He pakari tō tinana, kāore he take? Me kōrero tātou mō te Stiff Person Syndrome!

Ka rongo koe i ētahi wā ka whakawhāiti ō uaua, kāore i te mārama te take, ka wiriwiri rānei ō uaua? I ētahi wā ka puta tēnei ina rongo koe i tētahi haruru nui, ina tūtaki rānei tētahi ki a koe. Mena ka puta pinepine ēnei mea, he mea pai kia tirohia. I tēnei rā ka kōrero tātou mō tētahi mate tino onge ka puta ai ngā tohu rite. Ko te ingoa o tēnei ko te Stiff Person Syndrome (SPS) .

He aha te mate o te tangata uaua?

He whakarāpopototanga noa iho, ko te Stiff Person Syndrome (SPS) he mate pūnaha io onge . Ka puta mai i te wā e whakaeke hē ana te pūnaha ārai mate o tō tātou tinana, e tiaki ana i a tātou i te mate, i ō tātou ake pūtau io hauora. He mate pūnaha ārai mate tēnei. Ka meinga ngā uaua o tō puku, inā koa tō puku, tō uma, me tō tuara , kia pakari, kia mārō rānei . I roto i te wā, ka horapa pea tēnei mārō me te pupuhi ki ētahi atu uaua o ō waewae me ō ringa. Ka uaua pea te hikoi, ka hinga pinepine koe, ā, ka nui ake te tūponotanga ka whara koe.

I mua i huaina tēnei ko te "stiff man syndrome," engari kua whakarerekētia taua ingoa ināianei nā te mea ka pā tēnei mate ki te tangata katoa, ahakoa te pakeke, te ira tangata rānei.

He taumaha rawa te tātaritanga o tō mate. Engari mā tō rōpū hauora e waihanga he mahere maimoatanga e pai ana mōu me ō tohumate. Ka rerekē pea tēnei i ia tangata, i ia tangata. Ko te mea nui ko te toro atu ki ō tākuta i ia wā, te tiki i te tautoko e hiahiatia ana e koe, me te ngana kia noho hauora.

He aha ngā tohu o te Stiff Person Syndrome (SPS)?

E rua ngā tohu matua o tēnei mate:

• Te pakari, te pūmau rānei o te uaua.
• Ngā mamae o te uaua.

Ahakoa ka tīmata ēnei tohu i ngā tau katoa, ko te nuinga o te wā ka kitea i waenga i te 30 me te 40 tau .

Ka horapa ngā tohu o te Stiff Person Syndrome ki ētahi atu wāhanga o te tinana, ā, ka kino haere i roto i te wā. Mō ētahi tāngata, ka roa pea ngā marama ki ngā tau ka puta mai ngā tohu. Mō ētahi atu, ka noho tonu pea ngā tohu mō ngā tau. Mō ētahi tāngata, ka kino haere pea ngā tohu, ā, ka kore e taea te mahi i ngā mahi o ia rā.

Te pakari o te uaua

He maha ngā wā, ko te tohu tuatahi ka kitea e koe me te Stiff Person Syndrome ko te pakari o ngā uaua i roto i te tinana . Arā, i ngā wāhi pēnei i te puku, te uma, me te tuara. Ka taea e tēnei pakari te whakararu me te mamae. Ka taea e ēnei tohu te haere mai, te haere atu me te kore he take e kitea ana. Ka pā hoki te mate ki ngā ringa me ngā waewae. I te piki haere o te pakari, ka whanake pea ētahi tāngata i ngā tū āhua rerekē e uaua ai te hikoi, te neke rānei.

Ngā mamae o te uaua

Ko ngā mamae o te uaua tētahi atu tohu nui o tēnei mate. Ka pā pea ēnei ki te tinana katoa, ka pā rānei ki tētahi wāhi motuhake. Ka roa pea ēnei mamae mō ngā hēkona, meneti, tae noa ki ngā hāora. Whakaarohia, i a koe e tū noa ana, ka pāngia tō waewae, tō tuara rānei e te kikī me te wiri. He wheako tino mamae.

He aha ngā pūtake o te Stiff Person Syndrome (SPS)?

He maha ngā take ka puta ohorere mai ai ngā tohu pēnei i te wiri o te uaua:

• He haruru ohorere, he haruru nui rānei. Whakaarohia mēnā kei te hikoi koe i te tiriti, ā, ka tangi ohorere te pū o tētahi motuka, ka taka rānei tētahi mea i te kāinga ki te whenua me te pakū nui.
• Te pā atu ki te tinana, te whakaohooho rānei. Mēnā ka pā whakarere mai tetahi ki a koe, ka papaki atu.
• Ngā huringa o te pāmahana, inā koa ngā taiao makariri. Mēnā ka haere ohorere koe ki tētahi wāhi makariri, ka pā mai rānei te wai matao ki tō tinana.
• Ngā huihuinga ahotea. Ngā wā pouri, ngā wā whakamataku.

