Inkqubo ye-atrophy ye-Multiple-uhlobo lwepakini

Inkqubo ye-atrophy- parkinsonian type (MSA-P) emininzi yinto enqabileyo ebangela iimpawu ezifanayo nesifo sikaParkinson. Nangona kunjalo, abantu abane-MSA-P banomonakalo obanzi kwinxalenye yenkqubo yeemvakalelo elawula imisebenzi ebalulekileyo enjengokubetha kwentliziyo, uxinzelelo lwegazi kunye nokubila.

Olunye udidi lwe-MSA yi-MSA-cerebellar. Ichaphazela ikakhulu iindawo ezinzulu kwingqondo, ngaphezulu ngentambo yomqolo.

Isizathu seMSA-P asaziwa. Iindawo ezichaphazelekayo zobuchopho ziyadibana kunye neendawo ezichaphazeleka sisifo sikaParkinson, ezineempawu ezifanayo. Ngesi sizathu, le subtype ye-MSA ibizwa ngokuba yi-parkinsonian.

I-MSA-P ihlala ifunyaniswa kumadoda amadala kunama-60.

I-MSA yonakalisa inkqubo yeemvakalelo. Esi sifo sihamba ngokukhawuleza. Phantse isiqingatha sabantu abane-MSA-P baphulukene nobuninzi bezakhono zabo zemoto kwisithuba seminyaka emihlanu ukuqala kwesi sifo.

Iimpawu zingabandakanya:

• Iinyikima
• Ubunzima bentshukumo, njengokucotha, ukulahleka kwebhalansi, ukushukuma xa uhamba
• Ukuwa rhoqo
• Ubuhlungu bemisipha kunye neentlungu (myalgia), kunye nokuqina
• Utshintsho lobuso, njengokubonakala kobuso kwimaski ebusweni kunye nokujonga
• Kunzima ukuhlafuna okanye ukugwinya (ngamanye amaxesha), ukungakwazi ukuvala umlomo
• Ukuphazamiseka kwiipateni zokulala (rhoqo ngexesha lokuhamba kwamehlo ngokukhawuleza [REM] ukulala ebusuku kakhulu)
• Ukuba nesiyezi okanye ukuwa isiqaqa xa umile okanye emva kokuma ngxi
• Iingxaki zokwakhiwa
• Ukuphulukana nolawulo kwizisu okanye isinyi
• Iingxaki zomsebenzi ezifuna iintshukumo ezincinci (ukulahleka kwezakhono ezifanelekileyo zemoto), ezinje ngokubhala okuncinci ekunzima ukufundwa
• Ukuphulukana nokubila kulo naliphi na ilungu lomzimba
• Yehla ekusebenzeni kwengqondo
• Isihlunu kunye neengxaki ngokugaya
• Iingxaki zokuma, njengokungaqiniseki, ukugoba, okanye ukugoba ngaphezulu
• Umbono utshintsho, ukuncipha okanye ukungaboni kakuhle
• Ilizwi nentetho iyatshintsha

Ezinye iimpawu ezinokuthi zenzeke kwesi sifo:

• Ukudideka
• Isifo sengqondo esiyingozi
• Uxinzelelo
• Ubunzima bokuphefumla obunxulumene nokulala, kubandakanya ukuphefumla ubuthongo okanye ukuvaleka kwindlela yomoya ekhokelela kwisandi esingcangcazelisayo

Umboneleli wakho wezempilo uya kukuvavanya, kwaye ajonge amehlo akho, imithambo-luvo kunye nezihlunu.

Uxinzelelo lwegazi lwakho luya kuthathwa ngelixa ulele phantsi kwaye umile.

Akukho zimvavanyo zenziwayo ukuqinisekisa esi sifo. Ugqirha ogxile kwinkqubo yemithambo-luvo (ugqirha wemithambo-luvo) unokwenza uxilongo ngokusekwe:

• Imbali yeempawu
• Iziphumo zoviwo lomzimba
• Ukulawula ezinye izizathu zempawu

Ukuvavanya ukunceda ukuqinisekisa ukuxilongwa kunokubandakanya:

• I-MRI yentloko
• Amanqanaba ePlasma norepinephrine
• Uvavanyo lomchamo lweemveliso zokuqhekeka kwe-norepinephrine (umchamo catecholamines)

Akukho lunyango lwe-MSA-P. Ayikho indlela eyaziwayo yokuthintela isifo ukuba singabi sibi. Injongo yonyango kukulawula iimpawu.

