Kusagadzikana kweprodromal autonomic muzvirwere zvemuviri weLewy

zuva: May 2026
Yakagadzirirwa ne SIC Members: Sephira Ryman, PhD, naMichele Matarazzo, MD
Authors: David S. Goldstein, MD, PhD, Abhimanyu Mahajan MD, MHS; Alison Yarnall, PhD, MBBS
Vapepeti: Lorraine Kalia, MD, PhD; Per Svenningsson, MD, PhD
ziviso
Kusashanda zvakanaka kweprodromal autonomic disorder kwave kuri chinhu chakakosha pakutsvaga mukunzwisisa zvirwere zvemuviri zveLewy, zvinosanganisira chirwere cheParkinson (PD) uye Dementia neLewy bodies (DLB). Humbowo huri kuramba huchikwira hunoratidza kuti kusashanda zvakanaka kwemoyo, gastrointestinal, weti, thermoregulatory, nedzimwe nzvimbo dze autonomic kunogona kutanga zviratidzo zvemuviri kana zvepfungwa kwemakore.
Hurukuro iyi ine chinangwa chekukurukura humbowo hunotsigira kusashanda zvakanaka kwemuviri muzvirwere zveLewy zveprodromal, izvo zvikamu zvemuviri zvinogona kuva neruzivo rwakanyanya pakuonekwa kwekutanga, maturusi matsva ekuongorora, uye kunzwisisa kwezviwanikwa izvi uye kukosha kwazvo mukurapwa kwekiriniki.
Kuburitswa kweblog iri kwakagadzirwa pamwe chete naMichelle Matarrazo pamwe neMDS Podcast.
Teerera chikamu chepodcast:
1. Ndeupi humbowo hwekiriniki kana hwebiomarker hunotsigira kuvapo kwekusashanda zvakanaka kwemuviri muchikamu cheprodromal chezvirwere zvemuviri zveLewy?
Dr. David Goldstein
Humbowo hwakasimba hwekuti pane chimwe chinhu chisina kujairika muzvirwere zvemuviri weLewy hwakabva mukufungidzira kwemoyo kwetsinga dzeropa. Mushumo wedu wekutanga muna 1997 muNew England Journal of Medicine wakatsanangura kekutanga kuti muvarwere vane zvirwere zvemuviri weLewy, chirwere cheParkinson uye pure autonomic failure, vanoratidza kurasikirwa kwetsinga dzemwoyo dzinobatsira nemoyo vachishandisa [18F] fluordopamine PET scanning. Hatinawo kuratidza musiyano mu non-Lewy body synucleinopathy, Shy-Drager syndrome, iyo yakazonzi multiple system atrophy.
Mumakore makumi matatu apfuura takasimbisa zvakawanikwa izvi mushure mekufa nekuongorora huwandu hwe norepinephrine mumoyo. Pari zvino hapana mubvunzo kuti pane kushomeka kwakakura kwe noradrenergic yemwoyo muzvirwere zvemuviri zveLewy uye kazhinji hapana mu multiple system atrophy.
Ndirini panoitika zvirwere izvi apo kusarongeka uku kwemuviri kunotanga kuonekwa? Zvirinani mune izvo zvinozivikanwa semuviri wekutanga we central synucleinopathy, chirwere chemwoyo chinotanga kutanga kweParkinsonism kana kusagadzikana kwepfungwa sezvinoitwa mu dementia neLewy bodies.
Dr. Abhimanyu Mahajan
Imwe yemapepa ekutanga akanakisa pamusoro penyaya iyi yakaburitswa muna 2013 naProfessor Ron Postuma, kwavakatsanangura maitiro ekuzvitonga muboka revarwere 91. Vakaona chiratidzo chimwe chete pachikamu chimwe nechimwe chekuzvitonga, chaisanganisira zviratidzo zve orthostatic, kusashanda zvakanaka kweweti, kuvimbiwa, uye kusashanda zvakanaka kwenhengo dzepabonde muchikamu cheprodromal chechirwere.
