E aʻo kākou e pili ana i nā maʻi Neuromuscular ma ke ʻano maʻalahi.

Ke manaʻo nei ʻoe i kekahi manawa ʻaʻohe ou ikaika, paʻakikī ke hāpai i ka lima a i ʻole ka wāwae, a i ʻole e mae kou mau lālā i kou hele ʻana, a nāwaliwali mālie kou mau ʻiʻo? ʻAʻole kēia he mau mea wale nō. Malia paha ma hope o kēia mau hōʻailona ke waiho nei nā maʻi aʻalolo a me nā ʻiʻo a mākou e kamaʻilio nei i kēia lā, ʻo ia hoʻi, kahi kūlana i kapa ʻia ʻo "Neuromuscular Disorders". Mai hopohopo, ʻaʻohe mea e makaʻu ai ma hope o ka lohe ʻana i kēia. E kamaʻilio kākou e pili ana i nā mea āpau me ka maopopo a me ka maʻalahi.

He aha nā maʻi Neuromuscular?

I ka ʻōlelo maʻalahi, he hui kēia o nā maʻi e hoʻopilikia i nā ʻāpana o kā mākou ʻōnaehana nerve, ʻoiai nā aʻalolo peripheral ( nā aʻalolo e puka ana mai ka lolo a me ke kaula spinal), nā ʻiʻo, a i ʻole ke kamaʻilio ʻana ma waena o nā mea ʻelua. E noʻonoʻo e pili ana i ia mea, hana ʻia ko mākou neʻe ʻana o ke kino i ka wā e hele ai nā leka mai ka lolo ma o nā aʻalolo e hoʻāla i nā ʻiʻo. Inā he hewa ma kekahi wahi o kēlā kaʻina hana, hiki ke loaʻa kēia mau maʻi.

ʻO kēia mau kūlana ka mea maʻamau e hoʻonāwaliwali ai i nā ʻiʻo , ka nawaliwali a i ʻole ka nawaliwali ʻana o nā ʻiʻo (muscle atrophy) , a me nā loli i ka manaʻo (e like me ka maneʻo a i ʻole ke kani ʻana). Eia nō naʻe, hiki ke ʻike ʻia nā hōʻailona ʻē aʻe, ma muli o ke ʻano o ka maʻi.

He aha kēia mau maʻi? Pehea lākou e hoʻopilikia ai i ko kākou kino?

ʻAʻole he maʻi hoʻokahi nā maʻi neuromuscular. He hui lākou o nā maʻi a loaʻa i kēlā me kēia hui nā ʻano maʻi like ʻole. Hiki iā lākou ke hoʻopilikia i nā ʻāpana aʻe o kā mākou ʻōnaehana neuromuscular:

• Nā pūnaewele pepeiaohao mua o ke kaulahao kuamoʻo
• Nā aʻa aʻalolo (e komo pū ana me ka ganglia aʻa dorsal)
• Nā pūnaewele aʻalolo (Plexuses) (e.g. (brachial plexus) - pili i ka poʻohiwi, (lumbosacral plexus) - pili i ka ʻāʻī)
• Nā aʻalolo peripheral
• Hoʻohui neuromuscular
• Nā olonā ʻiʻo

I kēia manawa, e nānā kākou i nā maʻi e hoʻopilikia ana i kēlā me kēia ʻāpana me nā kikoʻī hou aku.

Nā maʻi e pili ana i nā pūnaewele nerve motor (nā pūnaewele pepeiaohao mua) o ke kaula spinal

He mau maʻi holomua kēia e hoʻopau ai i nā neurons motor . ʻO nā neurons motor kahi ʻano cell i loko o ko mākou lolo a me ke kaula spinal e kōkua iā mākou e neʻe, e like me ka hele wāwae, ke kamaʻilio ʻana, a me ka hanu ʻana. Lawe kēia mau cell i nā leka mai ka lolo a i nā ʻiʻo.

