10-20% of patients with ALS have anti-NRIP autoantibody and the titer of anti-NRIP autoantibody is correlated with motor functional decline and mortality in ALS. The PALADIN2 clinical trial is a single arm study, which intends to enroll 20 ALS patients having anti-NRIP autoantibody in plasma. Patients will receive 3 courses of plasmapheresis per 3 months in order to maintain low concentration of anti-NRIP autoantibody in plasma. The study will follow up these patients for another 6 months after plasmapheresis. This project will potentially confirm the efficacy and safety of plasmapheresis for ALS patients having anti-NRIP autoantibody.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
20
Using plasmapheresis to remove plasma anti-NRIP autoantibody; each patient receive 3 courses of plasmapheresis with 3 months apart
National Taiwan University Hospital
Taipei City, Taipei, Taiwan
RECRUITINGChange in disease progression rates measuring using ALSFRS-R (Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised)
Difference between \[(ALSFRS-R at month -3 - ALSFRS-R at month 0)/3\] and \[(ALSFRS-R at month 0 - ALSFRS-R at month 9)/9\]; Months -3 to 0 vs. Months 0 to 9; the higher above ratio means higher disease progression rate
Time frame: 12 months
Change in disease progression rates measuring using ALSFRS-R (Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised)
Difference between \[(ALSFRS-R at month -3 - ALSFRS-R at month 0)/3\] and \[(ALSFRS-R at month 0 - ALSFRS-R at month 12)/12\]; Months -3 to 0 vs. Months 0 to 12; the higher above ratio means higher disease progression rate
Time frame: 15 months
Change in disease progression rates measuring using ALSFRS-R (Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised)
Difference between \[(ALSFRS-R at month -3 - ALSFRS-R at month 0)/3\] and \[(ALSFRS-R at month 0 - ALSFRS-R at month 3)/3\]; Months -3 to 0 vs. Months 0 to 3; the higher above ratio means higher disease progression rate
Time frame: 6 months
Change in disease severity measuring using ALSFRS-R (Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised)
The ALSFRS-R at months 3, 6, 9,12 as compared to that at month 0; the lower the AFSFRS-R, the worse disease severity
Time frame: 12 months
Change in disease progression rates measuring using MRC (Medical Research Council score) mega-score
Difference between \[(MRC mega-score at month -3 - MRC mega-score at month 0)/3\] and \[(MRC mega-score at month 0 - MRC mega-score at month 12)/12\]; Months -3 to 0 vs. Months 0 to 12; the higher above ratio means higher disease progression rate
Time frame: 15 months
Change in disease progression rates measuring using Grips force (lb)
Description: Difference between \[(Grips force at month -3 - Grips force at month 0)/3\] and \[(Grips force at month 0 - Grips force at month 12)/12\]; Months -3 to 0 vs. Months 0 to 12; the higher above ratio means higher disease progression rate
Time frame: 15 months
Change in disease progression rates measuring using 6MWT (6 minute walking test); distance in meters
Difference between \[(distance at month -3 - distance at month 0)/3\] and \[(distance at month 0 - distance at month 12)/12\]; Months -3 to 0 vs. Months 0 to 12; the higher above ratio, the higher disease progression rate
Time frame: 15 months
Change in disease progression rates measuring using EQ-5D (EuroQol Group Life Quality Score)
Difference between \[(Score at month -3 - Score at month 0)/3\] and \[(Score at month 0 - Score at month 12)/12\]; Months -3 to 0 vs. Months 0 to 12; the higher above ratio means higher disease progression rate
Time frame: 15 months
Change in disease progression rates measuring using respiratory vital capacity (standardized vital capacity, %)
Difference between \[(vital capacity -3 - vital capacity at month 0)/3\] and \[(vital capacity at month 0 - vital capacity at month 12)/12\]; Months -3 to 0 vs. Months 0 to 12; the higher above ratio means higher disease progression rate
Time frame: 15 months
Change in disease progression rates measuring using CMAP (compound muscle action potential, mV)
Difference between \[(CMAP -3 - CMAP at month 0)/3\] and \[(CMAP at month 0 - CMAP at month 12)/12\]; Months -3 to 0 vs. Months 0 to 12; the higher above ratio means higher disease progression rate
Time frame: 15 months
Change in disease progression rates measuring using MUNE (motor unit number estimation; number)
Difference between \[(MUNE -3 - MUNE at month 0)/3\] and \[(MUNE at month 0 - MUNE at month 12)/12\]; Months -3 to 0 vs. Months 0 to 12; the higher above ratio means higher disease progression rate
Time frame: 15 months
Change in disease severity measuring using plasma NF-L (Neurofilament-light chain; pg/mL)
The NF-L at months 3, 6, 9,12 as compared to that at month 0; the lower the NF-L, the milder disease activity
Time frame: 12 months
Any side effect under plasmapheresis
Time frame: 12 months
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