Background: To observe the short-term outcome of plasma adsorption PA therapy in amyotrophic lateral sclerosis (ALS).
Methods: 28 cases of als patients were recruited in this study, of which 20 were male and 8 were female with a mean age of 53.21±9.07 years and the average course of 33±23.35 months. The clinical manifestations were limb weakness (N=27), muscular atrophy (N=27), muscular tremor (N=5), dysphagia (N=12) and dysarthria (N=12). The clinical data of the patients recruited were graded by Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALSFRSR) : <10 (N=1), 11-20 (N=4), 21-30 (N=6), 31-40 (N=12), >40 (N=5). All patients received PA therapy once a week for three successive times after examining the conditions of blood coagulation and virus infection. PA therapy was supplemented with neurotrophic therapy meanwhile. All patients' clinical manifestations and scores of ALSFRSR before treatment and one week after treatment were evaluated and compared. The levels of serum superoxide dismutase (SOD), interleukin-10 (IL-10), serum creatine kinase (CK) and lactate dehydrogenase (LDH) before and after treatment were compared.
Results: After PA therapy, 14 patients have improved obviously in muscle strength, 4 patients in hypermyotonia partially, 3 patients in muscular tremor, 5 patients in dysarthria, 3 patients in salivation to some extent and 2 patients in swallowing function. The score of ALSFRSR after PA treatment (31.89±10.36) was remarkably higher than that before PA treatment (30.68±10.52) (P<0.01). The levels of SOD (155.10±21.87 IU/L) and IL-10 (138.06±185.88 pg/mL) after PA treatment were significantly higher than the levels before PA treatment (143.08.3±19.16 IU/L and 46.34±75.31 pg/mL, respectively) (P<0.05). The levels of CK (168.86±113.50 IU/L) and LDH (152.07±32.65 IU/L) after PA treatment were significantly lower than the levels before PA treatment (356.68±250.30 IU/L and 181.36±33.74 IU/L respectively) (P<0.01). At the end of follow-up period (November, 2019), five patients died of respiratory failure 16-21 months after PA treatment and two patents died of respiratory infection 15-20 months after PA treatment. 7 patients were still alive. The score of ALSFRS-R of these patients who survived at the end of follow-up (13.00±13.37) were significantly lower than before PA treatment (36.71±8.56) (P<0.05) and after PA treatment (38.14±8.82) (P<0.05).
Conclusions: Plasma adsorption (PA) therapy has shortterm therapeutic effects on als. The effects might be attributed to the anti-oxygen free radical effect by increasing SOD level and the anti-inflammation effect by increasing IL-10 level. As the efficacy of PA therapy was obtained in a small sample size and short follow-up period, the longterm observation of PA efficacy in treating als should be further investigated.
Uvod: Cilj je bio da se posmatra kratkoročni ishod terapije adsorpcije plazme (PA) kod amiotrofične lateralne skleroze (ALS).
Metode: U ovoj studiji je regrutovano 28 slučajeva obolelih od als, od kojih su 20 bili muškarci i 8 žene sa prosečnom starošću od 53,21±9,07 godina i prosečnim tokom 33±23,35 meseci. Kliničke manifestacije bile su slabost udova (N=27), mišićna atrofija (N=27), mišićni tremor (N=5), disfagija (N=12) i dizartrija (N=12). Klinički podaci regrutovanih pacijenata su ocenjeni revidiranom funkcionalnom skalom amiotrofične lateralne skleroze (ALSFRSR): <10 (N=1), 11-20 (N=4), 21-30 (N=6), 31-40 (N=12), >40 (N=5). Svi pacijenti su primali PA terapiju jednom nedeljno tri puta uzastopno nakon ispitivanja stanja koagulacije krvi i virusne infekcije. PA terapija je u međuvremenu dopunjena neurotrofnom terapijom. Procenjene su i upoređene kliničke manifestacije svih pacijenata i rezultati ALSFRSR pre tretmana i nedelju dana nakon tretmana. Upoređeni su nivoi serumske superoksid dismutaze (SOD), interleukina-10 (IL-10), serumske kreatin kinaze (CK) i laktat dehidrogenaze (LDH) pre i posle tretmana.
Rezultati: Nakon terapije PA, kod 14 pacijenata je došlo do očiglednog poboljšanja mišićne snage, 4 pacijenta u delimičnoj hipermiotoniji, 3 pacijenta u mišićnom tremoru, 5 pacijenata u dizartriji, 3 pacijenta u delimičnoj salivaciji i 2 pacijenta u funkciji gutanja. Skor ALSFRSR posle tretmana PA (31,89±10,36) bio je značajno viši od onog pre tretmana PA (30,68±10,52) (P 0,01). Nivoi SOD (155,10±21,87 IU/L) i IL-10 (138,06±185,88 pg/mL) nakon tretmana PA su bili značajno viši od nivoa pre tretmana PA (143,08,3±19,16 IU/L i 46,34±75.31 pg/mL, respektivno) (P 0,05). Nivoi CK (168,86±113,50 IU/L) i LDH (152,07±32,65 IU/L) nakon tretmana PA bili su značajno niži od nivoa pre tretmana PA (356,68±250,30 IU/L i 181,36±32,65 IU/L) respektivno. (P 0,01). Na kraju perioda praćenja (novembar 2019.), pet pacijenata je umrlo od respiratorne insuficijencije 16-21 mesec nakon tretmana PA, a dva pacijenta su umrla od respiratorne infekcije 15-20 meseci nakon tretmana PA. 7 pacijenata je još uvek bilo živo. Skor ALSFRS-R ovih pacijenata koji su preživeli na kraju praćenja (13,00±13,37) bio je značajno niži nego pre tretmana PA (36,71±8,56) (P<0,05) i posle PA tretmana (38,14±8,82) ( P<0,05).
Zaključak: Terapija adsorpcijom plazme (PA) ima kratkoročne terapijske efekte na als. Efekti se mogu pripisati dejstvu slobodnih radikala protiv kiseonika povećanjem nivoa SOD i anti-inflamatornom efektu povećanjem nivoa IL-10. Kako je efikasnost PA terapije dobijena u maloj veličini uzorka i kratkom periodu praćenja, dugotrajno posmatranje efikasnosti PA u lečenju als-a treba dalje istražiti.
Keywords: amyotrophic lateral sclerosis; plasma adsorption; therapeutic effect.
2023 Bin Li, Wei Zhang, Shaoxin Zhong, Jianyi Pan, Xiaohong Wang, Hequn Zou, Xianrui Dou, published by CEON/CEES.