ʻO nā "pūnaewele pepeiaohao mua" he mau pūnaewele aʻalolo hoʻokele i loko o ka mea hina o ke kaula kuamoʻo. He mea nui lākou no ka neʻe ʻana o ko kākou mau ʻiʻo iwi. Ke luku ʻia kēia mau pūnaewele, nalowale nā ​​ʻiʻo i ka lako aʻalolo (denervation) a lilo i nāwaliwali.

ʻO nā maʻi nui e pili ana i kēia mahele:

• ʻO ka maʻi ʻo Lou Gehrig a i ʻole ALS (Amyotrophic Lateral Sclerosis - ALS)
• ʻO ka Atrophy Muscular Spinal (SMA)
• ʻO ka atrophy muscular spinal-bulbar (SBMA) a i ʻole ka maʻi o Kennedy
• Polio

Nā pilikia e pili ana i ka Plexus (Plexopathies)

ʻO nā aʻalolo e puka mai ana mai ko kākou iwi kuamoʻo ma nā pae like ʻole e hui pū i ka hopena e hana i kahi pūnaewele paʻakikī, a i ʻole plexus . Loaʻa iā kākou ka brachial plexus ma ko kākou mau lima luna, a me ka lumbar plexus a i ʻole lumbosacral plexus ma ko kākou mau lima lalo, aia ma ko kākou ʻāʻī.

Hiki i kēia mau olonā aʻalolo ke hōʻino ʻia, hoʻopaʻa ʻia, a mumū paha, hiki ke hōʻeha, ʻeha, ʻūlū, a nāwaliwali i ka lima a i ʻole ka wāwae i hoʻopilikia ʻia.

Nā maʻi e pili ana i ke aʻa nerve

He mau lālā pōkole nā ​​aʻalolo o ke aʻalolo kuamoʻo. Puka mai nā aʻalolo kuamoʻo mai nā vertebrae (iwi) ma ko kākou kaula kuamoʻo. Loaʻa i kēlā me kēia aʻalolo kuamoʻo nā aʻalolo motor a me nā aʻalolo sensory. ʻO nā aʻalolo motor kahi e hele ai nā leka mai ka lolo a me ke kaula kuamoʻo i nā ʻiʻo ma o nā aʻalolo peripheral.

Ke kaomi ʻia kēia mau aʻa aʻalolo e nā ʻiʻo a puni, hiki ke loaʻa ka ʻeha a me ka maneo ma nā ʻāpana like ʻole o ke kino. Ua kapa ʻia kēia ʻo 'radiculopathy' . Ke kaomi ʻia nā aʻa aʻalolo motor, nāwaliwali nā ʻiʻo.

Aia nā ʻano radiculopathy ma muli o kahi i hoʻopaʻa ʻia ai ke aʻalolo spinal:

• ʻO ka radiculopathy cervical
• Mai waena a i luna o ka umauma ((thoracic radiculopathy))
• ʻO ka radiculopathy lumbar a i ʻole lumbosacral (e pili ana i ka ʻāʻī a me ke kua i lalo)

Nā maʻi e pili ana i nā aʻalolo peripheral

ʻO ka neuropathy peripheral kahi huaʻōlelo laulā no kekahi maʻi e hoʻopilikia ana i nā aʻalolo ma waho o ka lolo a me ke kaula spinal (nā aʻalolo peripheral). Nui nā ʻano a me nā kumu o kēia. Inā ʻaʻole hana pono nā aʻalolo peripheral, ʻaʻole hiki iā mākou ke hoʻoneʻe i ko mākou mau ʻiʻo. ʻO ia ke kumu e pili ai kēia mau maʻi i ka hui nui o nā maʻi neuromuscular.

Hiki i nā neuropathies peripheral ke hoʻopilikia i kekahi mau ʻāpana o ke kino, a i ʻole hiki iā lākou ke hoʻopilikia i ke kino holoʻokoʻa. ʻO ke ʻano maʻamau o ka neuropathy peripheral ʻo ia ka mea e hoʻopilikia ai i nā lima a me nā wāwae, ʻoi aku hoʻi nā manamana lima. No ka laʻana, loaʻa i kekahi poʻe me ka maʻi diabetes ka maneʻo ma nā kapuwaʻi o ko lākou mau wāwae, me he mea lā ʻaʻole hiki iā lākou ke hoʻopā i ka lepo. He laʻana kēlā o kēia.

