J 2017

Okulárna myasténia gravis v Slovenskej republike

MARTINKA, Ivan, F. CIBULČÍK, Josef BEDNAŘÍK and P. ŠPALEK

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

Language

Czech

Type of outcome

Článek v odborném periodiku

Field of Study

30103 Neurosciences

Country of publisher

Czech Republic

Confidentiality degree

není předmětem státního či obchodního tajemství

Impact factor

Impact factor: 0.508

RIV identification code

RIV/00216224:14110/17:00098518

Organization unit

Faculty of Medicine

UT WoS

000399960900011

Keywords in English

myasthenia gravis; ocular myasthenia gravis; diagnosis; prognosis; generalization

Tags

Tags

International impact, Reviewed
Změněno: 3/12/2018 12:41, Soňa Böhmová

Abstract

V originále

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

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.