Dystonia

Isishwankathelo soNyango

I-Dystonia yintlupheko yentshukumo ebonakaliswa kukucutheka kwezihlunu okuzinzileyo okanye okwethutyana okubangela okungaqhelekanga, okuhlala kuphindaphindwa, ukunyakaza, ukuma, okanye zombini. Iintshukumo zeDystonic ziqhele ukwenziwa iipateni, zijije, kwaye zinokungcangcazela. I-Dystonia isoloko iqalwa okanye imbi ngakumbi ngesenzo sokuzithandela kwaye ihambelana nokuphuphuma kokusebenza kwemisipha.

Uphawu lomzimba lwe-dystonia luchongwa ngokuqwalaselwa kwe-dystonic postures kunye nokunyakaza kunye neemeko ezenza ngokukodwa, ukwandisa okanye ukunciphisa le phenomenology. Umzekelo oqhelekileyo xa i-tactile stimulus okanye i-geste yokuzithandela (eyaziwa ngokuba yi-sensory trick okanye i-geste antagoniste), njengokuchukumisa inxalenye echaphazelekayo okanye esecaleni yomzimba, inokunciphisa i-dystonia. Ezi mpawu zibonwa kwiindidi ze-dystonia syndromes.

Inkqubo ye-neural ephantsi kwe-dystonia ibandakanya imimandla emininzi yenkqubo ye-nervous central (ingakumbi: i-basal ganglia, i-cerebellum, iindawo zemoto ezongezelelweyo kunye ne-sensorimotor cortex) ekhokelela ekuthinteleni okungahambi kakuhle, iplastiki engaqhelekanga kunye nokungasebenzi kakuhle kwe-sensorimotor. Amanqanaba ahlukeneyo okonakala kokusebenza akhokelela kumanqanaba ahlukeneyo obuzaza beklinikhi.

Jonga inkcazo epheleleyo

Iimpawu zeklinikhi ze-dystonia ziquka: iminyaka ekuqaleni (ukususela ebuntwaneni ukuya ebudaleni obudala), ukuhanjiswa komzimba (iifom ezigxininisekileyo zichaphazela ilungu lomzimba elinye, icandelo elichaphazela amalungu omzimba adibeneyo, iifom eziqhelekileyo zibandakanya i-trunk kunye neminye imimandla yomzimba emibini), kunye nepateni yesikhashana (ikhosi yesifo i-static okanye inkqubela phambili kunye nokwahluka okunxulumene nezenzo zokuzithandela okanye ukuguquguquka kwamaxesha). I-Dystonia ingaba yodwa okanye idibaniswe nenye ingxaki yokunyakaza, njenge-parkinsonism okanye i-myoclonus, okanye inokudibaniswa nezinye iimpawu ze-neurological okanye ze-systemic.

I-dystonia ekwanti eqala ebuntwaneni idla ngokuqhubela phambili ukuya kwi-generalization, ngelixa i-dystonia evela ebudaleni ihlala igxile okanye iyinxalenye. Ezinye ii-syndromes ze-dystonia ze-focal / zegmental zinamagama athile, njengoko ekuqaleni zazijongwa njengezifo ezizimeleyo: i-blepharospasm, okubangelwa ukuphindaphinda ukuvalwa kweenkophe ezinamandla; i-spasmodic dysphonia echaphazela intetho; ulwimi, ukuvulwa kwemihlathi kunye nokuvalwa kwemihlathi okubizwa ngokuba yi-oromandibular dystonia; i-cervical dystonia okanye i-torticollis ebandakanya intamo.

I-dystonia ye-focal inokuthi ibe ngumsebenzi othile, umzekelo, i-cramp yombhali yi-dystonia echaphazela isandla / ingalo ngexesha lokubhala. Eminye imizekelo ye-dystonia ethile yomsebenzi ibandakanya i-dystonia yomculi enokuchaphazela inxalenye yomzimba echaphazelekayo ekudlaleni isixhobo.