Eia kekahi mau laʻana:

• Ka pōʻino o ke aʻalolo i hoʻokumu ʻia e ka maʻi diabetes mellitus
• Ma muli o ka hoʻohana nui ʻana i ka waiʻona (Alcohol use disorder)
• Ka ʻai ʻole ʻana
• He kūlana i kapa ʻia ʻo 'Amyloidosis'
• Nā kumu hoʻoilina a i ʻole nā ​​​​kumu hoʻoilina (e like me ka maʻi Charcot-Marie-Tooth)
• Nā kumu autoimmune a i ʻole nā ​​​​kumu mumū (e.g., Chronic Inflammatory Demyelinating Polyneuropathy - CIDP, Guillain-Barré syndrome)
• Ka ʻike ʻia ʻana i nā mea ʻawahia (e like me nā kemika ʻawahia e like me nā wai hoʻomaʻemaʻe, nā pesticides)

Nā maʻi hui neuromuscular

ʻO ka neuromuscular junction (NMJ) ka pilina (synapse) ma waena o ke kikowaena o kahi aʻalolo motor a me kahi ʻiʻo. No ke kamaʻilio ʻana me ka ʻiʻo, hoʻokuʻu nā aʻalolo motor i kahi kemika i kapa ʻia ʻo acetylcholine ma o kēia junction. Inā he hemahema i kēia kamaʻilio ʻana, loaʻa nā maʻi e pili ana i ka neuromuscular junction.

ʻO nā maʻi nui e pili ana i kēia hui:

• ʻO ka 'Botulism' (kahi maʻi e hiki ke kū mai ma muli o ka lāʻau make meaʻai)
• ʻO ka maʻi myasthenic Lambert-Eaton (LEMS)
• ʻO ka Myasthenia Gravis

Nā maʻi ʻiʻo (Myopathies)

ʻO nā myopathies nā maʻi e hoʻopilikia pololei i ko kākou mau ʻiʻo iwi (nā ʻiʻo e pili ana i ko kākou mau iwi). Hōʻino kēia mau maʻi i nā olonā ʻiʻo a hoʻonāwaliwali i nā ʻiʻo. Nui nā ʻano myopathies. He congenital kekahi (genetic a i ʻole hereditary). Hiki i kekahi ke ulu ma hope o ke ola.

Eia nō naʻe, ʻaʻole i hāʻule nā ​​​​​​ʻeha pololei e like me ka haehae ʻana, ka huki ʻana, a i ʻole ka wili ʻana o kahi ʻiʻo i loko o kēia māhele o "myopathies".

ʻO ka Dystrophy Muscular

ʻO ka muscular dystrophy he hui o nā maʻi he kanakolu a ʻoi e hoʻopilikia i ka hana o nā ʻiʻo ma muli o nā kumu genetic (hoʻoilina). ʻO kekahi o nā ʻano nui:

• ʻO Duchenne muscular dystrophy (DMD) - ʻO kēia ke ʻano maʻamau.
• ʻO ka maʻi ʻiʻo ʻeha ʻo Becker (BMD)
• ʻO ka maʻi myotonic dystrophy
• ʻO ka maʻi ʻiʻo oculopharyngeal (OPMD)

Nā Myopathies Autoimmune a me Inflammatory

He mau maʻi kēia e hoʻopilikia ai i ka hana o nā ʻiʻo ma muli o nā hemahema i loko o kā mākou ʻōnaehana pale ponoʻī. Nā Laʻana:

• ʻO ka maʻi ʻili
• ʻO ka Polymyositis
• ʻO ka myopathy necrotizing i hoʻopili ʻia e ka pale ʻana

Nā Myopathies ʻē aʻe

Hiki ke loaʻa nā maʻi ʻeha no nā kumu like ʻole:

• Nā kaulike ʻole o ke electrolyte : No ka laʻana, hiki i ke kiʻekiʻe o ka calcium koko (hypercalcemia) a i ʻole ke kiʻekiʻe o ka potassium koko (hyperkalemia) ke hana i nā pilikia ʻiʻo.
• Nā myopathies endocrine : Nā kaulike ʻole o ka hormone e hoʻopilikia ai i ka hana o nā ʻiʻo. Eia kekahi laʻana,Hiki ke hana ʻia kēia mau mea e nā pilikia me ka ʻōpū thyroid, nā ʻōpū parathyroid , a i ʻole nā ​​​​ʻōpū adrenal .
• Nā myopathies metabolic : Hoʻokumu ʻia kēia e nā hemahema i loko o nā genes e kuhikuhi ana i nā ʻiʻo e hana i nā enzymes. Nā laʻana: nā maʻi mālama glycogen, nā maʻi mālama lipid.
• Nā myopathies Mitochondrial : Hoʻokumu ʻia kēia e nā hemahema i ka mitochondria, nā ʻāpana hana ikehu o nā cell.
• ʻO ka maʻi lolo manawa : He hui kēia o nā maʻi neurological hoʻoilina. I loko o kēia, lilo nā ʻiʻo i nāwaliwali a hiki ʻole ke ʻaelike i kekahi manawa.
• ʻO nā myopathies ʻawahia: Hoʻokumu ʻia kēia e kahi toxin a lāʻau lapaʻau paha e hoʻopilikia i ke ʻano a i ʻole ka hana o nā ʻiʻo.

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He aha nā maʻi aʻalolo a me nā ʻiʻo i maʻamau?

ʻO ka ʻoiaʻiʻo, ʻo ka neuropathy peripheral ka mea maʻamau o kēia mau kūlana. No ka mea he nui nā ʻano a me nā kumu like ʻole. Manaʻo nā kānaka noiʻi he ʻoi aku ma mua o 20 miliona mau kānaka ma ʻAmelika Hui Pū ʻIa i loaʻa i kekahi ʻano o ka neuropathy peripheral.

Akā, ʻaʻole manaʻo ka nui o ka poʻe i nā (peripheral neuropathies) ma ke ʻano he (neuromuscular disorders), no ka mea, he ʻano ʻokoʻa ko lākou. Eia nō naʻe, ʻo nā (neuromuscular diseases) a mākou i lohe ai, akā he mea kakaikahi maoli, ʻo ia ka (ALS) a me ka Muscular Dystrophy .

He aha nā hōʻailona maʻamau o kēia mau maʻi?

Hiki ke ʻokoʻa nā hōʻailona o nā maʻi neuromuscular, ma muli o ke ʻano kikoʻī o ka maʻi. Eia nō naʻe, ʻo nā hōʻailona maʻamau e pili ana i nā ʻiʻo:

• Ka nāwaliwali o nā ʻiʻo (ka paʻakikī i ka hāpai ʻana i ka lima a i ʻole ka wāwae, hiki ʻole ke hāpai i nā mea kaumaha)
• ʻO ka atrophy o nā ʻiʻo ( ka nawaliwali ʻana o nā ʻiʻo a me ka mae ʻana)
• ʻO ka ʻūlū ʻana o nā ʻiʻo (fasciculations), nā ʻūpā, a i ʻole nā ​​spasms
• ʻEha ʻiʻo
• ʻOʻoleʻa o nā ʻiʻo (Spasticity)
• Paʻakikī ka hele wāwae
• Nā pilikia kaulike a me ka hoʻonohonoho ʻana
• Nā maka maka e hāʻule ana a i ʻole nā ​​​​​​ʻāpana ʻē aʻe o ka maka
• Ka paʻakikī i ka ʻōlelo ʻana (dysarthria) ma muli o ka nāwaliwali o nā ʻiʻo o ke alelo a i ʻole ka waha/maka
• Ka paʻakikī i ka moni ʻana (dysphagia) ma muli o ka nāwaliwali o nā ʻiʻo pharynx
• Ka paʻakikī o ka hanu ʻana ma muli o ka nāwaliwali o ka ʻiʻo diaphragm

ʻO nā hōʻailona ʻē aʻe paha:

• Luhi
• Māʻule
• Nā manaʻo ʻē e like me ka hele wāwae ʻana o nā ʻanuʻu a i ʻole nā ​​​​pine a me nā nila (paresthesia)

Mea nui: Inā loaʻa iā ʻoe kekahi a ʻoi aku paha o kēia mau hōʻailona ma ka lālani, ʻoiai inā hiki koke mai ka nāwaliwali o nā ʻiʻo a lohi paha, e ʻike pono i ke kauka.

