A comparison of the clinical features between CIDP with MN and anti-CNTN1 antibody-positive CIDP revealed male preponderance, relatively higher age of onset, occasional acute/subacute onset, distal dominant sensorimotor neuropathy suggestive of typical CIDP, frequent proprioceptive sensory impairment leading to sensory ataxia, and very high CSF protein levels as common features, although in some CIDP patients with MN including our case, CSF protein levels were not very high, possibly reflecting hypoalbuminemia caused by nephrotic syndrome. Here, CNTN1 is linked to sensory ataxia.