Kusiyanisa mashandiro kubva kuidiopathic dystonia: Nzira itsva

Roberto, [00:01:00] tinokugamuchirai kupodcast uye ndinokutendai nekuve neni nhasi.

Purofesa Roberto Eleopra: Mhoro. Ndatenda Micki nekundikoka kupodcast iyi yakanaka. Zvinofadza kuva pano.

Dr. Michele Matarazzo: Zvakanaka, ngatitangei, Roberto. Ungatitsanangurira dambudziko iri muchidimbu here? Chii chinonzi functional dystonia uye nei zvakaoma kuiongorora kana tichienzanisa nedzimwe functional movement disorders?

Purofesa Roberto Eleopra: Ehe. Functional dystonia ndeimwe yezvirwere zvinowanzoonekwa uye zvinonetsa kuongororwa. Dambudziko iri rinosanganisira kufamba kusingawanzoitiki kunoitika pasina kuvhundutswa kwetsinga dzemuviri. Uye zvinowanzoonekwa nekusawirirana uye kusawirirana kwekiriniki.

Aya ndiwo maitiro makuru atinoshandisa pakuongorora chirwere chekufamba kwemuviri, asi functional dystonia inonyanya kuoma kuvanachiremba nekuti, [00:02:00] yakafanana zvakanyanya neidiopathic dystonia uye dzimwe nguva ine chinhu chisingawanzoitiki kana chakajairika, uye zvakare kana tikafunga kuti kutanga kamwe kamwe, chimiro chakagadzikana pakuzorora kana kuramba kufamba kwemunhu asinganyanyi kufamba.

Zvingangodaro zvichireva kuti chirwere che functional dystonia, asi zviratidzo izvi zvinogonawo kuonekwa mu idiopathic dystonia. Saka zvakaoma kuti vanachiremba vanzwisise uye vaone kuti chii chinonzi functional movement disorder. Chii chinonzi idiopathic disorder? Uye izvi zvichiri kuenderana neruzivo rwenyanzvi yezveuropi.

Pakutsanangurwa kwekiriniki, chirwere chinowanzoitika. Chinosiyanawo pakuongororwa kwechirwere kana tikaenzanisa kudengenyeka kana myoclonus kana chirwere chekufamba chinoshanda umo kupinda mune zvimwe zviratidzo zvekiriniki [00:03:00] zvinogona kutibatsira kusiyanisa zvinhu izvi.

Dr. Michele Matarazzo: Saka waitaura nezvesainzi yatinowanzo shandisa patinoongorora murwere ane dystonia inofungidzirwa kuti inoshanda, asi sezvawaitaura pane zvakawanda zvinoenderana ne idiopathic dystonia chaiyo. Uye izvozvo zvinoita kuti zviome zvikuru. Uye ndinofunga kuti idambudzikowo kana uchida kubatsira varwere nekuti zvechokwadi kutarisira murwere ane dystonia inoshanda uye ini diopathic dystonia zvakasiyana. Uye kunyangwe pakurapa kwepamusoro, zvinoita mutsauko mukuru. Zvino, waive nenzira yakasiyana zvikuru, iyo iri yekutanga kushandisa polyelectromyography ine protocol yepropofol sedation kuti uone kuti mashandiro emhasuru anoita sei mumatanho akasiyana ekuziva.

Chii chaive fungidziro huru? Waitarisira kuti propofol yaizoratidza chii mu functional dystonia zvichienzaniswa ne idiopathic dystonia?[00:04:00] 

Purofesa Roberto Eleopra: Ehe. Uku ndiko kutanga kubva pakuongorora zvirwere zviviri zvakasiyana. Mu idiopathic dystonia, tiri kufunga kuti pane kusashanda zvakanaka kwe motor network inosanganisira basal ganglia, cerebellum, primary secondary somatosensory cortex. Asi kunewo humbowo hunobatana hunoratidza kuti mu functional dystonia pane kusashanda zvakanaka kwe cortical caudal rostral pasina kufarira kwechokwadi basal ganglia uye network ye deeper neurological center.

Uye izvi zvakakosha nekuti kana uchishandisa mushonga unonyaradza marwadzo kana uchida kuongorora murwere panguva yekurara, pane chimwe chinhu chakasiyana. Kune mamwe mashoko. Kune bhuku rekare umo, semuenzaniso, panguva yekurara uye idiopathic dystonia pane kudzikira kwe [00:05:00] kushanda kwemhasuru pawakarekodhwa ne poly-EMG.

Asiwo pakugadziriswa kwemuviri, pane kudzikira kwemamiriro emuviri wekumuka. Kune kudzikira kwekushanda kwetsandanyama. Izvi hazvisi zvechokwadi kuvarwere vese. Asi zvinogona kunge zviri izvo chinangwa, kunyanya kufunga nezvekuongorora panguva yekunzwa kusuruvara, uchishandisa nzira yekunzwa kusuruvara.

