| Abstract | Cilj istraživanja: Cilj ovog istraživanja bio je prikazati kliničku sliku, incidenciju i preživljenje pacijenata sa bolesti motornog neurona hospitaliziranih u Klinici za neurologiju KBC Split u razdoblju od 1.siječnja 2008. do 31. prosinca 2017. godine te njihove demografske karakteristike.
Materijali i metode: Provedeno je retrospektivno istraživanje koje je obuhvatilo 59 pacijenata hospitaliziranih u Klinici za neurologiju od 1. siječnja 2008. do 31. prosinca 2017. godine. Uvidom u protokol i arhivu povijesti bolesti te kontaktiranjem pacijenata i njihovih obitelji prikupljeni su sljedeći podatci o pacijentima: osnovne demografske karakteristike (dob i spol), simptomi i znakovi bolesti, vrsta neurološkog deficita i dijagnoza bolesti te krajnji ishod bolesti. U statističkoj obradi korištene su metode deskriptivne statistike, t-test i Hi kvadrat (χ2 test) test.
Rezultati: Od ukupnog broja oboljelih bilo je 49,15% muškaraca i 50,85% žena. Nije pronađena statistički značajna razlika u obolijevanju od bolesti motornog neurona s obzirom na spol (χ2 test; P = 0,896). Medijan dobi u kojoj se postavljala dijagnoza iznosi 65 godina (raspon: 27-86 godina). Prosječna godišnja incidencija MND, neovisna o spolu, u promatranom razdoblju iznosila je 1,29 na 100 000 stanovnika Splitsko – dalmatinske županije, odnosno 0,66 na 100 000 stanovnika za ženski spol te 0,64 na 100 000 stanovnika za muški spol. U pogledu kliničke slike i dijagnoze bolesti, od ukupnog broja ispitanika, najveći broj pacijenata je imao ALS, njih 59,32%, 15,25% pacijenata imalo je kliničku sliku PBP, 13,57% pacijenata PMA, 10,14% pacijenata HSP te 1,69% pacijenata kliničku sliku PLS. Prosječno vrijeme od pojave prvih simptoma do hospitalizacije iznosilo je 8 mjeseci. Od ukupnog broja oboljelih od ALS-a bilo je 57,14% muškaraca i 42,86% žena, nije bila pronađena statistički značajna razlika među spolovima (χ2-test; P = 0,398). Kod ženskog spola srednja vrijednost dobi postavljanja dijagnoze bolesti iznosila je 65,60 ± 10,90 godina, dok je kod muškaraca srednja vrijednost dobi postavljanja dijagnoze bila nešto niža i iznosila je 60,75 ± 12,58 godina. Iako je razlika među spolovima iznosila 5 godina, nije bila statistički značajna (t-test, P = 0,241). Prosječno preživljenje od početka simptoma do smrti iznosilo je 29,2 mjeseca (95% CI 16,69-41,70). Svi pacijenti s kliničkom slikom PBP su bili ženskog spola, prosječne dobi 74 godine (raspon: 58-80 godina). Prosječno preživljenje od početka simptoma do smrti iznosilo je 14,25 mjeseca (95% CI 5,63-22,87). Od ukupnog broje oboljelih od PMA bilo je 75% muškaraca i 25% žena, nije bilo statistički značajne razlike među spolovima u ukupnom pobolu od PMA (χ2-test; P = 0,896). Prosječna dob pri kojoj je dijagnosticirana PMA iznosila je 64,5 godine (raspon: 31-77 godina). Prosječno preživljenje od početka simptoma do smrti iznosilo je 23,84 mjeseca (95% CI 9,09-38,58). Jedna 59-godišnja pacijentica imala je dijagnozu PLS te je na kraju ovog istraživanja bila živa sa ukupnim trajanjem simptoma od 8 godina i 11 mjeseci. Od ukupnog broja oboljelih od HSP bilo je 50% muškaraca i 50% žena. Prosječna dob pri kojoj je dijagnosticirana HSP je bila 49,5 godina (raspon: 27-62 godina).
