I ētahi wā, ka pēhia tō ringa, tō waewae, tō uaua rānei o te tinana, ā, kāore e taea te whakangā wawe pērā i tō whakaaro? Pērā i te wā e rūrū ringa ana koe ki tētahi, ā, ka uaua ki te kukume atu i tō ringa, i te wā rānei e ngana ana koe ki te whakatika i te tūru, ka roa te wā. He tohu noa iho tēnei o tētahi mate e kiia nei ko te myotonia. Kaua e manukanuka, ka kōrerohia e mātou tēnei i roto i ngā taipitopito.

He aha te Myotonia?

Ki te kī māmā noa iho, ko te myotonia te wā kāore ō uaua e tere whakangāwari i muri i te whakawhāiti. Whakaarohia kei te rūrū ringa koe ki tētahi, engari he uaua ki te kukume atu i tō ringa, i a koe rānei e noho ana, e ngana ana ki te whakatika, ka mārō ō waewae mō tētahi wā. Koinei te momo mea.

He rerekē ngā momo myotonia. Ko ia momo ka puta mai i tētahi huringa ira . Ko te tikanga tēnei ka puta mai i tētahi pānga ki ngā ira o roto i tō tātou tinana. Mō ia momo, he rerekē te ira e pāngia ana.

Nā ēnei huringa ira, ka pā pea te myotonia ki ētahi atu wāhanga o tō tinana. I runga i te momo myotonia kei a koe, ka pā pea tēnei ki ō:

• Ki te ngākau
• Ki ngā pukahukahu
• Ki te pūnaha nakunaku
• Ki te roro
• Mō ngā kanohi

Ka pā pea ki ngā okana pēnei i te.

He mea noa te mataku ina pāngia koe e tētahi tohu kāore anō kia pā ki a koe i mua, pērā i te pakari o ngā uaua. Tērā pea kei te whakaaro koe, "Ka kino haere tēnei?" "He aha te pānga o tēnei ki tōku ao?" Ko te mea pai rawa atu ka taea e koe ko te toro atu ki tētahi tākuta . He mate onge te myotonia, nō reira, tera pea ka roa te wā kia kitea he mate tika. Engari i tēnei wā, ka taea e tō tākuta te āwhina i a koe ki te whakamama i ō mataku me te whakautu i ō pātai.

He aha ngā momo matua o te myotonia?

E whakarōpūtia ana e ngā tākuta te myotonia kia rua ngā momo matua: te dystrophic me te non-dystrophic . Ka pāngia e ngā momo e rua ngā tohu hiko e whakahaere ana i te whakahekenga o te uaua. Heoi, i roto i te myotonia dystrophic, ka pakaru te uaua tonu. Ko te tikanga o te kupu 'dystrophy' ko 'te ngoikore haere, te memeha haere rānei o te kiko'.

Myotonia Dystrophic (DM)

Ko te Dystrophic Myotonia (DM) he āhuatanga e ngoikore ai, e mimiti ai hoki ngā uaua (atrophy) i te roanga o te wā, ā, nō roto i tētahi rōpū mate e kiia nei ko te muscular dystrophy. E rua ngā momo matua:

• Te mate pukupuku miotonika momo 1 (DM1) – Koinei te momo tino noa.
• Te Matepukupuku Myotonic momo 2 (DM2)

Myotonia kore-pōrearea

I roto i te myotonia kore-dystrophic, kāore te hanganga o te kiko uaua e pakaru. Ko ētahi atu momo e taka ana ki tēnei kāwai ko:

• Te mate Andersen-Tawil
• Te pararutiki ā-wā hyperkalemic
• Te pararutiki ā-wā hypokalemic momo 1 me te momo 2
• Myotonia congenita – Koinei te momo tino noa o tēnei rōpū.
• Paramyotonia congenita
• Te maiotonia o te hongere konutai

He aha ngā tohu o te Myotonia?

