Short-term Outcome of Plasma Adsorption Therapy in Amyotrophic Lateral Sclerosis
Plasma Adsorption Therapy in Amyotrophic Lateral Sclerosis
Abstract
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 u/ml) 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 u/ml and 46.34±75.31 pg/ml, respectively) (P<0.05). The levels of CK (168.86±113.50 u/L) and LDH (152.07±32.65 u/L) after PA treatment were significantly lower than the levels before PA treatment (356.68±250.30 u/L and 181.36±33.74 u/L respectively) (P<0.01). At the end of follow-up period (November, 2016), five patients died of respiratory failure 16-21 months after PA treatment and two patents died from respiratory infections 15-20 months after PA treatment. 7 patients were still alive. The scores of ALSFRS-R of these patients were 13.00±13.37, obviously lower than those before PA treatment (36.71±8.56) (P<0.05) and 4 week after PA treatment (38.14±8.82) (P<0.05).
Conclusions: Plasma adsorption (PA) therapy has short-term 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 long-term observation of PA efficacy in treating ALS should be further investigated.
References
2. Yamada C, Pham HP, Wu Y, Cooling L, Kim HC, Morgan S, et al. Report of the ASFA apheresis registry on muscle specific kinase antibody positive myasthenia gravis. J Clin Apher 2017; 32(1): 5-11.
3. Kaya E, Keklik M, Sencan M, Yilmaz M, Keskin A, Kiki I, et al. Therapeutic plasma exchange in patients with neurological diseases: multicenter retrospective analysis. Transfus Apher Sci 2013; 48(3): 349-52.
4. Hardiman O, van den Berg LH, Kiernan MC. Clinical diagnosis and management of amyotrophic lateral sclerosis. Nat Rev Neurol 2011; 7(11): 639-49.
5. Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, et al. The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci 1999; 169(1-2): 13-21.
6. Carri MT, Valle C, Bozzo F, Cozzolino M. Oxidative stress and mitochondrial damage: importance in non-SOD1 ALS. Front Cell Neurosci 2015; 9: 41.
7. Drechsel DA, Estevez AG, Barbeito L, Beckman JS. Nitric oxide-mediated oxidative damage and the progressive demise of motor neurons in ALS. Neurotox Res 2012; 22(4): 251-64.
8. Tzartos JS, Zisimopoulou P, Rentzos M, Karandreas N, Zouvelou V, Evangelakou P, et al. LRP4 antibodies in serum and CSF from amyotrophic lateral sclerosis patients. Ann Clin Transl Neurol 2014; 1(2): 80-7.
9. McLean JR, Smith GA, Rocha EM, Osborn TM, Dib S, Hayes MA, et al. ALS-associated peripherin spliced transcripts form distinct protein inclusions that are neuroprotective against oxidative stress. Exp Neurol 2014; 261: 217-29.
10. Svetoni F, Caporossi D, Paronetto MP. Oxidative stress affects FET proteins localization and alternative pre-mRNA processing in cellular models of ALS. Free Radic Biol Med 2014; 75 Suppl 1: S51.
11. Shibata N, Nagai R, Miyata S, Jono T, Horiuchi S, Hirano A, et al. Nonoxidative protein glycation is implicated in familial amyotrophic lateral sclerosis with superoxide dismutase-1 mutation. Acta Neuropathol 2000; 100(3): 275-84.
12. Michal FD, Kuncl RW, Weinstein SJ, Malila N, Virtamo J, Albanes D. Vitamin E serum levels and controlled supplementation and risk of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2013; 14(4): 246-51.
13. Nagase M, Yamamoto Y, Miyazaki Y, Yoshino H. Increased oxidative stress in patients with amyotrophic lateral sclerosis and the effect of edaravone administration. Redox Rep 2016; 21(3): 104-12.
14. Mehanna R, Patton EJ, Phan CL, Harati Y. Amyotrophic lateral sclerosis with positive anti-acetylcholine receptor antibodies. Case report and review of the literature. J Clin Neuromuscul Dis 2012; 14(2): 82-5.
15. Vlam L, Piepers S, Sutedja NA, Jacobs BC, Tio-Gillen AP, Stam M, et al. Association of IgM monoclonal gammopathy with progressive muscular atrophy and multifocal motor neuropathy: a case-control study. J Neurol 2015; 262(3): 666-73.
16. Kadhim H, Deltenre P, Martin JJ, Sebire G. In-situ expression of Interleukin-18 and associated mediators in the human brain of sALS patients: Hypothesis for a role for immune-inflammatory mechanisms. Med Hypotheses 2016; 86: 14-7.
17. Andersen PM, Abrahams S, Borasio GD, de Carvalho M, Chio A, Van Damme P, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)--revised report of an EFNS task force. Eur J Neurol 2012; 19(3): 360-75.
18. Glass JD, Boulis NM, Johe K, Rutkove SB, Federici T, Polak M, et al. Lumbar intraspinal injection of neural stem cells in patients with amyotrophic lateral sclerosis: results of a phase I trial in 12 patients. Stem Cells 2012; 30(6): 1144-51.
19. Winters JL. American Society for Apheresis guidelines on the use of apheresis in clinical practice: practical, concise, evidence-based recommendations for the apheresis practitioner. J Clin Apher 2014; 29(4): 191-3.
20. Yokoyama H, Wada T, Furuichi K. Immunomodulation effects and clinical evidence of apheresis in renal diseases. Ther Apher Dial 2003; 7(6): 513-9.
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