Kukurudzirwa kwehuropi kwakadzama muvana nevechiri kuyaruka vane ε-sarcoglycan myoclonus dystonia kunokonzera kuvandudzwa kwekushanda kwemuviri uye hupenyu hwakanaka.

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Chidzidzo ichi chinoongorora kushanda uye kuchengetedzeka kwe globus pallidus internus deep brain stimulation (GPi-DBS) muvana nevechiri kuyaruka vane SGCE-related myoclonus–dystonia (SGCE-MD). Parizvino hapana mishonga inochinja chirwere cheSGCE-MD, uye mishonga iripo inowanzopa mabhenefiti mashoma kana kukonzera migumisiro yakaipa. Kunyange zvazvo DBS yakasimbiswa zvakanaka muvakuru vane SGCE-MD, humbowo muvana huchiri hushoma, uye kuvhiyiwa kunowanzo miswa pasinei nekuremara kwekushanda kwakanaka muhudiki. 

nzira

Takaongorora varwere gumi vakaongororwa GPi-DBS pazera remakore 12.8 ± 3.4, uye avhareji yekutevera yakatevera makore 3.8 ± 2.4. Varwere vese vaiva nezviratidzo zvekusagona kurapwa. 

Zvakabuda mukushanda kwemuviri zvakaongororwa pachishandiswa zviyero zvakajairwa, zvinosanganisira Unified Myoclonus Rating Scale (UMRS), Burke-Fahn-Marsden Rating Scale (BFMRS), Writer's Cramp Rating Scale (WCRS), uye Gait Dystonia Rating Scale (GDRS). Hupenyu hwakaongororwa pachishandiswa zviyero zveNeuro-QoL, uye zvirwere zvepfungwa zvakaongororwa zvakarongeka nachiremba wepfungwa wevana. 

Kuti murwere wega wega ave nechokwadi chekuti murwere anowirirana uye kuderedza rusaruro rwevaongorori, murwere wega wega akaongororwa ari ega nemuongorori mukuru pamwe nenyanzvi isina ruzivo vachishandisa mavhidhiyo asina kurongeka asati avhiyiwa uye mushure mekuvhiyiwa. Zvibodzwa zvekupedzisira zvinomiririra avhareji yezvikamu zviviri zvakazvimiririra. 

Results

Mhedzisiro yemotokari:

Kuvandudzwa kwemota kwakasimba uye kwakasimba kwakaonekwa:  Myoclonus yakavandudzika neinenge 68% (UMRS-T).  Dystonia yese yakawedzera ne63% (BFMRS-T).  Dystonia yekunyora yakawedzera ne48% (WCRS).  Gait dystonia yakawedzera ne70% (GDRS-kufamba) uye kumhanya dystonia yakawedzera ne44% (GDRS-kumhanya).  Mabhenefiti ekurapa akaonekwa nekukurumidza (inenge mwedzi mitanhatu mushure mekuisirwa) uye akaramba akagadzikana mushure mekutevera kwenguva refu. Hafu yevarwere vakakwanisa kuderedza kana kurega mishonga yekunwa mushure mekuvhiyiwa.

 

Mhedzisiro isina chekuita nekufamba kwemuviri:

Zviratidzo zvepfungwa, zvinosanganisira kuzvidya mwoyo, maitiro ekumanikidzwa, uye matambudziko ekufunga, zvakaramba zvakagadzikana uye hazvina kuipa mushure meDBS. Kuyera hupenyu kwakaratidza kuvandudzika munzvimbo dzakadai sekuzvidya mwoyo, kuneta, uye kunyadziswa, pamwe nemafambiro akanaka mukushanda kwevanhu pamwe nepfungwa.

 

Kururama Kwekuvhiya, Zviyero Zvekukurudzira Uye Kuchengetedzeka:  

Kunyatsorongeka kwekuisa ma electrode kwaive kwakanyanya, neavhareji yekukanganisa kwekutarisa iri pasi pe 0.9 mm muma axes ese. Pakuongororwa kwekupedzisira, varwere vakakurudzirwa ne standard stimulation parameters (2.5 [1.8–3.5] milliamps, 64 [60–80] μs, 137.3 [120–200] Hz), zvichiratidzawo kururama kwemaitiro acho. 

Matambudziko ekuvhiyiwa akanga asingawanzoitiki uye achigona kugadziriswa, uye hapana zviitiko zvakakomba zvenguva refu zvakataurwa. 

Tora-kumba meseji

Chidzidzo ichi chinoratidza kuti DBS-GPi yakachengeteka, inoshanda uye ine basa guru muvana nevechiri kuyaruka vane SGCE-MD. 

Kukurudzirwa kwenguva refu kunounza kuvandudzika kwakakosha uye kunogara kwenguva refu mukushanda kwemuviri kwakakora uye kwakanaka. Kuvandudza zviratidzo zvemuviri pazera diki kunodzivirira migumisiro yenguva refu yemuviri, zvichigona kutungamira kumhedzisiro iri nani mudzidzo uye kukura kwehunyanzvi uye kwemagariro, uye hupenyu huri nani mukukura. 

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