Ko te tohu matua o te myotonia ko te kore e taea te whakangāwari i te uaua ina kua piko. Ka puta pea ēnei tohu i te wā pēpi, i te tamarikitanga, i te pakeketanga rānei, i runga i te momo myotonia.

Ko ētahi atu tohu noa ka kitea ko:

• He panonitanga ki te āhua o ō uaua – hei tauira, te whakanui uaua (hypertrophy) te memeha/ngoikore rānei o te uaua (hypotonia) .
• Te ngenge .
• Te mamae o ngā uaua .
• Te ngoikore o te uaua .
• Te pēhanga o te uaua, engari ka iti haere te pēhanga ina nekehia, ina whakakorikori tinana rānei (te āhua whakamahana tinana) .

Ngā tohu motuhake o te myotonia dystrophic

He tohu motuhake kei ia momo myotonia dystrophic. Ko ētahi tauira ko:

• Mamae puku, mate korere, mate korere, mate waikawa, pupuhi.
• Ngā rerekētanga o te āhua o te kanohi (ngā rerekētanga o te āhua o te kanohi) .
• Te katarata (te pōuri o te tirohanga) .
• Ko te hyperglycemia he mate e hua mai ana i te ātete ki te insulin, e hua ai te taumata huka toto tiketike.
• Hauātanga hinengaro .
• Te pā o te manawa kāore i te tika (arrhythmia) me ngā raruraru rānei ki ngā tohu hiko o te manawa.
• Te ngaro o te makawe i te taha mua o te mahunga i roto i ngā tāne (androgenic alopecia) .
• Te mate moe pōrearea (te mate moe pōrearea) .

He aha ngā tohu o te hunga e pāngia ana e te Periodic Paralysis?

He pararutiki ā-wā hyperkalemic tōu.Ki te pāngia koe e tēnei mate, tera pea ka pāngia koe e te āhua rite ki te pararutiki, ka ngaro te kaha o ō uaua mō te kotahi ki te whā hāora.

Mena he pararutiki ā-wā tōu , ka pāngia pea koe e te ngoikore o te uaua i te pō, i te atatu rānei. Ka roa pea tēnei mō ētahi haora ki ētahi rā.

He aha te take o te myotonia?

Ko te myotonia te take o tētahi huringa motuhake i roto i tētahi o ō ira . Hei whakamārama ake, ka pā ēnei huringa ira ki ngā hongere katote e whakahaere ana i te mahi a ō uaua.

Ko ia momo myotonia ka puta mai i tētahi whakarerekētanga ira rerekē. Hei tauira, ko te myotonic dystrophy momo 1 ka puta mai i tētahi raruraru ki te ira DMPK . Ko te myotonia congenita ka puta mai i tētahi raruraru ki te ira CLCN1 . Ka taea e koe te tuku iho i tēnei whakarerekētanga ira mai i tētahi, i ō mātua tokorua rānei. Kāore rānei, ka taea te whanake matapōkere.

Me pēhea koe e mōhio ai mēnā he myotonia tōu?

I te tīmatanga o te tukanga tātaritanga, ka pātai atu tō tākuta ki a koe mō ō tohu me tō hītori hauora whānau . Kātahi, i mua i te whakaaro i ngā whakamātautau mō te myotonia, ka whakakorea e rātou ētahi atu āhuatanga noa e puta ai ō tohu, pērā i te hypothyroidism .

Koinei ngā whakamātautau e whakamahia ana hei tautuhi i te myotonia:

• Whakamātautau Toto Creatine Kinase (CK): Ko te Creatine kinase he whākōkī e kitea nuitia ana i roto i ngā uaua. I te nuinga o te wā ka piki ake ngā taumata CK i roto i te myotonia.
• Ine hiko (EMG): Ka aromatawaihia e tēnei whakamātautau te hauora me te mahi a ō uaua angaiwi me ngā io e whakahaere ana i aua uaua.
• Ngā whakamātautau ira: Ka taea e ēnei whakamātautau te tautuhi i ngā huringa ira e puta ai ngā momo myotonia rerekē.
• Whakamātautau toto pāhare pākawa: Ka whakamahia pea tēnei whakamātautau e tō tākuta hei tirotiro mō te hyperkalemic periodic paralysis (he nui te pāhare pākawa i roto i te toto) te hypokalemic periodic paralysis rānei (he iti te pāhare pākawa i roto i te toto).
• Whakamātautau whakakori tinana poto: I roto i tēnei whakamātautau, me whakakorikori ō uaua i ngā wā kua whakaritea i te wā e mātakitaki ana te tākuta i te urupare a ō io me ō uaua.

