ʻO Lambert-Eaton maʻi

ʻO Lambert-Eaton Syndrome (LES) kahi maʻi laha ʻole kahi kamaʻilio hewa ʻole ma waena o nā aʻalolo a me nā mākala e alakaʻi ai i ka nāwaliwali o nā mākala.

He maʻi maʻi autoimmune ʻo LES. Kuhi hewa kēia i kāu ʻōnaehana pale pale i nā hunaola olakino a me nā ʻaʻa i ke kino. Me LES, nā mea ʻāpala i hana ʻia e ka ʻōnaehana pale pale i nā hunaola nerve. ʻAʻole hiki i nā nerves ke hoʻokuʻu i kahi kemika i kapa ʻia ʻo acetylcholine. Lawe kēia kemikala i nā impulses ma waena o nā aʻa a me nā mākala. ʻO ka hopena ka nāwaliwali o nā mākala.

Hiki paha ʻo LES me nā maʻi ʻino e like me ka maʻi ʻaʻai liʻiliʻi liʻiliʻi a i ʻole nā ​​maʻi autoimmune e like me vitiligo, e hiki ai i ka nalo ʻana o ka ʻili kala.

Hoʻopili pinepine ʻo LES i nā kāne ma mua o ka wahine. ʻO nā makahiki maʻamau o ka hanana aia ma kahi o 60 mau makahiki. Kākaʻikahi ka LES i nā keiki.

Nawaliwali a nalowale paha o ka neʻe ʻana i hiki ke ʻoi aku a ʻoi paha o ke koʻikoʻi, me:

• Piʻi i ka piʻi ʻana i ke alapiʻi, ka hele wāwae ʻana a hāpai paha i nā mea
• ʻEha ʻeha
• Kaʻu ʻana o ke poʻo
• ʻO ka pono e hoʻohana i nā lima e ala aʻe mai kahi noho a moe paha
• Nā pilikia e kamaʻilio ana
• Pilikia i ka nau ʻana a me ka ale ʻana paha, i pili pū paha me ka gagging ʻana a me ke ʻānai ʻana
• Hoʻololi ka hihiʻo, e like me ka hihi o ka hihi, ʻike lua, a me ka pilikia e mālama pono i nā maka

Māmā ka nāwaliwali ma LES. Hoʻopili nui ʻia nā mākala o ka wāwae. Hiki ke hoʻomaikaʻi i ka nāwaliwali ma hope o ka hoʻoikaika kino, akā ʻo ka hoʻoikaika mau i ka luhi i kekahi mau hihia.

Kū pinepine nā ʻōuli e pili ana i nā ʻāpana ʻē aʻe o ka ʻōnaehana hopohopo, a hoʻopili pū me:

• Hoʻololi ke kahe o ke koko
• Ka paupauaho i ke kū ʻana
• Waha maloo
• ʻO ka hana Erectile
• Nā maka maloʻo
• Konipōpō
• Hoʻoiho ka sweating

E hana ka mea mālama ola i kahi hoʻokolohua kino a nīnau e pili ana i nā ʻōuli. Hōʻike paha ka hōʻike:

• Hoʻoemi i nā reflexes
• Hiki ke lilo i kaʻiʻo muscle
• Ka nāwaliwali a i ʻole ka palapū e ʻoi aku ka maikaʻi me ka hana

Nā ho'āʻo e kōkua diagnose a hōʻoia LES e komo pū paha:

• Nānā ke koko e nānā i nā lāʻau ʻino e hoʻouka i nā aʻalolo
• Electromyography (EMG) e hoʻāʻo i ke olakino o nā mākala nāʻiʻo
• ʻO ka wikiwiki conduction nerve (NCV) e hoʻāʻo i ka wikiwiki o ka hana uila ma nā ʻalolo

CT scan a me MRI o ka umauma a me ka ʻōpū, ukali ʻia e bronchoscopy no nā mea puhipaka e hana ʻia e kāpae ʻia i ka maʻi ʻaʻai. Hiki ke hana pū ʻia me ka scan PET inā kuhi ʻia kahi tumo māmā.

ʻO nā pahuhopu nui o ka mālama ʻana i:

• E ʻike a mālama i nā maʻi maʻi pū, e like me ka maʻi ʻaʻa maʻi
• E hāʻawi i ka lāʻau e kōkua i ka nāwaliwali

ʻO ka pānaʻi Plasma, a i ʻole plasmapheresis, kahi lapaʻau e kōkua i ka lawe ʻana mai ke kino i nā proteins (antibodies) ʻino e keʻakeʻa nei i ka hana nerve. Hoʻopili kēia i ka hoʻoneʻe ʻana i ke koko koko i loaʻa ka maʻi ʻino. Hoʻokomo ʻia nā protein ʻē aʻe (e like me albumin) a i hāʻawi ʻia i plasma i loko o ke kino.

ʻO kekahi hana e pili ana i ka hoʻohana ʻana i ka immunoglobulin intravenous (IVIg) e hoʻokomo i kahi nui o nā mea kōkua kōkua pono i loko o ke kahe o ke koko.

ʻO nā lāʻau e hoʻāʻo ʻia paha e hoʻopili ʻia:

• Nā lāʻau e kāohi i ka pane o ka ʻōnaehana pale
• ʻO nā lāʻau Anticholinesterase e hoʻomaikaʻi ai i ke kani o ka muso (ʻoiai ʻaʻole maikaʻi loa kēia mau mea ke hāʻawi wale ʻia)
• Nā lāʻau e hoʻonui ai i ka hoʻokuʻu ʻia o acetylcholine mai nā pūnana nerve

Hiki ke hoʻomaikaʻi ʻia nā ʻōuli o ka LES ma ka mālama ʻana i ka maʻi ma lalo, ke kaomi ʻana i ka ʻōnaehana pale, a i ʻole ka hemo ʻana i nā mea ʻino. Eia nō naʻe, ʻaʻole pane paha ʻo Larane paraneoplastic i ka lāʻau. (Nā Paraneoplastic LES hōʻailona ma muli o ka hoʻololi ʻana o ka ʻōnaehana pale pale i kahi tumo). ʻO ka make ma muli o ke kumu hōʻino.

Komo pū nā pilikia o LES:

• Ka hanu paʻakikī ʻana, me ke kō ʻole o ka hanu ʻana (ʻoi aku ka maʻamau)
• Pilikia i ka ale ʻana
• Nā maʻi ʻino, e like me ka niumonia
• Nā ʻeha mai ka hāʻule a me nā pilikia me ka hoʻohui ʻana

Kāhea i kāu mea hāʻawi inā ulu nā hōʻailona o LES.

Myasthenic maʻi maʻi; Maʻi maʻi Eaton-Lambert; ʻO Lambert-Eaton myasthenic maʻi; LEMS; LES

• Nā mākala mua ma mua

ʻO Evoli A, ʻo Vincent A. Nā maʻi o ka lawe neuromuscular. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 394.

Moss HE. Nā pilikia ʻili maka a me nā helehelena. I: Liu GT, Volpe NJ, Galetta SL, eds. ʻO Liu, Volpe, a me Galetta's Neuro-Ophthalmology. ʻEkolu huaʻōlelo. Piladelapia, PA: Elsevier; 2019: chap 14.

Sanders DB, Guptill JT. Nā maʻi o ka hoʻoili neuromuscular. I: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. ʻO Bradley's Neurology i ka hana lapaʻau. ʻEpekema 7 Piladelapia, PA: Elsevier; 2016: chap 109.