No ke aha e loaʻa ai kēia mau maʻi? He aha nā kumu?

Nui nā kumu o nā maʻi neuromuscular. Eia nō naʻe, he genetic kekahi. ʻO ke ʻano kēia hiki ke hana ʻia e nā genes i hoʻoilina ʻia mai nā mākua a i ʻole e kahi loli maʻamau i kahi gene (mutation). Hiki ke hana ʻia kēia mau maʻi e kahi kīnā i loko o hoʻokahi gene a i ʻole e nā hemahema i loko o kekahi mau genes.

ʻO kekahi mau maʻi neuromuscular he mau maʻi autoimmune . ʻO ke ʻano kēia, hoʻouka kā mākou ʻōnaehana pale ponoʻī i kā mākou mau cell ponoʻī. Hiki ke hana ʻia kekahi e kahi ulia, kahi hemahema meaʻai, kahi maʻi metabolic, ka ʻike ʻana i kahi toxin, a i ʻole ka mumū. I kekahi manawa, ʻaʻole hiki ke loaʻa ke kumu.

Pehea e ʻike ai ke kauka i kēia mau maʻi?

Ke ʻike ʻoe i ke kauka, e nīnau ʻo ia iā ʻoe e pili ana i kou mōʻaukala olakino, inā paha ua loaʻa i kekahi o kou ʻohana kēia maʻi, nā lāʻau lapaʻau āu e lawe ai, a me nā hōʻailona āu e loaʻa ai. A laila, e hana lākou i kahi hoʻokolohua kino maʻamau a me kahi hoʻokolohua neurological . Hiki i kēia hoʻokolohua ke nānā i nā pilikia me ka lolo, ke kaula spinal, a me ka ʻōnaehana nerve peripheral (me nā ʻiʻo).

Hiki ke kuhikuhi ʻia ʻoe i kahi neurologist no ka hoʻāʻo hou aku. ʻO kekahi mau hoʻokolohua ʻē aʻe e hiki ke kōkua i ka hōʻoia ʻana i kēia mau kūlana:

• Electromyography (EMG) : Hoʻāʻo kēia i ke olakino a me ka hana o kou mau ʻiʻo iwi a me nā aʻalolo e kaohi ana iā lākou.
• Hoʻāʻo hoʻoili aʻalolo : Hoʻāʻo kēia i ka hana o kou mau aʻalolo peripheral.
• Nā hoʻāʻo koko : Hiki i kēia ke nānā i nā mea like ʻole, e like me nā hemahema o ka enzyme a me nā hōʻailona o nā kūlana autoimmune.
• Nā hoʻāʻo kiʻi : Hiki i nā hoʻāʻo e like me MRI , CT scan , a me ka ultrasound neuromuscular ke "ʻike" inā he mau pilikia me ka lolo, ke kaula spinal, a me/a i ʻole nā ​​​​aʻalolo.
• ʻO ka biopsy ʻiʻo: Ma kēia hoʻokolohua , lawe ʻia kahi hāpana liʻiliʻi o kou ʻiʻo a nānā kekahi pathologist iā ia ma lalo o kahi microscope e ʻike inā he mau mea ʻē.
• Ka hoʻāʻo ʻana i ka genetics : Kōkua kēia mau hoʻokolohua e hōʻoia i kekahi mau maʻi neuromuscular genetic.

He aha nā lāʻau lapaʻau no kēia mau maʻi?