He mate onge te Myotonia,Tērā pea ka roa te wā kia kitea ai he mate tika. Kaua e manukanuka ki tēnā.

He aha ngā maimoatanga mō te myotonia?

Ko te maimoatanga mō te myotonia he mea motuhake ki tō momo me ngā tohumate. Ka mahi tahi kōrua ko tō tākuta ki te whakawhanake i tētahi mahere maimoatanga e hāngai ana ki ō hiahia. Kaua e mangere ki te pātai i ō pātai.

Ko te whakaora tinana he wāhanga o tō mahere maimoatanga. Ka taea e te kaiwhakaora tinana te āwhina i a koe ki te karo i ngā mea e mōhiotia ana, e taea ana rānei te whakaoho, me te whakawhanake i tētahi mahere whakakori tinana hei āwhina i a koe kia noho pukumahi tonu. Ka taea hoki e ngā kaiwhakaora mahi te āwhina i a koe ki te kimi huarahi hou hei mahi i ngā mahi o ia rā. Ka tūtohu pea rātou i ngā āwhina urutau, āwhina nekeneke rānei.

Te Maimoatanga o te Myotonia Dystrophic

Ka rongoatia te Dystrophic Myotonia mā te whakamahi i ngā tohumate. Me tirotiro tonu koe i te hauora o ngā whekau e pāngia ana e tō momo Dystrophic Myotonia.

Ko te rongoā e tohua ana e ngā tākuta ko te mexiletine . He rongoā ārai-arrhythmic tēnei, engari he āwhina anō hoki ki te wiri me te pakari o ngā uaua. Tērā pea ka āwhina anō hoki te Mexiletine i ētahi momo myotonia kore-dystrophic.

Te Maimoatanga Myotonia Kore-dystrophic

Haunga ngā rongoā, ko te maimoatanga mō te myotonia kore-dystrophic ko te karo i ētahi mea e whakaoho ana. Kei roto i ēnei ko:

• Te karo i te pāngia e te makariri.
• Te whakawhāiti i ngā mahi whakakori tinana uaua, taumaha.

Te Maimoatanga Pararutiki Ā-Wā

Ko te maimoatanga mō te pararutiki ā-wā ko te whakahaere i ngā taumata pāhare pāporo me te karo i ngā mea e whakaoho ana. Tērā pea ka tūtohu tō tākuta kia karohia e koe ēnei e whai ake nei:

• Te okioki mō te wā roa i muri i te whakakori tinana.
• Te kai i ngā kai he nui te warowaihā.
• Te kai i ngā wā koretake.
• Te moe koretake.
• Te mahi i te pō.

Mena he hypokalemic periodic paralysis tōu, ka tūtohu pea tō tākuta kia kai koe i ngā kai whai rawa i te pāhare pāporo, pērā i te panana, te pīni, te hua maroke, te māngo, me te papaya.

Mena he hyperkalemic periodic paralysis tōu, me karo pea koe i ngā kai he nui te pāhare pāporo .

Mena kāore e ranea ēnei tikanga hei whakahaere i ō tohumate, ka tohua pea e tō tākuta tētahi o ngā rongoā e kiia nei ko te dichlorphenamide , ko te acetazolamide rānei.

Ka taea te ārai i te whanaketanga o te myotonia?

