MARTINKA, Ivan, F. CIBULČÍK, Josef BEDNAŘÍK and P. ŠPALEK. Okulárna myasténia gravis v Slovenskej republike (Ocular Myasthenia Gravis in Slovak Republic). Česká a slovenská neurologie a neurochirurgie. Praha: Česká lékařská společnost J.E. Purkyně, 2017, vol. 80, No 2, p. 190-196. ISSN 1210-7859. Available from: https://dx.doi.org/10.14735/amcsnn2017190.
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Basic information
Original name Okulárna myasténia gravis v Slovenskej republike
Name (in English) Ocular Myasthenia Gravis in Slovak Republic
Authors MARTINKA, Ivan (703 Slovakia), F. CIBULČÍK (703 Slovakia), Josef BEDNAŘÍK (203 Czech Republic, guarantor, belonging to the institution) and P. ŠPALEK (703 Slovakia).
Edition Česká a slovenská neurologie a neurochirurgie, Praha, Česká lékařská společnost J.E. Purkyně, 2017, 1210-7859.
Other information
Original language Czech
Type of outcome Article in a journal
Field of Study 30103 Neurosciences
Country of publisher Czech Republic
Confidentiality degree is not subject to a state or trade secret
Impact factor Impact factor: 0.508
RIV identification code RIV/00216224:14110/17:00098518
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.14735/amcsnn2017190
UT WoS 000399960900011
Keywords in English myasthenia gravis; ocular myasthenia gravis; diagnosis; prognosis; generalization
Tags EL OK
Tags International impact, Reviewed
Changed by Changed by: Soňa Böhmová, učo 232884. Changed: 3/12/2018 12:41.
Abstract
Okulárna symptomatika je najčastejším prvým prejavom myasténie gravis (MG), pri prirodzenom priebehu dochádza obvykle k rýchlej generalizácii. O čisto okulárnej MG (OMG) hovoríme, ak izolovaná okulárna symptomatika pretrváva aj po 3 rokoch od vzniku MG. Cieľ: Prezentujeme retrospektívnu longitudinálnu štúdiu súboru 946 pacientov s iniciálnou okulárnou syrnptomatikou MG (oMG) evidovaných v slovenskom Centre pre neuromuskulárne ochorenia v rokoch 1978-2015. Cieľom práce bola analýza klinických údajov, laboratórnych nálezov a prognostických faktorov. Materiál a metodika: Metódou štúdia bol rozbor zdravotnej dokumentácie pacientov s oMG. Zisťovali sme vek pri vzniku oMG, pohlavie, titer auroprotilátok proti acetyicholínovým receptorom (AChR) a svalovo špecifickej kináze (MuSK). Hodnotili sme použité terapeutické postupy, klinický stav pri poslednej kontrole a prognózu. Výsledky: Okulárna Symptomatika bola iniciálnym príznakom MG u 946 pacientov (M = 487, 2 = 459). oMG generalizovala u 554 (58,6 %) pacientov. Ako lizikové faktory generalizácie oMG sme identifikovali mužské pohlavie (RR 1,23), vek nad 50 ro
Abstract (in English)
Background: Ocular symptoms are the most common initial symptoms of myasthenia gravis (MG) and they generalize rapidly in the majority of MG patients. In purely ocular MG (OMG), impairment of neuromuscular transmission remains limited to extraocular muscles even after three years from OMG onset. Aim: We performed retrospective longitudinal study in 946 MG patients with initial ocular symptoms (oMG) registered between 1978 and 2015 at the Slovak Centre for Neuromuscular Diseases. The aim of this study was to analyze clinical assessments, laboratory findings and prognostic factors in oMG. Material and methods: We analyzed data and findings in medical records of oMG patients including age at onset, sex, autoantibodies against acetylcholine receptors (AChRs) and muscle-specific kinase (MuSK), a type of clinical symptomatology. We evaluated the therapies used, clinical status at the last examination and prognosis in this initial clinical pattern. Results: Impairment of ocular muscles as the first MG sign occurred in 946 patients (M = 487, F = 459). oMG generalized in due course in 554 (58.6%) patients. We identified male gender (RR 1.23), age at onset of over 50 years (RR 1.23), association with other autoimmune diseases (RR 1.57), AChR - seropositivity (RR 2.09) and thymoma (RR 15.71) as the risk factors of oMG generalization. We identified female gender (RR 1.2), AChR - seronegativity (RR 1.58), oMG generalization (RR 1.63) and age at onset of below 50 years (RR 2.08) as predictive factors of unfavourable outcome (repeated exacerbations, unfavourable post-intervention status). Conclusion: To improve oMG prognosis, it is important to recognize the oMG patients with high risk of generalization and/or unfavourable outcome and to select appropriate therapy for these patients.
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