Munguva pfupi yapfuura takashandisa dhatabhesi reParkinson's Progression Markers Initiative (PPMI) kuti tione kana mutoro wekutanga we autonomic uchifanotaura kana chiitiko chekukanganisika kwepfungwa mu PD yeprodromal. Takashandisa Scales for Outcomes in Parkinson's Disease Autonomic Dysfunction (SCOPA-AUT), inova chiyero chakabvumidzwa neMDS. Kana ukapindura zvakanaka pane mumwe wemibvunzo iyoyo mu subdomain yega yega, zvinoshamisa kuti vangani vevarwere ava vari muprodrome vane kanenge chiratidzo chimwe chete che autonomic.
Dr. Alison Yarnall
Mu prodromal DLB, kukanganiswa kwepfungwa kudiki neLewy bodies, tine mapoka emhando dzakasiyana-siyana pano muNewcastle atakatevera kwemakore akati wandei uye takaenzanisa izvozvo nekukanganiswa kwepfungwa kudiki neAlzheimer's uye nevanodzora. Zvechokwadi, kubva pane zvakaitika kwatiri, takaona kuti zviratidzo zve autonomic zvaiwanzotaurwa, uye izvozvo zvaiwanzoonekwa kana tichienzanisa nekukwegura kwakajairika. Zvisinei, zvaisawanzoonekwa kana tichienzanisa neavo vane MCI vane Alzheimer's type pathology.
1.1. Ko zvikamu zvakasiyana-siyana zve autonomic (semuenzaniso cardiovascular vs. gastrointestinal) zvinowanzova zvisina kujairika here mumapoka eprodromal cohorts?
Dr. Abhimanyu Mahajan
Pane chikonzero chekuti kusashanda zvakanaka kwemoyo neropa kune simba guru pakuchinja kwemuviri uye mhedzisiro yekuziva. Takaratidza kuti kusashanda zvakanaka kwemoyo neropa zvichibva paSCOPA-AUT kwakabatana zvakanyanya nenjodzi yakawedzera yekusashanda zvakanaka kwepfungwa kana dementia (P < 0.001). Mutoro wezviratidzo zvemwoyo neropa, kunyanya, waive chiratidzo chakasimba chega chega (HR = 5.21, P = 0.01).
Kusashanda zvakanaka kwemoyo neropa kwakakonzera kuti zviratidzo zvemuviri zvemwoyo zvishande zvakanaka. Zvisinei, patinoziva zvakawanda, ndipo patinoziva zvishoma nezvekusashanda zvakanaka kwemuviri. Kana zviratidzo zviri nani zvikagadziriswa mune mamwe ma subdomain, izvi zvinogonawo kuva nesimba guru.
1.2. Zviratidzo zveprodromal autonomic zvinoenzaniswa sei mukunzwisisa uye zvakananga nezvimwe zviratidzo zveprodromal?
Dr. Alison Yarnall
Takaona kuti zviratidzo zvemudumbu uye zvekunzwa kwemukati memuviri zvaiwanzotaurwa, asi zviratidzo zvemwoyo zvaive zvakanyanya mumapoka edu eDLB ekubuda kweropa. Zviratidzo izvi zvakawanda zvine manzwiro akanyanya, saka zvinowanzoonekwa. Tinozviona muvanhu vanopfuura hafu yevarwere veDLB vekubuda kweropa, kunyanya kuvimbiwa kana hypotension ye orthostatic. Zviratidzo izvi zvine hunyanzvi hwakaderera zvikuru kana tichienzanisa nezvimwe zviratidzo zvikuru zvatinoona, zvakaita sekuvapo kweREM sleep behavior disorder kana dopamine transporter imaging isina kujairika.
