Validation of Cutaneous Nerve Demyelination in Diagnosis and Treatment of CIDP

Inclusion Criteria:

• Adults (age ≥ 18 years) with definite or probable CIDP according to the EFNS/PNScriteria may enter the trial within 12 months of treatment onset or treatment naïvepatients. Written informed consent is obtained by the local investigator beforeentry into the study.

2. IVIg treatment of 0.8 up to 1.2 g/kg per 30 days will be allowed up to 6months. It is expected some patients may be on variable treatment regimens, andas along as they are not significantly declining, efforts will be made tocontinue treatment regimens already instituted.

3. Prednisone in doses up to 20 mg /day will be allowed as long as dose has beenstable for 90 days and is not being escalated or tapered during study.

4. Previous plasma exchange will be allowed as long as it is not continued duringthe study.

Exclusion Criteria:

1. Other causes of polyneuropathy, multifocal motor neuropathy, diabetes mellitus,alcohol, family history of neuropathy, monoclonal gammopathy or malignancy,history of drug or toxin exposure, which could reasonably cause neuropathy.Coexistent monoclonal gammopathy will also be used as an exclusion as thesepatients may not have the same response to IVIg.

2. Any other disease that may cause neurological symptoms and signs or that mayinterfere with treatment or outcome assessments.

3. Severe conditions that may interfere with an evaluation of the study product orsatisfactory conduct of the study such as current malignancy or history ofallogeneic bone marrow/stem cell transplant, cardiac insufficiency (New YorkHeart Association Classes III/IV), cardiomyopathy, cardiac arrhythmia requiringtreatment, unstable or advanced ischemic heart disease, congestive heartfailure or severe hypertension, chronic kidney disease deemed too severe tosafely use IVIg, known hyperprolinemia, known bleeding disorders, severe skindisease at the planned injection sites or biopsy site, alcohol, drug ormedication abuse.

4. History of keloids or other reactions to local anesthetic making skin biopsiesunsafe.

5. Patients with the following laboratory results:

6. Positive result at screening on any of the following viral markers: humanimmunodeficiency virus-1 or 2, or hepatitis B or C virus.

7. Abnormal laboratory parameters: creatinine greater than 1.5 times the upperlimit of normal (ULN), blood urea nitrogen greater than three times the ULN ifthe increase is related to potential kidney disease, or hemoglobin less than 10g/dL b. Abnormal laboratory parameters: creatinine greater than 1.5 times the upper limitof normal (ULN), blood urea nitrogen greater than three times the ULN if theincrease is related to potential kidney disease, or hemoglobin less than 10 g/dL 6.Fulfilling the following general criteria: inability to comply with study proceduresand treatment regimen; mental condition rendering the patient unable to understandthe nature, scope, and possible consequences of the study; pregnancy or nursingmother; intention to become pregnant during the course of the study; female patientsof childbearing potential either not using or not willing to use a medicallyreliable method of contraception for the entire duration of the study or notsexually abstinent for the entire duration of the study or not surgically sterile;participation in another clinical study or use of another investigational medicinalproduct within the same time period of the study.

8. Additional medications and treatments other than prednisone and IVIg fortreatment of CIDP such as azathioprine, mycophenolate, rituximab and ongoingplasma exchange as this will either confound or interfere with effects of IVIg.