Plasmapheresis in Amyotrophic Lateral Sclerosis With Autoantibody Against NRIP

Study Overview
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Brief Summary
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Patient with amyotrophic lateral sclerosis (ALS) having anti-NRIP autoantibody showed titer-dependent detrimental Effects. Plasmapheresis might benefit this subgroup of patients via removal of anti-NRIP autoantibody

Official Title
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Plasmapheresis in Amyotrophic Lateral Sclerosis With Autoantibody Against NRIP

Conditions
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Amyotrophic Lateral Sclerosis, Plasmapheresis

Intervention / Treatment
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* Procedure: Plasmapheresis


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients with amyotrophic lateral sclerosis (ALS) at the age more than 20 years and having plasma anti-NRIP autoantibody. Agree to receive plasmapheresis intervention. Agree to participate in the trial and receive serial examinations and follow up. Exclusion Criteria: Patients without plasma anti-NRIP autoantibody. Patients requiring permanent ventilator support for ALS progression. Not able to receive plasmapheresis or trial-related examinations. Under pregnancy. Blood fibrinogen level less than 50 mg/dl. Specific ALS subtypes, including primary lateral sclerosis, progressive muscular atrophy, flail arm syndrome, or flail leg syndrome.

Ages Eligible for Study
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Minimum Age: 20 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: ALS patients receiving plasmapheresis<br>Plasmapheresis in ALS patients with different titers of autoantibody against NRIP | Procedure: Plasmapheresis<br>* Regular plasmapheresis to remove anti-NRIP autoantibody<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in ALSFRS-R decline | Change in ALSFRS-R decline before (3-month) and after (3-month) intervention. <ALSFRS-R indicates revised ALS functional rating scale, ranged from 0 to 48 with the higher, the better motor function> | Before (3-month) and after (3-month) intervention | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in ALSFRS-R decline | Change in ALSFRS-R decline before (3-month) and after (6-month) intervention | Before (3-month) and after (6-month) intervention | 
| Changes in ALSFRS-R score | Changes in ALSFRS-R score from day 0 to days 30, 90, and 180 | Day 0 to days 30, 90, and 180 | 
| Change in force vital capacity | Change in force vital capacity before intervention and on day 90 | Before intervention and on day 90 | 
| Change in compound motor action potentials | Change in compound motor action potentials before intervention and on day 90 | Before intervention and on day 90 | 
| Changes in anti-NRIP titer | Changes in anti-NRIP titer from day 0 to days 30, 90, and 180 | Day 0 to days 30, 90, and 180 | 
| Any adverse effect under plasmapheresis | Any adverse effect during and within 6 months after plasmapheresis | Within 6 months during and after plasmapheresis |