Neurofascin-155 IgG was detected in 10% (21/225) of CIDP and 4% (1/24) of AIDP cases. Median age at onset was 48 years (range: 34 – 81 years, 14 males) and follow-up duration was 36 months (range: 1-192 months). The majority of patients (20/22) were neurofascin-155 IgG4 positive. Patient presented with one/more of the following: paresthesia, (19, 86%), neuropathic pain (8, 36%), sensory loss (13, 59%), proximal muscle weakness (6, 27%) distal (7, 32%) or both (7, 32%). Neurological-examination findings were proprioception/vibration loss (18, 81%), reduced/absent deep tendon reflexes (20, 90%), sensory ataxia (17, 77%), dysarthria/tremor (9, 40%), proximal (14, 64%) or distal weakness (8, 36%).  Reduced motor (21, 95%) or sensory (18, 82%) amplitudes, prolonged F-wave latencies (15, 68%) and slowed motor conduction velocities (16, 73%) were common on NCS. Temporal dispersion (6) and conduction blocks (2) were noted in the minority.  Most cases had prolonged blink reflexes (17/19). Among 12 cases with autonomic evaluation, eight had evidence of dysautonomia, commonly sudomotor dysfunction (5/8). Nerve biopsy demonstrates gradation of nodal/paranodal expansions (5/13, 38%), small-large perivascular epineurial inflammation (5/13, 38%) without onion-bulbs. All cases were treated with immunotherapy, most commonly IVIG (19/22). Other immunotherapies were utilized in 17 cases, among these 13 had a favorable response (4/5 treated with rituximab improved). Five cases demonstrated clinical improvement only with IVIG. Both neurofascin-155 IgG4 negative (neurofascin-155 IgG1 positive) cases showed initial favorable response to IVIG; however one patient relapsed and was subsequently IVIG refractory.