ʻOkoʻa loa nā lāʻau lapaʻau no nā maʻi neuromuscular ma muli o ke ʻano kikoʻī o ka maʻi. Ma keʻano laulā, ʻaʻole hiki ke ho'ōla loa ʻia ka hapa nui o nā maʻi neuromuscular. Eia nō naʻe, aia nā lāʻau lapaʻau a me nā therapies e hiki ke kōkua i ka hōʻemi ʻana i nā hōʻailona, ​​​​​​e hoʻōki i ka holomua o ka maʻi, a i ʻole e hoʻolohi i kona holomua. Hiki i kāu hoʻolālā lapaʻau ke komo pū me:

• Nā lāʻau lapaʻau
• Hoʻōla kino a me ka hoʻōla hana
• Hoʻōla ʻōlelo
• Ka lāʻau lapaʻau meaʻai
• Nā mea kōkua e kōkua ai i ka neʻe ʻana
• Nā kaʻina hana lapaʻau ʻē aʻe, me nā ʻoki kino

I ka manawa pinepine, ʻo kahi neurologist (ʻoi aku ka loea neuromuscular) kāu kauka mālama mua no kēia mau kūlana. Eia nō naʻe, ma muli o ke ʻano o ke kūlana, pono paha ʻoe i ke kōkua mai nā loea ʻē aʻe a me nā mea lawelawe mālama ola. Hiki i kēia mau mea ke komo pū me:

• Nā mea hoʻoponopono kino a me nā mea hoʻoponopono hana
• Nā Kauka Lapaʻau ʻŪhā
• Nā kauka pulmonologists a me nā mea lapaʻau hanu
• Nā kauka maʻi
• Nā kauka lapaʻau
• Nā kauka orthopedist
• Nā kauka ʻōlelo
• Nā mea lapaʻau meaʻai
• Nā kānaka noʻonoʻo
• Nā limahana kaiaulu

He aha kāu e manaʻo ai i ka wā e noho ai me kēia ʻano maʻi?

He lōʻihi (mau) ka nui o nā maʻi aʻalolo a me nā ʻiʻo. ʻO ia hoʻi, hiki iā lākou ke ola a pau. ʻOiai ʻaʻohe lāʻau lapaʻau, hiki i nā lāʻau lapaʻau a me nā lāʻau lapaʻau ʻē aʻe ke kōkua i ka kaohi ʻana i nā hōʻailona, ​​​​​​e hoʻōki i ka holomua o ka maʻi, a i ʻole e hoʻolohi iā ia. Hiki i kāu kauka ke hāʻawi iā ʻoe i kahi manaʻo maikaʻi aʻe o ka mea e manaʻo ai, ma muli o kou kūlana kikoʻī.

ʻO nā maʻi neuromuscular kahi ʻano ākea o nā kūlana e hoʻopilikia nui i ka hana o nā aʻalolo peripheral a me nā ʻiʻo. Inā loaʻa iā ʻoe ka nāwaliwali koke o nā ʻiʻo a i ʻole ka wehewehe ʻole ʻia, e kamaʻilio me ke kauka. Hiki iā ia ke loiloi i kāu mau hōʻailona a ʻōlelo i nā hoʻokolohua e hoʻoholo ai i ke kumu.

ʻO ka mea hope loa, ʻo ka mea nui loa (Leka Lawe-Home)

Mai makaʻu i ka lohe ʻana i ka inoa ʻo Neuromuscular Disorders. Hiki ke hoʻomalu ʻia ka nui o kēia mau kūlana me ka ʻike mua ʻana, ka mālama kūpono, a me ka hoʻokele ʻana. ʻO ka mea nui, inā loaʻa iā ʻoe nā hōʻailona ʻē (ʻoi aku ka nāwaliwali o nā ʻiʻo, ka mae ʻana o ka ʻiʻo, ka paʻakikī o ka hele wāwae),Mai hoʻowahāwahā iā lākou a e ʻimi koke i ke kōkua lapaʻau.

E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. Aia nā kauka akamai, nā mea hoʻōla, a me nā hui kākoʻo e hiki ke kōkua iā ʻoe e hoʻoponopono i kēia mau kūlana. ʻO ka loaʻa ʻana o ka ʻike kūpono a me ka hana mua ʻana ke ala maikaʻi loa i ke ola olakino.

Nā maʻi neurological, nā maʻi ʻiʻo, nā maʻi Neuromuscular, ALS, Muscular Dystrophy, Peripheral Neuropathy, Myasthenia Gravis