Inzvimbo iri kuchinja uye tiri kuona kusashanda zvakanaka kwemuviri. Kune rumwe rutivi, zviratidzo izvi zvakawanda zvinowanzoonekwawo muvanhu vakuru.
2. Ndedzipi nzira dziri kubuda dzekuongorora mashandiro emuviri dzinopa vimbiso huru yekuona chirwere cheprodromal?
Dr. David Goldstein
Kushaya simba kweropa muuropi (orthostatic hypotension) hakunyatso ongororwa. Vanachiremba vetsinga havawanzo funga nezvazvo kunze kwekunge murwere aine chiratidzo. Uchaderedza kuwanda kwekushaya simba kweropa muuropi kana uchivimba nezviratidzo.
Kune biomarkers dzemuviri, dzeuropi, dzepfungwa, dzepfungwa, uye dzemicro-neuroimaging dzinopindirana muVenn diagram. Kana zvasvika paphysiological, zviratidzo zve baroreflex uye sympathetic neural function zvinogona kubatsira. Kana zvasvika pa neurochemical, CSF levels ye catecholamines uye metabolites dzadzo dzinogona kubatsira. Kana zvasvika pa neuroimaging, takatotaura nezve cardiac PET scanning mu body-first PD ne DLB. Munyika chaiyo, MIBG SPECT scanning inogona kubatsira. Kana zvasvika pa micro-neuroimaging, skin biopsies inogona kubatsira, ichitsvaga alpha-synuclein deposition mu sympathetic noradrenergic nerves inopa blood vessels, sweat glands, uye arrector pili muscle. Kuwedzera kune neurochemistry nezve catecholamines, kune CSF synnuclein seeding, iyo yaifanotaura mukudzidza kwedu kwenguva refu.
Mune ramangwana, ndinotarisira kuti pachava nekuchinja kubva pakuongorora kwekiriniki chete kuenda kune nzira yealgorithmic inosanganisira biomarkers. Tanga nezvinhu zvinokurumidza, zvakachipa, zvakachengeteka, asi zvinonzwisisika zvakakwana - kubva pamaonero iwayo, kuongorora kusiyana kwekurova kwemoyo kunyanya munzvimbo yema frequency. Mushure meizvozvo, enda kune synuclein markers uchiteverwa ne fluorodopa PET scanning yeuropi. Kana zvichikwanisika, fluorodopamine PET scanning yemoyo.
Dr. Abhimanyu Mahajan
Kuva nezviratidzo zvebiomarker zvakananga kwakakosha kuti tinzwisise pathophysiology. Kwete pathophysiology chete, asi physiology yekuti zvese izvi zvinoshanda sei. Panguva imwe chete, pane zvakawanda zvakanaka kuti tisarerutse kubatsira kwezvatinogona kuita padivi pemubhedha.
Kumanikidzwa kweropa kweOrthostatic hakushandiswi zvakakwana. Takaita ongororo paYunivhesiti yeCincinnati tikaongorora kuyerwa kweropa re orthostatic hypotension padivi pemubhedha. Vanenge 30% vevarwere vaisaziva kuti BP yavo yaive orthostatic. Chinonyanya kukosha ndechekuti, kunyangwe vasina zviratidzo, njodzi yemabasa akaipa ezuva nezuva uye kudonha yaive ichiri yakakwira, zvichiratidza kuti kunyangwe murwere asingazive orthostatic hypotension, hazvirevi kuti haina zvayakanganisa.
Kunyangwe zviratidzo zvisinganyanyi kunzwisiswa, zvakakosha kuzvibata padivi pemubhedha. Ruzivo rwakawanda rwatinarwo nezvemhedzisiro yezviratidzo zve orthostatic uye kukanganisa kwazvinoita pane dzimwe nzvimbo muPD runobva muzvidzidzo zvekutarisa uye mapoka anoteverwa mukiriniki, zvinosanganisira zviyero zvakaita seSCOPA-AUT nezvimwe. Zvakakosha kubata chirwere panguva yese yekufamba kwacho. Kukwanisa kuita zviyero izvi uye kuongororwa uku padivi pemubhedha kunotipa kugona kubata ruzivo kubva kumasampuru makuru izvo zvinotibatsira kunzwisisa kusiyana kwezvirwere zvatinoona.