Nā te mea ka puta ēnei māuiui uaua i ngā wā e kore e taea te matapae, ka pāngia ētahi tāngata e te Stiff Person Syndrome e te manukanuka me te agoraphobia . Ko te agoraphobia he wehi nui ki te haere ki ngā wāhi kīkī, ki te haere ki waho, ki te wehe atu rānei i te kāinga. Nā te mea i waho, he uaua ki te mawhiti atu i ēnei whakaohooho.

He aha te take o te Stiff Person Syndrome (SPS)?

Kāore anō kia tino mārama ngā kairangahau he aha te pūtake o tēnei mate, engari e whakapono ana rātou he mate pūnaha ārai mate tēnei, arā, ko te pūnaha ārai mate o te tinana ka whakaeke i ngā pūtau hauora.

Kua whakaatuhia e ngā rangahau he pānga kei roto ko ngā paturopi . He maha ngā tāngata e pāngia ana e te Stiff Person Syndrome, he paturopi ā rātou ki tētahi whākōkī e kiia nei ko te Glutamic Acid Decarboxylase (GAD) . Ka āwhina tēnei whākōkī (GAD) ki te hanga i te neurotransmitter gamma-aminobutyric acid (GABA) . Ka āwhina tēnei neurotransmitter (GABA) ki te whakahaere i te mahi a te uaua.

Engari kāore anō kia tino mārama ngā kairangahau ki te pānga o te whākōkī `(GAD)` ki te whanaketanga me te haere whakamua o te Stiff Person Syndrome. He mea hei maumahara, ko te whai paturopi `(GAD)` i roto i tō tinana, ehara i te mea kei a koe te Stiff Person Syndrome. Tērā pea he iti noa iho o te taupori whānui kei roto i ō rātou tinana ēnei paturopi `(GAD)`, ā, kāore he pānga kino.

Haunga te GAD, kua kitea he hononga ētahi atu paturopi pērā i te glycine receptor, te amphiphysin, me te DPPX (dipeptidyl peptidase-like protein 6) ki te mate. Waihoki, tera pea kāore ētahi o ngā tūroro i te whai i tētahi o ēnei paturopi. Kei te haere tonu ngā rangahau hei whakatau he aha atu ngā paturopi kei roto.

He aha ngā āhuatanga mōrearea mō te Stiff Person Syndrome (SPS)?

E rua ngā wā ka nui ake te kitea o te Stiff Person syndrome i roto i ngā wāhine . Ka taea hoki te puta me ētahi atu mate pūnaha ārai mate. Hei tauira:

• Mate huka momo 1
• Mate tairoi ā-tinana
• Te kiriuhi
• Te mate toto kino
• Mate celiac

He pēhea te noa o te Stiff Person Syndrome (SPS)?

He mate tino onge tēnei. E kiia ana kotahi anake i roto i te miriona tāngata e pāngia ana e tēnei mate. Ko te tikanga he mate tino onge.

Me pēhea te tātaritanga o te Stiff Person Syndrome (SPS)?

Ka tātaritatia tēnei mate e te tākuta mā roto i ngā tohu, ngā whakamātautau, me ngā whakamātautau motuhake. Ka pātai atu rātou ki a koe mō ō tohu, ka whakahaere i tētahi whakamātautau tinana, ā, ka whakahaere hoki i tētahi whakamātautau io.

Mena kei te whakapae te tākuta kei a koe te Stiff Person Syndrome, ka whakahaerehia pea e rātou ngā whakamātautau pēnei i:

• Whakamātautau toto paturopi: He āwhina tēnei ki te tirotiro i te aroaro o ngā paturopi ki te (GAD) (me ētahi atu paturopi e pā ana) me te whakakore i ētahi atu mate hauora.
• Te Electromyography (EMG): Mā tēnei ka ine i te ngohe hiko o ō uaua. Ka āwhina pea tēnei ki te whakakore i ētahi atu take o ō tohumate.
• Wero tuarā (tapā tuaiwi): I tēnei whakamātautau, ka whakamahia e te tākuta he ngira hei tango i tētahi tauira wai mai i tō taurakira, ā, ka whakamātauria mō ngā paturopi ki te GAD. He mea nui anō hoki tēnei hei whakakore i ētahi atu mate.

