IQela loFundo lwe-Progressive Supranuclear Palsy (PSP).

Umgangatho wegolide wokuxilonga ukuxilongwa kwe-pathological (Hauw et al., Neurology. 1994; 44: 2015-9). Ukuxilongwa kweklinikhi kuhlala kungumngeni. I-National Institute of Neurological Disorders kunye ne-Stroke kunye ne-Society for PSP (NINDS-SPSP) iikhrayitheriya ziye zacetywa ukuxilongwa kwekliniki (Litvan et al., Neurology 1996; 46: 922-930). Ukuqinisekiswa kwezi migaqo kwiiseti ezizimeleyo zezigulane zibonise ixabiso eliphezulu eliqikelelweyo, nangona uvakalelo oluphantsi ngokukodwa ngexesha lekhosi yokuqala yesifo kunye neentetho zeklinikhi ezihlukeneyo (Osaki et al., Mov Disord. 2004; 19; 181-189; Respondek et al., Mov Disord. 2013 do. Ngokukodwa, iikhrayitheriya ze-NINDS-SPSP azizange zivumele ukuqatshelwa kweenkcazo ezichazwe kutshanje ze-phenotypic phenotypic PSP kunye nokubonakaliswa kweklinikhi kwasekuqaleni.

Inkalo ebaluleke kakhulu yi-diagnostic diagnostic vs. corticobasal degeneration (CBD).

Ngoko ke, iqela lesifundo se-MDS-PSP liseke iindlela ezintsha ze-MDS zokuxilongwa kwe-PSP ngokwesiseko sovavanyo olucwangcisiweyo lwamanqaku ophando ashicilelweyo angaphezu kwama-6000 kunye nentlanganiso egxile kwisivumelwano kunye neengcali zamazwe ngamazwe ezingama-40 (uHöglinger et al., zeQela leSifundo se-PSP elivunyiwe yiMovement Disorder Society. Ukuxilongwa kweKlinikhi kweProgressive Supranuclear Palsy - Iikhrayitheriya zeMovement Disorder Society. Iingxaki zeMovement, 2017;32(6):853-864.

Ukuqinisekiswa kweekhrayitheriya ze-MDS-PSP kwiimeko ezizimeleyo zeklinikhi-pathological kuyaqhubeka.

Ukuqinisekiswa kwentsebenziswano okulindelweyo kwenkqubo ye-MDS-PSP nako kuyaqhubeka (jonga injongo yesi-2).

Sijonge ukudala uthungelwano lophando lweklinikhi ukuphucula umgangatho wedatha yembali yendalo yeklinikhi, idatha ye-neuroimaging kunye ne-biomaterials. Ke ngoko, iinethiwekhi zophando lweklinikhi ziye zaqaliswa

• France (NS-PARK/FCRIN network PIs: Jean-Christoph Corvol, Olivier Rascol)

• EJamani (Chaza-PSP / iziFundo zeProPSP: PI: Günter Höglinger)
• I-Italiya (iRegistry ye-PSP yase-Italian: PIs: Paolo Barone / Angelo Antonini)
• Japan (JALPAC Study, PI: Takahiko Tokuda, Ikuko Aiba, Kenji Nakashima)
• UK (PROSPECT-UK Study, PI: Huw Morris)
• USA (4RTNI / ARTFL Studies, PI: Adam Boxer)

Siqhubeleka neenzame zethu zokungqinelanisa iiprothokholi zezifundo zeqela lesizwe ukwandisa iziphumo zenzululwazi. Kukho imfuneko ethile yokuvelisa idatha yembali yendalo yeendlela ezahlukeneyo zokubonakaliswa kwekliniki ye-PSP, njengoko iqhutywe ngoku okokuqala kwiikhrayitheriya ze-MDS-PSP, njengesiseko sezilingo zonyango ezizayo.

Iinketho zonyango ezikhoyo ngoku zilinganiselwe kungenelelo lweempawu kunye nokusebenza okuphantsi. Akukho ndlela yonyango yokhuselo okanye yonyango ekhoyo ngoku. Ke ngoko, sijonge ukuqalisa imilinganiselo yokuququzelela izilingo zonyango zonyango kwi-PSP.

Isayensi esisiseko iyaqhubeka nokuchonga iithagethi zeemolekyuli kunye nongenelelo oluhambelanayo. Ukuguqulelwa kulingo lweklinikhi ukuvavanya ukhuseleko kunye nokusebenza kwabo kusemva. Izizathu ke ngoko zilele ngokuyinxenye kuluhlu olunyiniweyo loyilo lophononongo olungqale kwi-PSP. Ngenxa yale njongo kufuneka siphuhlise kwaye sisebenzise amanqaku ezonyango kunye nezikali ukuze silinganise iimpawu ezijoliswe kwisifo esithile, kunye nokuchonga iimpawu ezihambelana nokunyuka kwesifo. Ukufunyanwa kwedatha yembali yendalo efunekayo ekubaleni amandla kunzima ngakumbi kwi-PSP ngenxa yobubanzi bayo bezonyango, ukungabikho kweenqobo zolwahlulo oluqhelekileyo olwamkelweyo lwezonyango, kunye nokwahlulwa ngokwejografi kuphando lwezonyango.