Amayeza e-Dopaminergic, anjenge-levodopa kunye ne-carbidopa, anokusetyenziselwa ukunciphisa ukungcangcazela kwangethuba okanye kancinci.

Kodwa, kubantu abaninzi abane-MSA-P, la mayeza awasebenzi kakuhle.

Amayeza anokusetyenziselwa ukunyanga uxinzelelo lwegazi olusezantsi.

I-pacemaker ecwangciselwe ukukhuthaza intliziyo ukuba ibethe ngokukhawuleza (ngokukhawuleza ngaphezu kwe-100 beats ngomzuzu) inokunyusa uxinzelelo lwegazi kwabanye abantu.

Ukuqunjelwa kunokunyangwa ngokutya okunesondlo esiphakamileyo kunye ne laxatives. Amayeza ayafumaneka ukunyanga iingxaki zokwakhiwa.

Olunye ulwazi kunye nenkxaso kubantu abane-MSA-P kunye neentsapho zabo zifumaneka kwi:

• Umbutho weSizwe weengxaki zoRare- rarediseases.org/rare-diseases/multiple-system-atrophy
• Umdibaniso weMSA- www.multiplesystematrophy.org/msa-resource/

Iziphumo zeMSA zihlwempuzekile. Ukuphulukana nemisebenzi yengqondo neyomzimba ngokuthe ngcembe kuya kusiba mandundu. Ukufa kwangethuba kunokwenzeka. Abantu bahlala iminyaka esi-7 ukuya kweli-9 emva kokuxilongwa.

Fowunela umnikezeli wakho ukuba uphuhlisa iimpawu zoku kuphazamiseka.

Tsalela umnikezeli wakho ukuba ufumene ukuba une-MSA kwaye iimpawu zakho ziyabuya okanye ziba mandundu. Biza kwakhona ukuba iimpawu ezintsha ziyavela, kubandakanya neziphumo ebezingalindelekanga zamayeza, ezinje:

• Utshintsho kulumkiso / indlela yokuziphatha / imo
• Ukuziphatha kakubi
• Ukuba nesiyezi
• Ukuba nemibono
• Iintshukumo zokuzibandakanya
• Ukuphulukana nokusebenza kwengqondo
• Isihlunu okanye ukugabha
• Ukudideka okukhulu okanye ukudideka

Ukuba unelungu losapho eline-MSA kwaye imeko yabo iyehla ukuya kwinqanaba lokuba awukwazi ukumkhathalela umntu ekhaya, funa ingcebiso kumboneleli welungu losapho.

Isifo seShy-Drager; I-neurologic orthostatic hypotension; Isifo seSy-McGee-Drager; Parkinson kunye nesifo; I-MSA-P; I-MSA-C

• Inkqubo ye-nervous central kunye ne-peripheral system

UFanciulli A, Wen WK wolwazi. Inkqubo yeatrophy yenkqubo ezininzi. N Engl J Med. Ngo-2015; 372 (3): 249-263. IINKCUKACHA: 25587949 pubmed.ncbi.nlm.nih.gov/25587949/.

Isifo sikaJankovic J. Parkinson kunye nezinye iingxaki zokuhamba. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, ii-eds. I-Neurology kaBradley kwiKlinikhi yokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2016: isahluko 96.

URomero-Ortuno R, uWilson KJ, uHampton JL. Ukuphazamiseka kwenkqubo ye-nervous autonomic. Ku: Gcwalisa i-HM, i-Rockwood K, i-Young J, ii-eds. Incwadi ka-Brocklehurst ye-Geriatric Medicine kunye neGerontology. Ngomhla we-8. IPhiladelphia, PA: Elsevier; Ngo-2017: isahluko 63.