BHUKU 29, CHIKAMU 4 • ZVITA 2025.
Myoclonus kufamba kamwe kamwe, kwenguva pfupi, kwakafanana nekuvhunduka, kusingatarisirwi kunokonzerwa nekumanikidzwa kwetsinga dzeropa kana kudzikira kwetsinga. Myoclonus yemuviri yakadai sekupindura kwekushamisika, kuvhunduka, uye myoclonus yekurara, inowanzoonekwa muvanhu vane hutano hwakanaka vasina kuremara kana kufambira mberi. Kuratidzwa kutsva kwemyoclonus kusingazivikanwe, uko kunogona kutsanangurwa se "benign idiopathic myoclonus" (BIM), kwakawanikwa.
Varwere vechidiki vane chirwere chepfungwa chekusvetuka-svetuka kwemyoclonic distal uye kunyunyuta kwekuzvibata kwemaoko avo, pasina kufambira mberi kwechirwere, vakaongororwa nekiriniki uye neuropi. Zvinodiwa zvekusavapo zvaive kuvapo kwezvimwe zviratidzo zvetsinga uye kushandiswa kwemishonga inozivikanwa kuti inokonzera myoclonus kureva, serotonin reuptake inhibitors, mishonga yekudzivirira epileptic inowedzera kutapurirana kweGABAergic, tricyclic antidepressants. Varwere gumi nevashanu (varume vana [26.7%]; zera rekutanga, makore 18.1±3.6; nguva yechirwere, makore 5.3±3.7) vakaongororwa.
Varwere vese havana kuratidza nhoroondo yemhuri yakanaka. Miedzo yekurabhoritari yakaongorora zvikonzero zvinorapika zve myoclonus (semuenzaniso, bvunzo dzeglucose, dzeitsvo, dzechiropa, uye dzethyroid) uye mimwe MRI yeuropi yakaitwa: Miedzo yese yakadzoka isina kunaka. Varwere vakaongororwa majini, kunyanya aisanganisira mapaneru ejini akavakirwa pawhole-exome sequencing (WES) nekuongorora kwakatevera majini akakodzera, nemhedzisiro isina kunaka. Kuongororwa kweuropi, kusanganisira EMG, somatosensory evoked potentials (SEPs), EEG-EMG witch back-averaging (BA), uye cortico-muscular coherence (CMC) kwakasimbisa kuongororwa kwekiriniki kwe myoclonus muvarwere vese. Varwere vese vaive neEMG burst duration ye <100 ms neavhareji burst duration ye63 ms (±13.7, range 39-95 ms), uye cortical correlate yakaratidzwa neBA kana CMC kana nekuvapo kwegiant SEPs muvarwere vatanhatu.
Zvakawanikwa izvi zvinoratidza kuti cortical myoclonus iri muvarwere vamwe asi kwete vese, zvichiratidza kuti BIM inogona kunge yakasiyana. Kutevera kwakaitwa pamakore 0.5-8 kwakaratidza mamiriro ezvinhu akagadzikana mukiriniki kana kuti kurerukirwa kwakazara muvarwere vese, kunze kwemumwe akataura nezvekufambira mberi kudiki.
Zvakabuda zvinoratidza kuti pane chimwe chinhu chitsva chemyoclonus chinogona kunzi "benign idiopathic myoclonus." Kufamba uku kusingafadzi kunonyanya kuoneka muvakadzi mumakore gumi echipiri, nekupararira kwemakumbo ekumusoro uye nzira isina njodzi muzviitiko zvakawanda pasina kurapwa nemishonga. Zvikonzero zvinogona kukonzera chirwere zvakabviswa uye hapana zvikonzero zvakawanikwa.
Kana nyanzvi yezvirwere zvekufamba ikaona kuti myoclonus iri pakati nepakati kana kuti iri pakati nepakati, pasina zvimwe zviratidzo zvetsinga, kunyanya mumakore gumi echipiri ehupenyu, BIM inofanira kufungwa nezvayo. Kuongororwa kwerabhoritari kunofanirwa kuitwa nguva dzose kuti pasave nezvikonzero zvinorapika uye kuteverwa kwekiriniki kunokurudzirwa. Sezvo nzira yechirwere ichi ichiita seyakanaka, pasina chikonzero chemishonga panguva yekutevera muzviitiko zvinenge zvese, kurapwa kunokurudzirwa chete pakunyunyuta kwevarwere nezvekupindira kwekufamba-famba kwakashata pamabasa ezuva nezuva. Uyezve, mukana wekuvandudzika kwega kwega kana kubviswa kwezvichemo zvachose unofanirwa kugoverwa nevarwere. Iyi phenotype inogona kumiririra boka diki re physiologic myoclonus, kuti risimbiswe mumapoka akawanda. Kucherechedzwa uku kutsva kunogona kujekesa mhando itsva ye myoclonus inoda kuongororwa kwakananga uye kwakaganhurirwa uye kurapwa.
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