Nzira dzese dzekuongorora kusashanda zvakanaka kwemuviri dzinofanira kushanda panguva imwe chete. Ehe, hadzina basa.
2.1. Munoona sei kuti kutarisa varwere vari kure nguva dzose kuri kuvandudza kuongororwa kwezvirwere uye kunzwisisa kwedu varwere vari kunze kwekiriniki?
Dr. Alison Yarnall
Kugara tichitarisa zvinhu ndizvo zviri kufamba pasi rose. Pano muUK tine chirongwa chemakore gumi cheNHS yedu. Chimwe chezvinhu zvitatu ndechekuchinja kubva kuchipatara kuenda kune nharaunda, kubva pakurwara kuenda pakudzivirira, kusvika pakutarisira kutarisirwa kumba. Izvi zvichabatsira kuyera kusashanda zvakanaka kwemuviri, pamwe nezvimwe zvinhu zvakawanda, zvakaita sekurara, kuneta, kufamba-famba, kufamba-famba, uye kuziva. Kuyera izvi munzvimbo yemumwe munhu mukati memazuva manomwe, kana zvakafanana, zvinoreva kuti hatifanirwe kuvimba nemufananidzo mumwe chete usingazotore kuchinja-chinja kuripo kuri chikamu chePD neDLB.
Zvinogona kuderedza mutoro wenguva yekurapwa. Kana tikawana migumisiro yevarwere panguva chaiyo, tinogona kukwanisa kuchinja tiri kure kudzivirira vanhu kuti vauye kukiriniki. Izvi zvinobvumirawo kubatanidzwa. Varwere vakwegura, vasina simba, vanhu vari kumaruwa vangawana rubatsiro rwakafanana nerwevaya vanogara pedyo nemayunivhesiti, zvese maererano nemikana yekurapwa uye yekutsvaga ruzivo.
Kutora nzira yealgorithm yakafanana neyakataurwa naDavid ndiyo nzira yakanakisisa. Kutora zviyero zvakawanda uye mamakisi zvinogona kugadzirwa kusiyanisa avo vanofambira mberi nekukurumidza, zvishoma nezvishoma, uye avo vanoda kupindira.
Dr. David Goldstein
Ndingawedzerawo matanho ekutarisa BP nguva dzose. Zvakakosha kuziva kuti kana hypotension yeropa iri kukwira zvakanyanya masikati. Kazhinji inokwira zvakanyanya mangwanani, uye kana ikanyanya kuipa mangwanani, hausi kuzorapa munhu wacho nemushonga wevasoconstrictor nguva dzese.
3. Ndezvipi zvinorehwa nepfungwa idzi pakuongorora njodzi uye kutarisa vanhu vane prodromal autonomic dysfunction?
Dr. David Goldstein
Ndichataura nezve PD risk study nekuti ndeimwe yezvidzidzo zvishoma zvenguva refu zvevanhu vane zvinhu zvakawanda zvine njodzi kuti vaone kukosha kwekufungidzira kwemhando dzakasiyana dze biomarkers. Kufanotaura, kwakavakirwa pazvinhu zvine njodzi, nhoroondo yemhuri, maitiro ekurota, kusashanda zvakanaka kwemafuta, uye kusashivirira orthostatic kwakasimba kwazvo. Pamwe chete, zvinowedzera zvakanyanya mhedzisiro yekufanotaura uye yekufanotaura maererano ne phenoconversion mune ramangwana. Kana biomarkers dze laboratory dze catecholamine deficiency ye alpha-synucleinopathy dzawedzerwa, saka muchokwadi haisi nyaya yenjodzi zvakare. Une chirwere ichi. Inyaya yenguva zviratidzo zvekiriniki zvisati zvaonekwa. Biomarkers idzodzo dzichachinja njodzi kusvika zana muzana. Remangwana riri mukuwedzera biomarkers kune zvinhu zvine njodzi zvatakatotaura nezvazvo.
