Incidence of myasthenia and myasthenic crises in patients hospitalized at the Clinic for Neurology of KBC Split in the period from January 1st, 2019 to December 31st, 2021

Cilj istraživanja: Cilj ovog istraživanja bio je prikazati broj hospitaliziranih pacijenata s miastenijom gravis, broj novodijagnosticiranih pacijenata i broj miasteničkih kriza na Klinici za neurologiju KBC Split u razdoblju od 1. siječnja 2019. do 31. prosinca 2021. godine, te steći uvid u osnovne demografske i kliničke značajke tih pacijenata i usporediti dobivene rezultate s rezultatima iz literature. Materijali i metode: Provedeno je retrospektivno istraživanje koje je obuhvatilo 33 pacijenta hospitalizirana u Klinici za neurologiju od 1. siječnja 2019. godine do 31. prosinca 2021. godine. Uvidom u protokol i arhivu povijesti bolesti, te kontaktiranjem Odjela za laboratorijsku imunologiju KBC Zagreb i pacijenata ili članova njihovih obitelji prikupljeni su sljedeći podaci o pacijentima: osnovne demografske karakteristike (dob i spol), simptomi i znakovi bolesti, klinička slika i dijagnoza bolesti, serološki profil, predisponirajući čimbenici za nastanak miasteničke krize, pridružene neonkološke bolesti i novotvorine, primljena terapija i ishod bolničkog liječenja. U statističkoj su obradi korištene metode deskriptivne statistike i Hi kvadrat (χ2-test) test. Rezultati: Od 33 hospitalizirana pacijenta s dijagnozom od miastenije gravis, njih je 19 bilo novodijagnosticirano, a ostalih je 14 imalo pogoršanje ranije dijagnosticirane bolesti. Od ukupnog broja hospitaliziranih pacijenata, 57,6% bile su žene, a 42,4% bili su muškarci. U skupini novodijagnosticiranih pacijenata bilo je 52,63% žena i 47,37% muškaraca. Nije pronađena statistički značajna razlika između spolova ni u skupini svih bolesnika (P=0,384) niti u skupini novodijagnosticiranih (P=0,818). Prosječna dob postavljanja dijagnoze za ukupan broj pacijenata s MG-om iznosila je 57 godina, a za novodijagnosticirane pacijente 59 godina. Distribucija oboljenja za ženski spol bila je bimodalna, s vrhuncem oko 45. i 65. godine, dok je za muški spol bila unimodalna s vrhuncem incidencije oko 63. godine. U novodijagnosticiranih pacijenata s ranim oblikom bolesti, omjer žene:muškarci bio je 3:2, dok je kod kasnog oblika bolesti omjer muškarci:žene bio 4:3. Novodijagnosticiranih je pacijenata u 2019. godini bilo 63,16%, u 2021. godini 26,31% i u 2020. godini 10,53%. Razlika u učestalosti postavljanja dijagnoze između navedenih godina bila je statistički značajna (P=0,016). Ukupan broj miasteničkih kriza bio je 41, od toga ih je u 2021. godini bilo 39,02%, u 2019. godini 36,59% i u 2020. godini 24,39%. Najčešći predisponirajući čimbenik za nastanak miasteničke krize bila je akutna infekcija u 68,28% slučajeva, potom emocionalni stres u 21,43% slučajeva te fizički napor i ugriz krpelja u 7,14% slučajeva. Najviše je miasteničkih kriza zabilježeno u rujnu, njih 6, potom u siječnju, kolovozu i studenom po 5, u svibnju i listopadu po 4, u ožujku 3, u veljači, lipnju, srpnju i prosincu po 2 i u travnju po 1 miastenička kriza. Pozitivna protutijela na N-AChR imalo je 80,6% pacijenata, dok protutijela na MuSK nisu pronađena niti u jednog pacijenta. Od invazivnih terapija, plazmafereza je korištena u liječenju 39,02% miasteničkih kriza, intravenski imunoglobulini u liječenju njih 34,15%, kombinacija plazmafereze i imunoglobulina u liječenju njih 7,32% i mehanička ventilacija u liječenju njih 7,32%. Na ordiniranu je terapiju poboljšanje nastupilo u 38 slučajeva, stanje je ostalo nepromijenjeno u jednom slučaju, dok je smrtni ishod nastupio u dvoje pacijenata. Neonkološki je komorbiditet imalo 8 od 33 pacijenata (24,2%), a najčešće su bile bolesti štitnjače. Pridružene je novotvorine imalo 8 od 33 pacijenata (24,2%), a najčešći su bili timomi. Zaključak: Naše istraživanje nije pokazalo razliku u distribuciji pacijenata po spolu. Prosječna dob postavljanja dijagnoze MG-a za ukupan broj pacijenata iznosila je 57 godina. U skupini novodijagnosticiranih pacijenata, kod ranog oblika bolesti omjer oboljelih žena i muškaraca iznosio je 3:2, dok je kod kasnog oblika bolesti omjer muškaraca i žena iznosio 4:3. Ženski spol pokazuje bimodalnu distribuciju oboljenja s vrhuncima oko 45. i 65. godine, dok je za muški spol distribucija unimodalna s vrhuncem