Iqela lophononongo lwe-MDS-PSP lithatha inxaxheba esebenzayo ekwandiseni indlela yolingo lwezonyango, umz

• Stamelou M, Schöpe J, Wagenpfeil S, del Ser T, Antonelou RCh, Oertel WH, Boxer AL, Höglinger GU. Ukubala kwamandla kunye ne-Placebo Effect Estimation for Clinical Trials kwi-Progressive Supranuclear Palsy. Ukuphazamiseka kweMovement, 2016; 31 (5): 742-7.
• Höglinger GU, Schöpe J, Stamelou M., Kassubek J., Del Ser T, Boxer AL, Wagenpfeil S, Huppertz HJ, Longitudinal MRI volumetry njengokufundwa kwezilingo zeklinikhi kwi-progressive supranuclear palsy: amanqaku amatsha adibeneyo. Iziphazamiso zokuhamba. 2017;32(6):842-852.

Amalungu eqela lophononongo lwe-MDS-PSP abonakalise ubuchwephesha bokuqhuba ngokuchubekileyo ulingo lwezonyango kwi-PSP, umz.:

• Stamelou M, Reuss A, Pilatus U, Magerkurth J, Niklowitz P, Eggert KM, Krisp A, Menke T, Schade-Brittinger C, Oertel WH, Höglinger GU. Iziphumo zexesha elifutshane ze-coenzyme Q10 kwi-progressive supranuclear palsy: uvavanyo olungahleliwe, olulawulwa yi-placebo. Ukuphazamiseka kweMovement, 2008; 23: 942-9.
• Tolosa E, Litvan I, Höglinger GU, Burn D, Lees A, Andrés MV, Gómez-Carrillo B, León T, Del Ser T; TAUROS Abaphandi. Ulingo lwesigaba sesi-2 se-GSK-3 inhibitor tideglusib kwi-progressive supranuclear palsy. Mov Disord. 2014 Epreli;29(4):470-8.
• Höglinger GU, Huppertz HJ, Wagenpfeil S, Andrés MV, Belloch V, León T, Del Ser T; TAUROS MRI Abaphandi. I-Tideglusib yehlisa ukuqhubela phambili kwe-atrophy yobuchopho kwi-progressive supranuclear palsy kulingo olungenamkhethe. Mov Disord. 2014 Epreli;29(4):479-87.
• I-Boxer AL, Lang AE, Grossman M, Knopman DS, Miller BL, Schneider LS, Doody RS, Lees A, Golbe LI, Williams DR, Corvol JC, Ludolph A, Burn D, Lorenzl S, Litvan I, Roberson ED, Höglinger GU, Koestler JCR M, Rando Dech, Van Dechr, Koestler JCR, van Deach IV, Heuer HW, Gozes I, Parker L, Whitaker S, Hirman J, Stewart AJ, Gold M, Morimoto BH; AL-108-231 Abaphandi. I-Davunetide kwizigulane ezine-progressive supranuclear palsy: i-randomised, i-double-blind, i-placebo-controlled controlled phase 2/3. I-Lancet Neurol. 2014 Jul;13(7):676-85.

Ngaloo ndlela, amalungu eqela lokufunda le-MDS-PSP abandakanyeka ngokukhutheleyo ekucwangciseni nasekuqhubeni ulingo lwesigaba II/III ngongenelelo olujoliswe kwi-tau.

Icandelo laseYurophu leqela lenze i-Continuing Medical Education Course 'iminyaka eyi-50 ye-Progressive Supranuclear Palsy' ngo-Oktobha 2014 eMunich. Ngenxa yomdla ophezulu kunye neempendulo ezilungileyo, sijonge ukulungiselela ikhosi yesibini ka-2019.

Iqela lijolise ekulungiseleleni isixhobo sewebhusayithi kunye nesifundo esisekelwe kwividiyo ekuphunyezweni okubanzi kwendlela entsha yokuxilonga i-MDS kwindlela yeklinikhi.

Isishwankathelo, iQela lokufunda le-MDS le-PSP liseke izifundo kunye namanyathelo okuphucula ulwazi, ukuxilongwa kwangaphambili kunye nonyango lwe-PSP kunye ne-CBD.