Uye mune zvepfungwa, kunewo mamwe maitiro ekurapa akavakirwa pakushandiswa kwerunako umo mune kudzidziswa kwekuzorodza kana kugadzirisa apo mumwe murwere wepfungwa anogona kuratidza kuvandudzika mune zvehutano. Saka zvakakosha kunzwisisa kuti kana tikakwanisa kuongorora mashandiro emhasuru semhedzisiro yekupedzisira yemotor [00:06:00] system panguva dzakasiyana dzekumuka, zvinogona kuitika.

Iyi ndiyo fungidziro yechidzidzo chedu yekusiyanisa mashandiro emuviri kubva kuidiopathic dystonia.

Dr. Michele Matarazzo: Zvakanaka. Saka, pfungwa yacho ndeyekuti pathophysiologically, idiopathic uye functional dystonia dzine topography yakasiyana muuropi zvichivhara zvikamu zvakasiyana zveuropi, izvo zvinoitika mumamiriro akasiyana ekunyaradza, zvicharatidza misiyano muongororo ye poly-EMG. 

Purofesa Roberto Eleopra: Ndizvo chaizvo. Chinangwa chekudzidza kwedu, chaive chekuona zviratidzo zve neurophysiological, chakakwanisa kuvandudza musiyano wekuongorora pakati pe functional ne idiopathic. Nekuti tinoziva kuti chikamu chekuongorora cheGupta Lang criteria ndechei. Pane zvimwe zvinhu zvisipo uye zvimwe zvisipo patinoda kuziva idiopathic chaiyo [00:07:00] dystonia.

Saka kana tikasarudza kudzidza murwere ane dystonic idiopathic 17 akanyatsotsanangurwa tichienzanisa nemurwere ane dystonic functional clearly functional murwere gumi zvichibva pane zvinodiwa naGupta Lang. Muchiitiko ichi tinoenzanisa uye tinoenderana nezera, murume kana mukadzi, dzidzo, maitiro ekupararira kwe dystonic, nzira yekutanga, kuvapo kana kusavapo kwechimiro chakagadzika, nguva yechirwere, uye zvakare mhinduro ye botulinum toxin.

Uye tinoedzawo kuenzanisa zviratidzo zvemanzwiro nekuzvidya mwoyo, uye tinoda kuongorora zvinoitika kana ukashandisa propofol senzira yakanaka uye inopedza simba rekudzivirira muviri. Tinoziva kuti propofol ibasa rinowanzoitika pa cortical layers, pa [00:08:00] cortical network, uye maererano nedzidziso, kana dystonic iri idiopathic dystonic, mashandiro makuru ari pasi pecortical mu basal ganglia uye kubatanidzwa neiyi somatosensory uye motor.

Uye zvinogona kunge zvisina simba guru pane functional dystonia kana nzira yekurapa chirwere ichi yakajairika mu cortex.

Dr. Michele Matarazzo: Zvakanaka, saka ndanzwisisa pfungwa yedzidziso uye nzira dzakawanda dzekuita, asi ndinofunga kuti vanhu vazhinji vaiverenga chinyorwa chino vaifunga nezvekuti nzira yese yakaoma sei. Zvinokwanisika sei muhupenyu chaihwo? Nzira yese yakatora nguva yakareba sei?

Uye unoda zvinhu zvingani kuti uzviite? Zvakaoma sei?

Purofesa Roberto Eleopra: Ehe, nzira iyi haina kuoma zvakanyanya nekuti ndiyo nzira imwe chete yatinoshandisa patinoisa MRI pasi peanesthesia. Saka kuisirwa kwepropofol hakupfuuri maminetsi makumi maviri. Tine nguva iri pakati pemaminitsi gumi nemana nemakumi maviri nemasere mumurwere wese anoshivirira, saka murwere akachengeteka, uye tinotanga nedosi shoma yepropofol, microgram imwe chete yemL yemurwere wega wega uye haisi bolus.

Kuwedzera kwemaminetsi maviri, muchikamu chemaminitsi maviri kana kupfuura. Uye tinoona kubva pakuona kwekiriniki kuti kusiyana kwehana, asi tinoongororawo neEEG uye nzira dzekutanga dzinoshandiswa nenyanzvi yekurapisa kuti tinzwisise nzira yakadzama yekurapisa. Uye tinofanira kungonyora izvi nemaelectrode mumhasuru ane avhareji yemhasuru gumi.

Saka tine maminetsi makumi maviri ekugadzirira murwere. Maminetsi makumi maviri ekuita bvunzo kazhinji mumaminitsi makumi mana [00:10:00] tinopedzisa bvunzo uye tinoshandisa mukurapa kana murwere atanga kuisirwa mushonga weMRI neanonyaradza. Zvinobatsira zvikuru uye zvine hungwaru kuongorora kwete chete maitiro eEMG, asiwo mamiriro ekurapa uye zvakaitika kune maitiro e dystonic.