Zaključak: Naše istraživanje je pokazalo da nema razlike po spolovima u pobolu od ALS-a, PMA i HSP, dok su PBP i PLS češći u žena. Prosječna dob oboljelih od ALS i PMA je jednaka i iznosila je 65 godina. PLS i HSP se češće javljaju u mlađoj životnoj dobi, a PBP u starijoj. Preživljenje je najduže kod oboljelih od PLS (8,92 godine), zatim kod oboljelih od ALS-a (29,2 mjeseci) i PMA (23,84 mjeseca), dok je najkraće kod oboljelih od PBP (14,25 mjeseci). Incidencija oboljelih od MND u Splitsko – dalmatinskoj županiji (0,66-1,98/100 000) i dalje je niža u odnosu na ostatak europske (1,47-2,43/100 000) i sjevernoameričke (1,75-2,02/100 000) populacije. |
| Abstract (english) | Objective: The aim of this study was to present the clinical picture, incidence and survival of motor neuron disease in patients hospitalized at the Department of Neurology, University Hospital of Split from 1st January 2008 to 31st December 2017 and their demographic characteristics.
Patients and methods: This retrospective study was conducted at the Department of Neurology, University Hospital of Split. 59 patients, with the diagnosis of MND, diagnosed during the period from 1st January 2008 to 31st December 2017,were included. The following patient data was collected from the protocol and archives of history of the disease: basic demographic characteristics (age and sex), symptoms and signs of the disease, types of neurological deficits and diagnosis of the disease, while the survival data was collected through a direct contact with patient or patient’s family. In the statistical analysis, the methods of descriptive statistics, the Chi squared (χ2 test) and t-tests were used.
Results: Out of the total number of patients with motor neuron disease, 49.15% of them were men and 50.85% were women. There wasn’t a statistically significant difference in the diagnosis of MND in regards to sex (χ2 test; P = 0.896). The median age of the diagnosis is 65 years (min-max: 27-86 years). The average annual incidence rate of MND during a ten-year period, regardless of sex, was 1.29 per 100 000 of the population in Split – Dalmatia county, i.e. 0.66 per 100 000 of the population for women and 0.64 per 100 000 of the population for men. In terms of the clinical picture and diagnosis of the disease, out of the total number of patients, the majority of patients had ALS - 59.32% of them, 15.25% of patients had clinical picture of PBP, 13.57% of patients had PMA, 10.14% of patients had HSP and 1.69% of patients had clinical picture of PLS. The average time between the appearance of the first symptoms and hospitalization was 8 months. Out of the total number of patients with ALS diagnosis, there were 57.14% of men and 42.86% of women; there was no statistically significant difference in regards to sex (χ2-test; P = 0.398). In the case of female patients, the mean age of diagnosing the disease was 65.60 ± 10.90 years, while the average age of the male sex was slightly lower, 60.75 ± 12.58 years. Although the difference between sexes was around 5 years, it wasn’t statistically significant (t-test; P = 0.241). The mean time of survival of patients with ALS from the beginning of the symptoms was 29.2 months (95% CI 16.69-41.70). All the patients with the diagnosis of PBP were women with the mean age of 74 years (min-max: 58-80 years). The mean time of survival from the beginning of symptoms was 14.25 months (95% CI 5.63-22.87). Out of the total number of patients with PMA, there were 75% of men and 25% of women. There wasn’t a statistically significant difference in the diagnosis of PMA regarding the sex (χ2-test; P = 0.896). The mean age of diagnosing the disease was 64.5 years (min-max: 31-77 years). The mean time of survival from the beginning of symptoms was 23.84 months (95% CI 9.09-38.58). There was one 59-year-old female patient with the diagnosis of PLS and at the end of this research she was still alive with the symptom duration of 8 years and 11 months in total. Out of the total number of patients with HSP, there were 50% of men and 50% of women. The mean age of diagnosing the disease was 49.5 years (min-max: 27-62 years).
Conclusion: Research has shown that there was no difference in the morbidity in regards to sex in patients with ALS, PMA and HSP, while PBP and PLS were more common in female sex. The mean age of patients with ALS and PMA was 65 years. PLS and HSP diseases were more common in a younger age, while PBP was more common in the older age. The patients with PLS had the longest mean time of survival (8.92 years), followed by the patients with ALS (29.2 months) and PMA (23.84 months), while the patients with PBP had the shortest time of survival (14.25 months). Incidence of the patients with MND in Split – Dalmatia county (0.66-1.98/100 000) is still lower than in the rest of the European (1.47-2.43/100 000) and North American (1.75-2.02/100 000) population. |