Kukho izizathu ezininzi ze-dystonia. Ulwahlulo lwe-etiological luqwalasela imigca emibini ephambili: ubungqina be-nervous system pathology kunye nokuba i-dystonia izuzwe njengefa okanye ifunyenwe. Kwiimeko ezininzi ze-dystonia ezizimeleyo akukho bungqina bokuwohloka okanye i-structural lesion (umzekelo, i-DYT1, i-DYT6; eyayibizwa ngokuba yi-"primary" dystonias). Kungenjalo, kunokubakho ubungqina besilonda esingatshintshiyo esibangela i-dystonia, njengakwimeko ye-dystonic cerebral palsy elandela ukwenzakala kwengqondo ye-perinatal, okanye ukuwohloka okuqhubekayo (umzekelo, i-PKAN, PLAN; eyayibizwa ngokuba yi-"dystonia plus").

I-dystonias ezuzwe njengefa ingaba nazo zonke iintlobo zeepateni zosulelo; i-dystonias efunyenweyo iqaphela izizathu ezahlukeneyo (ukulimala kwengqondo ye-perinatal, usulelo, iziyobisi, ityhefu, i-vascular, njl.); idiopathic dystonias (nokuba ayiqhelekanga okanye yeyosapho) ziindlela ezingahlelwanga ngokwemfuza, zijongwa njengezinxulumene nemfuza engekafunyanwa.

Ixhaswe nguProf Alberto Albanese, MD
Unjingalwazi weNeurology
Istituto Neurologico C. Besta
IMilano, eItali

Iingcebiso eziphambili

Ukuchonga nokunyanga

"Izikhokelo zokuxilongwa kunye nonyango lwe-dystonia yokuqala (idiopathic)" ingxelo yi-EFNS MDS-European Section Task Force

Funda iphepha

Ukuqondwa

Iphepha lemvumelwano yamazwe ngamazwe "Inkcazo kunye noHlelo lweDystonia."

Funda iphepha

izixhobo

Ukwenziwa kweklinikhi kunye nophando kwi-dystonia

Uvavanyo lweziphumo zoNyango

Nceda ulinde ngelixa siqokelela iziphumo zakho.

Iinyani kwizigulane zakho

Ulwazi olusisiseko malunga nezihloko zokuphazamiseka kwentshukumo, eziguqulela kwiilwimi ezingama-90+.

Funda nzulu "

MDGene Database

Isishwankathelo sedatha epapashiweyo malunga neenguqu zemfuza ezibangelayo.
✪ SIMAHLA KUMALUNGU E-MDS

Funda nzulu "

ulwazi

Nceda ulinde ngelixa siqokelela iziphumo zakho.

Ibonisa iphepha loku-1 lama-2

Tsibela kwiPagination

Ukhetho loMhleli: Amaphepha acetyiswayo

Amaphepha ereferensi aluncedo malunga ne-dystonia, egcinwe yiBhodi yoHlelo lweWebhusayithi ye-MDS.

Iminwe yeminwe yeCortical network iqikelela iziphumo ezinzulu zokuvuselela ingqondo kwi-dystonia

Ulandelelwano lwexesha elide lwezigulane ze-DYT1 ze-dystonia zinyangwa kukuvuselelwa kwengqondo enzulu: Uphononongo oluvulelekileyo.

Uphononongo lombutho we-Genomewide kwi-dystonia yomlomo wesibeleko lubonisa unxulumano olunokwenzeka kunye nejelo lokuvuza kwe-sodium

I-Bilateral Pallidotomy ye-Dystonia: Ukuphononongwa okuCwangcisiweyo

Iinaliti ze-toxin ye-botulinum zinciphisa i-plasticity ye-associative kwizigulane ezine-primary dystonia

I-Pallidotomy ye-dystonia eqhelekileyo

Iividiyo ezifundisayo: Ndenza Njani?

Iividiyo ezikhethiweyo ezivela kwijenali yeMovement Disorders Clinical Practice :

imizekelo: Amatyala eVidiyo

Bhrawuza indawo yogcino lwamatyala angaphezu kwama-2,000 evidiyo asuka kwiijenali ze-MDS, kuquka neentetho ezahlukeneyo ze-dystonia.
✪ AMALUNGU KUPHELA

Jonga iimeko

Education

Umsebenzi weSifundo osisiseko

Izinto ezisisiseko:
Indlela yeDystonia

Qonda inkcazo, iimpawu zeklinikhi, oonobangela besibini, kunye neendlela eziqhelekileyo zonyango ze-dystonia, kubandakanywa nolwazi lwangoku malunga nokuphazamiseka kwedystonic enyangekayo.

Jonga ikhosi