Dr. Michele Matarazzo: Zvakanaka. Saka, zvinotora nguva asi zvinogoneka, zvakadaro.

Kunze kwekutsvaga, imaminitsi makumi mana, hachisi chinhu chaunogona kuita nevarwere vese. Nevarwere vakasarudzwa. Chinhu chinogoneka kana chikabatsira.

Zvino, ngationei kana zvichibatsira. Ndezvipi zvakabuda muongororo yenyu?

Purofesa Roberto Eleopra: Mhedzisiro huru inonakidza nekuti, zvechokwadi, hazvidikanwi kushandiswa muvarwere vakasarudzwa kana tichida kunzwisisa mibvunzo yakasiyana. Asi mhedzisiro ndeyekuti kana tisina simba, hongu, tine maitiro eEMG ekubatanidza, anoenderera mberi [00:11:00] kuputika, dzimwe nguva kuwanda kwekubatanidzwa mutsandanyama dzisingadikanwi.

Asi tinoona zvakafanana mukusagadzikana kwemafambiro ebasa. Pane kupindirana kwechokwadi, mune imwe kusagadzikana kwemafambiro ebasa pane musiyano mukutsvaga vanhu, asi izvi hazvina kukwana kusiyanisa rudzi urwu rwevanhu.

Dr. Michele Matarazzo: Ndingangokuvhiringidzai here kwesekondi imwe chete? Pamwe kune vamwe vateereri vasiri kufarira izvi, chii chiri, chii chiri co contraction uye chii chiri overflow yauri kutaura?

Purofesa Roberto Eleopra: Zvakanaka. Kudzikira kwe co-independent kunoitika kana tsandanyama dzinochinjika kana kuti dzinochinjika dzichiita kudzikama panguva imwe chete dzisiri dzemuviri. Patinochinjika chimwe chikamu chemuviri, pane kudzikira kwetsandanyama dzinochinjika uye kudziviswa kwetsandanyama dzinopesana. Muchiitiko ichi, mu dystonic, chimwe chezvinhu [00:12:00] chinodiwa ne neurophysiology pakuongororwa ikudzikira kwe co-independent tsandanyama. Uye chimwe chinhu, patinodzokorora kufamba, chiitiko ichi chisingadiwi chinowedzera ne topographic, semuenzaniso, kubva ku distal kusvika ku proximal chikamu che links. Uye ichi chimwe chinhu chinowanzo shandiswa kune organic, kune idiopathic dystonia. Izvi hazviwanzoonekwa mu functional movement disorder, asi hazvisi zvechokwadi nguva dzose nekuti imwe functional movement disorder ine co-independent ...

Saka chii chinoitika kana tikaedza panguva yekuraramisa kwatisina kuona, hapana musiyano wakawanda. Varwere vese, vaiwanzoderedza mashandiro emhasuru. Asi mukusagadzikana kwekufamba [00:13:00], kana tiine nzira yekuraramisa yakadzika, pane mhinduro yakakwana mu100% yekurwadziwa kwemhasuru mu idiopathic dystonia mu53%, pane kuramba kwekurwadziwa kwemhasuru. Saka chinhu chekutanga icho dudziro yacho ndeyekuti kana aripo, inogona kunge iri idiopathic dystonia, asi chinonyanya kufadza kana tikamisa kuisirwa uye pane kupora mukudzoka zvishoma nezvishoma. Muvhiki imwe chete yemurwere, pane shanduko yakasiyana.

Tsandanyama dzisina simba dzinodzokazve nenzira imwecheteyo yekuzvidira muchimiro chimwe chete pakutanga hana isati yapora zvachose. Mukusagadzikana kwekufamba kwebasa, pane kunyarara kwenguva refu uye kana, [00:14:00] kazhinji panguva yekuonekwazve kwehana, maitiro acho anochinja kubva pakutanga.

Saka pfungwa mbiri idzi huru pakunyaradza muviri zvakanyanya uye panguva yekupora dzinobatsira zvikuru kusiyanisa rudzi urwu rwe dystonia, iyi dystonia idiopathic kune functional dystonia.

Dr. Michele Matarazzo: Izvi zvinoita sezvinobatsira zvikuru panyaya idzi dzisina chokwadi dzatinombotaura kuti dzimwe nguva, sezvamakataura, zvakaoma kusiyanisa functional dystonia neidiopathic dystonia uye ikozvino maratidza izvi muchidzidzo chino. Zviri pachena kuti izvi zvinonakidza, izvi zvitsva, asi munofunga kuti tinofanira kushandisa izvi mukiriniki here?

