele i nā kūlana ʻē aʻe, e like me ka maʻi ʻaʻa maʻi.

Koi paha kāu kauka i ka immunoglobulin intravenous (IVIG) i ka lāʻau. No kēia mālama ʻana, e hoʻomoʻi kāu kauka i kahi antibody nonspecific e hōʻoluʻolu ai i ka ʻōnaehana pale. ʻO kekahi hana kūpono he plasmapheresis. Lawe ʻia ke koko mai ke kino, a hoʻokaʻawale ʻia ka plasma. Wehe ʻia nā mea ʻino, a hoʻihoʻi ʻia ka plasma i ke kino.

ʻO nā lāʻau e hana pū me kāu ʻōnaehana muscular hiki i kekahi manawa ke hoʻomaha i nā ʻōuli. Hoʻopili kēia i ka mestinon (pyridostigmine) a me 3, 4 diaminopyridine (3, 4-DAP).

He paʻakikī kēia mau lāʻau i loaʻa, a pono ʻoe e kamaʻilio me kāu kauka e ʻike i ka ʻike hou aku.

He aha ka hiʻohiʻona lōʻihi?

Hiki ke hoʻomaikaʻi ʻia nā ʻōuli ma ka mālama ʻana i nā kūlana kumu ʻē aʻe, kaomi ʻana i ka ʻōnaehana paleʻoniʻoni, a i ʻole ka wehe ʻana i nā mea ʻino mai ke koko. ʻAʻole pane maikaʻi nā kānaka āpau i ka lāʻau. E hana pū me kāu kauka e kiʻi i kahi hoʻolālā lapaʻau kūpono.