oko 63. godine.Objective: The goal of this research was to show the number of hospitalized patients with myasthenia gravis, the number of newly diagnosed patients and the number of myasthenic crises at the KBC Split Neurology Clinic in the period from January 1st, 2019 to December 31st, 2021, and to gain insight into the basic demographic and clinical features of these patients and compare the obtained results with the results from the literature. Subjects and methods: A retrospective study was conducted that included 33 patients hospitalized in the Clinic for Neurology from January 1, 2019 to December 31, 2021. By examining the protocol and archive of the medical history, and by contacting the Laboratory Immunology Department of KBC Zagreb and the patients or their family members, the following information about the patients were gathered: basic demographic characteristics (age and sex), symptoms and signs of the disease, clinical picture and diagnosis of the disease, serological profile, predisposing factors for the onset of myasthenic crisis, associated nononcological diseases and neoplasms, received therapy and the outcome of hospital treatment. Descriptive statistics and the Chi-square (χ2-test) test were used in statistical processing. Results: Out of 33 hospitalized patients with a diagnosis of myasthenia gravis, 19 of them were newly diagnosed, and the other 14 had a worsening of the previously diagnosed disease. Of the total number of hospitalized patients, 57.6% were women and 42.4% were men. In the group of newly diagnosed patients, 52.63% were women and 47.37% were men. No statistically significant difference between genders was found either in the group of all patients (P=0.384) or in the group of newly diagnosed patients (P=0.818). The average age of diagnosis for the total number of patients with MG was 57 years, and for newly diagnosed patients 59 years. The distribution of the disease for the female gender was bimodal, with a peak around the age of 45 and 65, while for the male gender it was unimodal with the peak incidence around the age of 63. In newly diagnosed patients with the early form of the disease, the female to male ratio was 3:2, while in the late form of the disease the male to female ratio was 4:3. There were 63.16% of newly diagnosed patients in 2019, 26.31% in 2021 and 10.53% in 2020. The difference in the frequency of diagnosis between the mentioned years was statistically significant (P=0.016). The total number of myasthenic crises was 41, of which there were 39.02% in 2021, 36.59% in 2019 and 24.39% in 2020. The most common predisposing factor for the onset of myasthenic crisis was acute infection in 68.28% of cases, followed by emotional stress in 21.43% of cases, and physical exertion and tick bite in 7.14% of cases. The most myasthenic crises were recorded in September; a total of 6, and then in January, August and November recording 5 each, in May and October with 4 each, in March there were 3, in February, June, July and December 2 each and in April 1 myasthenic crisis. 80.6% of patients had positive antibodies to N-AChR, while antibodies to MuSK were not found in any patient. Of the invasive therapies, plasmapheresis was used in 39.02% of myasthenic crises, intravenous immunoglobulins in the treatment of 34.15%, a combination of plasmapheresis and immunoglobulins in the treatment of 7.32% and mechanical ventilation in the treatment of 7.32%. The prescribed therapy improved in 38 cases, the condition remained unchanged in one case, and death occurred in two patients. 8 out of 33 patients (24.2%) had non-oncological comorbidities, while thyroid diseases were the most common. Associated neoplasms were present in 8 out of 33 patients (24.2%), and the most common were thymomas. Conclusion: Our research showed no difference in the distribution of patients by gender. The average age of diagnosis of MG for the total number of patients was 57 years. In the group of newly diagnosed patients, in the early form of the disease, the ratio of women to men was 3:2, while in the late form of the disease, the ratio of men to women was 4:3. The female gender shows a bimodal distribution of the disease with peaks around the age of 45 and 65, while for the male gender the distribution is unimodal with a peak around the age of 63