Tinoda humwe humbowo here kana kuti izvi zvakakwana?

Purofesa Roberto Eleopra: Ehe ndinobvumirana newe. Ichi chidzidzo chekuedza. Pane zvimwe zvipingamupinyi. Muganho wekutanga ndewehukuru hwemuenzaniso nekuti tine varwere gumi nevanomwe chete uye varwere gumi vane dystonia inoshanda, uye tinosarudzawo murwere [00:15:00] ane chirwere ichi. Asi kubva pakuona kwekiriniki, zvaive zvechokwadi, asi kana tiri vamwe vanhu vasina kujeka, tinofanira kusimbisa bvunzo iyi nekuti pamwe inoita seinobatsira kana paine chikonzero chekuti hatikwanise kusiyanisa chirwere chekufamba chinoshanda chakajeka kubva kudystonia idiopathic.

Chinhu chekutanga ndechekusimbisa sainzi iri nyore uye kusanganisira nyaya yakatsanangurwa uye yakajeka ye functional idiopathic dystonia. Uye zvakare tiri kuedza kuyera zviri nani EMG pattern nekuti ingori qualitative observation kusvika panguva ino. Ehe, tinoshandisa blinded neurophysiologist kuti tinzwisise kuti chii chinonzi EMG pattern pasina humbowo hwe functional, ye [00:16:00] idiopathic dystonia, asi tinofanira kusimbisa kubva kune dzimwe quantitative measurements.

Dr. Michele Matarazzo: Uye chimwe chinhu chaindifadza pandaiverenga bepa iri ndechekuti hauna kungoshandisa EMG chete, asiwo kushandisa passive manipulation panguva iyi yekunyaradza muviri zvakanyanya. Saka izvi ndizvo zviri kuitika pakiriniki. Ungatiudze zvishoma zvakaitika nekushandisa passive manipulation here?

Purofesa Roberto Eleopra: Ehe, izvi zvakakosha kune vanachiremba nekuti kana uine chimiro chakagadzikana, dzimwe nguva hatikwanise kunzwisisa kana chiri chimiro chechokwadi che dystonic kana kuti pane kudzokera shure kubva pakunyorova kana kuchinja kwemapfupa emapfupa. Saka kana murwere ari pasi pekurara zvakanyanya, tinokwanisa kuva nekufamba kwechikamu chemusoro pasina kupindira.

Uye zviri nyore kuti tinzwisise, semuenzaniso, kana murwere ane chimiro chakagadzika [00:17:00] angabatsirwa nebotulinum kana neDBS kana kubva kune imwe nzira yekurapa nekuti kana paine kubviswa kwemapfupa kubva kune imwe osteoarthritis, izvi zvinokanganisa kuvandudzwa kwechimiro.

Dr. Michele Matarazzo: Zvakakwana. Tava kusvika kumagumo ebvunzurudzo iyi. Ndaida kubvunza sezvamataura kare kuti iyi iongororo yekutanga. Pane zvipingamupinyi zvishoma, chimwe chazvo chiri muhukuru hwemuenzaniso. Saka mati tatofanira kusimbisa izvi mumapoka makuru. Muri kushanda pane izvi here kana kuti munotarisira kuti mumwe munhu azviite?

Purofesa Roberto Eleopra: Tiri kushanda pane izvi. Tiri kuvandudza saizi yemuenzaniso uye tiri kusanganisira makiyi asina chokwadi kuti tinzwisise zvakaitika. Uye kunyanya, tiri kuedzawo kunzwisisa kana paine musiyano pakati pechimiro chemajini cheidiopathic dystonia uye [00:18:00] chimiro chisiri chemajini.

Nekuti tinoziva, semuenzaniso, nekurekodha mavara madiki panguva yeDBS, zvinogona kuitika kuona patani yakasiyana ye electrophysiological mumajini uye mune imwe mhando yakasiyana kana primary dystonia, isiri yemajini. Saka izvi ndizvo zvatiri kuita izvozvi kuti tisimbise, kuti tivandudze data redu.

Dr. Michele Matarazzo: Roberto, ndatenda. Ndatenda zvikuru nekubatana nesu. Makorokoto nekudzidza kwenyu kwamuri pamwe chete nevamwe vanyori venyu. Ichi chidzidzo chekutanga chinomutsa pfungwa. Ndatenda.

Purofesa Roberto Eleopra: Ndatenda nekundikoka pamwe nevanhu vese vanoteerera kuti muri pano nesu.

Dr. Michele Matarazzo: Uye kune vateereri vedu, tanga tichikurukura nezvepepa rakaburitswa muMovement Disorders Journal rine musoro unoti Poly Electromyography Under Propofol to Differentiate Functional From Idiopathic Dystonia: A Pilot Study. Tinokutendai mose nekuteerera. [00:19:00] [00:20:00]