He uaua te tautuhi i te Stiff Person Syndrome nā te mea he onge, ā, he rite tonu ōna tohu ki ētahi atu mate pūnaha ārai mate, pērā i te ankylosing spondylitis me te multiple sclerosis.

He momo mate tangata uaua (SPS) anō kei reira?

He maha ngā momo rerekē o tēnei mate:

• Te mate tangata pakari matarohia: Koinei te momo tino noa. He maha ngā wā ka hono atu ki ngā paturopi GAD, engari kua kitea e ngā rangahau he maha atu anō ngā paturopi e whai wāhi ana.
• Ngā momo rerekē o te mate tangata uaua:I raro i tēnei, kei reira ngā momo rerekētanga e pā ana ki ngā wāhanga motuhake o te tinana anake, e whakaatu ana rānei i ngā tohu maha atu pēnei i te ngaronga o te mana whakahaere i te wā e hikoi ana (`ataxia`).
• Te mate roro nui haere me te uaua me te myoclonus (PERM): He momo kino ake te PERM o te mate tangata uaua. Ka taea e ia te ngaro o te māramatanga, ngā raruraru nekehanga kanohi, te ataxia, me te ngoikore o te pūnaha autonomic. Me whakauru te hunga whai PERM ki te hōhipera mō te ngoikore o te pūnaha autonomic.

Me pēhea te rongoā i te Stiff Person Syndrome (SPS)?

E rua ngā tikanga matua mō te rongoā i tēnei:

• Ngā rongoā me ngā maimoatanga hei whakahaere i ngā tohu.
• Te rongoā ārai mate, te rongoā whakarerekē mate rānei.

He rerekē ngā whiringa maimoatanga i runga i ō tohumate. Ko te whāinga matua o te maimoatanga he whakahaere i te pānga o ō tohumate ki a koe, me te whakapai ake i tō nekehanga me tō whakamarie.

Tērā pea kei roto i tō tīma hauora ētahi tohunga, pērā i:

• Ngā tohunga roro, inā koa ngā tohunga roro.
• Ngā kaiwhakaora mahi me ngā kaiwhakaora tinana.
• Ngā tohunga rongoā tinana me te whakaora tinana.
• Ngā kaiwhakaora kōrero.
• Ngā tohunga hauora hinengaro, hei tauira, ngā tohunga hinengaro.

Ngā rongoā me ngā maimoatanga

Ka taea e ēnei rongoā te āwhina ki te whakaiti i te pakari o te uaua, te pakari, me te mamae o ngā uaua:

• Benzodiazepines: He momo rongoā ēnei e whakamahia ana hei rongoā i ngā momo āhuatanga, tae atu ki te manukanuka, te mate haurangi, me te kore moe. Ka pāngia e rātou te tohu neurotransmitter (GABA). He maha ngā wā ka tohua e ngā tākuta te diazepam hei rongoā tuatahi mō te Stiff Person Syndrome.
• Ngā rongoā whakangāwari uaua: Ka taea e ngā rongoā pēnei i a Baclofen te āwhina i ngā mamae o te uaua. Ka whakangāwari i ngā uaua, ka whakaiti hoki i te pakari.
• Ngā rongoā mamae neuropathic: Ko ngā rongoā pēnei i te gabapentin me te pregabalin ka pā ki ngā tohu GABA me te āwhina i ngā tohu.

Ko ētahi atu maimoatanga hei āwhina i te whakahaere i ngā tohu ko:

• Te whakaora tinana, te whakaora mahi rānei.
• Mirimiri.
• Whakaora wai (whakaora wai).
• Te rongoā wera.
• Te wero ngira.

Te rongoā ārai mate

He taunakitanga kei te wātea mai i te rongoā immunoglobulin ā-uaua (IVIg) (he momo immunotherapy anō hoki) hei āwhina i te whakaiti i ngā tohu o te Stiff Person Syndrome. Kei roto i te IVIg ngā immunoglobulin (ngā paturopi tūturu) i kohaina mai e ngā mano tini o te tangata he pūnaha ārai mate hauora.

Ka taea te whakaora i te Stiff Person Syndrome (SPS)?

I tēnei wā, kāore he rongoā mō te Stiff Person Syndrome. Ko te rongoā anake ka āwhina i te whakahaere i ngā tohumate.

He aha te tirohanga mō te Stiff Person Syndrome (SPS)?

He mate pumau tēnei mō te wā katoa . He rerekē te tirohanga mai i te tangata ki te tangata, i runga i ētahi āhuatanga:

• Te kaha o ngā tohu.
• Te tere o te horapa o te mate.
• E pēhea ana te angitu o te maimoatanga?