Ko te myotonia te take o ngā huringa ira, nō reira kāore he mea e taea e koe te mahi hei ārai. Mena he myotonia tō tētahi o tō whānau, he mate rānei tōu, ā, kei te whakaaro koe ki te whānau tamariki, whakaarohia te toro atu ki tētahi kaitohutohu ira . Ka taea e rātou te whakamārama i tō tūponotanga ki te tuku iho i te mate me te tuku iho ki tō whakatupuranga e whai ake nei.

He aha te āhua o te mate mō te tangata e pāngia ana e te myotonia? (Te āhua o te mate)

Mena he myotonia tōu, ka whakawhirinaki tō tirohanga ki ētahi āhuatanga. Kei roto i ēnei ko:

• Te momo myotonia kei a koe.
• Te kaha o ō tohumate.
• Te urupare a tō tinana ki te maimoatanga.
• Tō hauora whānui me tō tau.

Ka taea e tō tākuta te hoatu he whakaaro pai ki a koe mō te āhua o tō āhua ā muri ake nei, engari ko te tikanga whānui he penei:

• He pai te āhua o te mate mō ngā myotonia kore-dystrophic , engari ka rerekē pea tēnei i runga i te kaha o ō tohumate.
• Ko te matapae mō ngā myotonia dystrophic e whakawhirinaki ana ki te kaha o te pānga ki ētahi atu pūnaha tinana. Ka taea hoki e te myotonic dystrophy momo 1 me te 2 te whakapoto i te roanga o te oranga.
• Ka kino haere te pararutiki ā-wā i te kaumātuatanga, ā, ka arahi pea ki ngā raruraru uaua kino, tuturu (myopathy) .

Āhea ahau me toro atu ki te tākuta?

He mate mau tonu/roa te myotonia. Nō reira, me toro atu koe ki tētahi tākuta puta noa i tō oranga kia aroturukihia ai ō tohumate me te kite i te pai o te mahi a tō maimoatanga.

He mea nui, ko ngā āhuatanga e pā ana ki te myotonia ka pā ki tō urupare ki te mate whakamoe . Mena kua whakaritea kia pokangahia koe, me kōrero koe ki tō tākuta mō tēnā. Me kōrero anō hoki koe ki te tohunga whakamoe e tiaki ana i a koe i te wā o te pokanga.

He mea noa te ohorere me te mokemoke ina kitea he mate onge kei a koe. He rerekē te pānga o te myotonia ki te katoa, nō reira he maha āu pātai. Ka noho tahi tō tīma hauora me koe puta noa i tō haerenga tātaritanga me te maimoatanga. Tikina he tautoko mai i a rātou, me ō whanaunga hoki.

Ngā mea tino nui hei maumahara mā tātou (Karere hei tango ki te kāinga)

Ko te myotonia he āhuatanga e kore ai e tere te whakangā o ngā uaua i muri i te whakapiko. Nā ngā āhuatanga ira tēnei.

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- E rua ngā momo matua o tēnei: ​​te mate pukupuku (te pakaru o te hanganga o ngā uaua) me te mate pukupuku kore-mate pukupuku (kāore he pakaru o te hanganga).

- Tērā pea ko te pakari o ngā uaua, te ngoikore, te mamae, me te ngenge. He tohu motuhake anō hoki ō ētahi momo.

- Ka whakamahia ngā whakamātautau toto, EMG, me ngā whakamātautau ira hei tautuhi i te mate.

- Ka rerekē te maimoatanga i runga i te momo mate me ngā tohu. Kei roto i tēnei ko te whakaora tinana, te rongoā, me te karo i ētahi mea (ngā mea whakaoho).

- Kāore e taea te ārai i tēnei, engari ka taea e koe te tiki āwhina mai i te tohutohu ira.

- Ki te pā mai ēnei tohu ki a koe, me toro atu ki te tākuta. He mea tino nui te tautuhi mate me te mahere maimoatanga tika māu.

` Myotonia, Myotonia, Pakaritanga uaua, Ngā mate ira, Myotonia ngoikore, Myotonia ngoikore, Myotonia kore-kino, Ngā mate uaua