4. Kana tichitarisa mberi, ndezvipi zvingangoitika pakurapa?
Dr. Alison Yarnall
Pamwe mune ramangwana, murwere achauya kukiriniki, waizoziva physiology yemuviri uye zviratidzo zvinogona kushandiswa kutsanangura marapirwo avo uye sarudzo dzekurapa kwavo. Hunhu hwe autonomic prodromal hunogona kutiudza nezvenjodzi uye kupatsanurwa kwenjodzi. Izvi zvinogona kupa rutsigiro kune waunoisa pamberi mukuyedzwa kwekiriniki.
Muedzo wekupedzisira wekiriniki uchava chimwe chinhu chinodzivisa munhu kushanduka kubva kuchirwere cheprodromal kuenda kuDLB kana PD inoratidzwa. Zviratidzo zvekiriniki uye zvebiomarker zvechirwere cheautonomic zvinogona kutibatsira kusarudza kuti ndiani anopinda muyedzo ipi yekiriniki.
Dr. Abhimanyu Mahajan
Kuvandudza miedzo yekiriniki ndiyo nzira chaiyo yekutevera. Zvino zvatava kuziva kuti kusashanda zvakanaka kwemuviri panguva yekutanga kunobatanidzwa nenguva inokurumidza yekuchinja kwemasero, kurwara kwakanyanya, kupona kushoma, uye pamwe njodzi huru yekuziva, zvakakosha kuisa muenzaniso muzvikamu zvakasiyana mumiedzo yekiriniki.
5. Ndezvipi zvinhu zviri kukosheswa pari zvino munharaunda iyi?
Dr. David Goldstein
Kuziva zviratidzo zvechirwere chisati chatanga. Kunyangwe muchirwere cheprodromal, pane kukuvara kukuru kwatovepo. Tariro yeramangwana ndeyekuziva maitiro echirwere munhu asati aona zviratidzo zvachose. Chinangwa hachisi chekurebesa nguva yechirwere, asi chakasiyana. Chinangwa ndechekunonotsa kutanga kwechirwere. Dai waigona kuwedzera nguva yehutano, ndicho chaizova chinangwa chevanhu.
Dr. Alison Yarnall
Kutarisira zviratidzo izvi zvakaoma kurapa zvemuviri kwakakosha. Pari zvino pane kushomeka kweuchapupu huripo, uye kazhinji kunokanganisa hupenyu hwemunhu zvakanyanya. Ndiyo ndiyo yandinonyanya kukoshesa pakutsvaga.
Dr. Abhimanyu Mahajan
Kuziva nzira dzekubata mutoro wemuviri wemurwere wese ane synucleinopathy anouya kukiriniki, angave ari murwere weprodromal, PD yekutanga, kana DLB yekupedzisira. Chero bedzi uchikwanisa kubata zvakakodzera uye kurapa zviratidzo izvozvo, kwete chete nekuda kwemhedzisiro yezviratidzo izvozvo, asiwo, uye uyu mufananidzo wepadenga, kudzivirira zviratidzo zvine chekuita nazvo mune ramangwana. Ndiwo waizova wekutanga kwandiri.
Hatinawo dzimwe nzira dzakakwana dzekurwisa dambudziko rekuziva. Kusashanda zvakanaka kwemuviri, kusanganisira kusagadzikana kwemoyo neropa, zvinogona kubatsira kunangana nekuvandudza dambudziko rekuziva. Tinofanira kuona nzira dzinovandudza kunzwisisa.
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