He mea nui kia tīmata te maimoatanga i te tīmatanga tonu o ngā tohu. Ka āwhina tēnei ki te ārai, ki te whakaiti rānei i te horapa o te mate, ā, ka āwhina hoki ki te ārai i ngā raruraru roa. Ahakoa he maha ngā tāngata e whiwhi ana i te whakaoranga mā te rongoā, he uaua pea i ētahi wā ki te whakahaere i ngā mea e whakaoho ana i te mamae o te uaua.

Tērā pea ka uaua haere te hikoi i roto i te wā. Tērā pea ka heke haere te kaha ki te mahi i ngā mahi o ia rā. I te haere whakamua o te mate, ka piki ake te tūponotanga o te hinga. Me whakamahi pea koe i te tokotoko, i te taputapu hikoi, i te tūru wīra rānei.

Ka taea te ārai i te Stiff Person Syndrome (SPS)?

Nā te mea he mate pūnaha ārai mate tēnei, kāore he mea e taea e koe te mahi hei ārai .

Me pēhea taku tiaki i ahau anō mēnā kei a au te Stiff Person Syndrome (SPS)?

Ki te taea, ngana ki te kimi i tētahi tākuta e tohunga ana ki te rangahau me te rongoā i te Stiff Person Syndrome. He onge tēnei mate, nō reira he uaua pea. Ki te hiahia koe ki te whiwhi i te tiaki hauora pai rawa atu, me te whiwhi i te kounga pai o te oranga, me kōrero koe, me te ngākau nui ki tēnei mate.

He mea noa ki te hunga whai mate nei te pāngia e ngā raruraru hauora hinengaro, pērā i te manukanuka , te pouri rānei. Mena kei te pāngia koe e ēnei tohu, he mea nui kia kōrero koe ki tō tākuta, ki tētahi tohunga hauora hinengaro rānei, pērā i te tohunga hinengaro.

Ki te taea e koe me tō whānau, ka taea hoki e koe te whakauru atu ki tētahi rōpū tautoko hei tūtaki māu ki ētahi atu e mārama ana ki ō wheako.

Āhea ahau me toro atu ki te tākuta?

Mena kei a koe te Stiff Person Syndrome, me hui tonu koe me tō tīma hauora kia kite ai mēnā kei te angitu tō maimoatanga, kia aroturukitia ai hoki te whanaketanga o ō tohu.

Ki te kite koe i ngā tohu hou, i ngā pānga taha rānei o ngā rongoā, me kōrero ki tō tākuta.

He aha ngā pātai me pātai atu e au ki taku tākuta?

Mena kei a koe te Stiff Person Syndrome, he pai pea te pātai atu ki tō tākuta i ēnei pātai:

• He aha ngā maimoatanga e taunaki ana koe mō aku tohumate?
• He aha taku e taea ai te mahi i te kāinga hei āwhina i aku tohumate?
• He aha ngā tohu o ngā raruraru me tirotiro e au?
• He aha te āhua o tōku hauora ā muri ake nei?
• He whakamātautau haumanu kei reira mō te Stiff Person Syndrome?

Hei whakamutunga, ngā mea hei maumahara (Karere hei tango ki te kāinga)

Ehara i te mea ngāwari te mōhio kei a koe tētahi mate onge pēnei i te Stiff Person Syndrome. Tērā pea ka pōuri koe, ka manukanuka, ka kore e tino mōhio mō te heke mai. Engari kāore koe e hiahia ki te aro atu ki tēnei ko koe anake. Kei konei tō tīma hauora hei whakautu i ō pātai me te āwhina i a koe ki te whakahaere i ō tohumate.

Ahakoa e aro ana tō maimoatanga ki tō hauora tinana, kia tūpato hoki ki tō hauora hinengaro . He rerekē ngā tohu i ia tangata, i ia tangata. Engari ko te noho me te mamae, te kore whakamarie, me ngā uaua e kore e taea te matapae ka pā kino ki tō oranga hinengaro. Ka uaua pea te haere ki waho me te mahi i ngā mahi o ia rā nā tēnei mate. Nō reira, ki te hiahia tautoko koe, kaua e mangere ki te toro atu ki tētahi tākuta me ō whanaunga. Kia mahara, ehara i te mea ko koe anake.

` Stiff Person Syndrome, te pakari o te uaua, ngā mate pūnaha io, te ārai mate, ngā paturopi GAD, te GABA, te wiri o te uaua, ngā tohu SPS