Dystonia

Clinical Overview

Dystonia chirwere chekufamba chinoratidzwa nekugadzikana kana kupindirana kwemhasuru kusungirirana kunokonzera kusagadzikana, kazhinji kudzokorora, mafambiro, mamirire, kana zvese zviri zviviri. Dystonic mafambiro anowanzo kurongeka, kumonyoroka, uye anogona kunge achidedera. Dystonia inowanzotangwa kana kuipa nekuita kwekuzvidira uye inosanganiswa nekufashukira kwemhasuru activation.

Chiratidzo chemuviri che dystonia chinoratidzwa nekucherechedza kwe dystonic postures uye mafambiro uye nemamiriro ezvinhu anonyanya kushandura, kuwedzera kana kuderedza chiitiko ichi. Muenzaniso wakajairika ndewekuti kana tactile stimulus kana geste yekuzvidira (inozivikanwa senge sensory trick kana geste antagoniste), sekubata yakakanganisika kana yepedyo nhengo yemuviri, inogona kuderedza dystonia. Izvi zvinoonekwa mumhando dzakasiyana dze dystonia syndromes.

Iyo neural mechanism iri pasi pe dystonia inosanganisira matunhu mazhinji epakati tsinga system (kunyanya: the basal ganglia, cerebellum, nzvimbo dzekuwedzera mota uye sensorimotor cortex) zvinotungamira mukukanganisa inhibition, abnormal plasticity uye sensorimotor dysfunction. Matanho akasiyana ekukanganiswa kwekushanda anotungamira kune akasiyana madhigirii ekucherechedzwa kwekiriniki kuomarara.

Ona tsananguro yakazara

Zvimiro zvekiriniki zvedystonia zvinosanganisira: zera rekutanga (kubva paucheche kusvika pakukura), kugoverwa kwemuviri (focal mafomu anokanganisa chikamu chemuviri chimwe chete, segmental inokanganisa nhengo dzemuviri dzinosangana, mafomu akajairwa anosanganisira hunde uye mamwe matunhu maviri emuviri), uye chimiro chenguva (chirwere kosi static kana kufambira mberi uye musiyano une chekuita nezviito zvekuzvidira kana kushanduka kwezuva nezuva). Dystonia inogona kuparadzaniswa kana kusanganiswa neimwe chirwere chekufamba, senge parkinsonism kana myoclonus, kana inogona kubatanidzwa kune mamwe tsinga kana systemic kuratidzwa.

Isolated dystonia ine mavambo muhudiki inoita kufambira mberi kusvika kune generalization, nepo dystonia inomuka muhukuru inowanzoramba yakatarisana kana segmental. Mamwe mafocal/segmental dystonia syndromes ane mazita chaiwo, sezvaakamboonekwa sezvirwere zvakazvimiririra: blepharospasm, zvichikonzera kudzokorora kwekuvhara kwemaziso ane simba; spasmodic dysphonia inokanganisa kutaura; rurimi, kuvhura shaya uye kuvhara shaya kunonzi oromandibular dystonia; cervical dystonia kana torticollis inosanganisira mutsipa.

Focal dystonia inogonawo kunge yakanangana nebasa, semuenzaniso, cramp yemunyori dystonia inobata ruoko / ruoko panguva yekunyora. Mimwe mienzaniso yebasa rakananga dystonia inosanganisira muimbi dystonia inogona kukanganisa nhengo yemuviri inobatanidzwa mukuridza chiridzwa.

Pane zvikonzero zvakawanda zve dystonia. Iyo etiological classification inofunga mitsetse miviri mikuru: humbowo hwetsinga system pathology uye kana dystonia yakagara nhaka kana kuwanikwa. Muzviitiko zvakawanda zveiyo yoga dystonia hapana humbowo hwekuderera kana kukuvara kwechimiro (semuenzaniso, DYT1, DYT6; yaimbonzi "primary" dystonias). Zvikasadaro, panogona kunge paine humbowo hweiyo static structural lesion inokonzera dystonia, senge iri nyaya ye dystonic cerebral palsy inotevera perinatal uropi hwekukuvara, kana yekuderera kunowedzera (semuenzaniso, PKAN, PLAN; yaimbonzi "dystonia plus").

Dystonias inogarwa nhaka inogona kuva nemhando dzose dzekutakura maitiro; vakawana dystonias vanoziva zvakasiyana-siyana zvikonzero (perinatal uropi kukuvara, utachiona, zvinodhaka, chepfu, vascular, etc.); idiopathic dystonias (ingave ari sporadic kana emhuri) maitiro asina kunyorwa mazita, anoonekwa seane hukama neasati awanikwa majini.

Yakapiwa naProf. Alberto Albanese, MD
Purofesa weNeurology
Istituto Neurologico C. Besta
Milano, Italy

Mazano Anokosha

Kuongororwa & Kurapa

"Nhungamiro dzekuongororwa uye kurapwa kwekutanga (idiopathic) dystonia" mushumo neEFNS MDS-European Section Task Force.

Verenga bepa

kuongorora chirwere

International concensus bepa pa "Tsanangudzo uye Kurongeka kweDystonia."

Verenga bepa

Tools

Zvekiriniki maitiro uye tsvagiridzo mu dystonia

Kuongororwa Kwemakiriniki

Tapota mirai tichitora maresults enyu.

Chokwadi kune Varwere Vako

Ruzivo rwekutanga nezve nyaya dzekufamba kusagadzikana, izvo zvinoshandura mumitauro makumi mapfumbamwe+.

Dzidza ZVIMWE "

MDGene Database

Mhedziso yedata rakaburitswa pane causative gene mutations.
✪ MAHARA KUNE MEMBA E MDS

Dzidza ZVIMWE "

ruzivo

Tapota mirai tichitora maresults enyu.

Kuratidza peji 1 ye2

Enda kuPagination

Sarudzo dzeMhariri: Yakakurudzirwa Mapepa

Mapepa ereferenzi anobatsira nezve dystonia, akachengetwa neMDS Webhusaiti Edhiyo Board.

Cortical network zvigunwe zvigunwe zvinofanotaura zvakadzika uropi kukurudzira mhedzisiro mudystonia

Yenguva refu yekutevera yeDYT1 dystonia varwere vanobatwa nekudzika kwehuropi kukurudzira: yakavhurika-label kudzidza.

Genomewide association kudzidza mucervical dystonia inoratidza inogona kushamwaridzana ne sodium leak channel

Bilateral Pallidotomy yeDystonia: Ongororo Yakarongeka

Botulinum toxin jekiseni inoderedza associative plasticity kune varwere vane primary dystonia

Pallidotomy ye generalized dystonia

Mavhidhiyo ekudzidzisa: Ndoita sei?

Mavhidhiyo akasarudzwa kubva mumagazini yeMovement Disorders Clinical Practice :

mienzaniso: Vhidhiyo Nyaya

Bhurawuza mudura remakesi evhidhiyo anopfuura zviuru zviviri kubva kumajenari eMDS, kusanganisira akasiyana emharidzo yedystonia.
✪ NHENGO CHETE

Ona nyaya

dzidzo

Basic Coursework

Zvinonyanya kukosha:
Nzira yeDystonia

Nzwisisa tsananguro, hunhu hwekiriniki, zvikonzero zvechipiri, uye nzira dzakajairika dzekurapa dystonia, kusanganisira ruzivo rwechizvino-zvino pamusoro pezvinorapika dystonic kusagadzikana.

Tarisa kosi