Patient 1ref.3 Patient 2ref.3 Patient 3ref.3 Patient 4ref.3 Patient 5ref.4 Patient 6ref.4 Patient 7ref.4 Patient 8ref.4 Patient 9ref.4 Present patient age/sex 24/M 37/F 33/F 11/M 66/F 62/M 50/F 50/M 46/M 65/F disease duration (years) 12 12 4 8 3 3 5 1 2 7 clinical course relapsing relapsing relapsing relapsing relapsing relapsing relapsing relapsing relapsing relapsing number of relapses 45 10 7 15 5 4 7 4 9 14 paresthesia - - - - finger - - finger finger - CSF proteins (mg/dl) 123 60 70 96 42 86 98 113 145 175 sensory conduction velocity                     sural nerve (m/sec) 44 54 43 52 39.4*a 42.3*a 46.2 52.6 42.7 48.6 other sensory nerves normal normal normal normal normal normal normal normal normal normal sural nerve biopsy normal normal normal - mild abnormal mild abnormal - - - mild abnormal Response to therapies                     corticosteroids (ST) No No No No No No No No No No IVIG Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes plasma exchange (PE) Yes Yes - Yes - - done*b - - - cyclosporin A - - - No - - - done*b - No cyclophosphamide - - - - - - - - done*b No azathioprine Yes No No No - - - - done*b - interferon alpha - Yes - Yes - - - - - - Outcome                     entered remission Yes Yes Yes Yes Yes Yes Yes No No Yes therapy during  remission azathioprine, PE interferon alpha IVIG - ST - ST ST ST ST remission periods (years) 3 1 1 7 1.8 1.5 1.3 - - 4 CSF: cerebrospinal fluid, IVIG: intravenous immunoglobulin, M: male, F: female *a: authors suggested that sensory nerve conduction slowing was caused by diabetes mellitus. *b: the therapy was performed, but the effectiveness was not mentioned.

Table 1: Clinical features of reported patients with motor-dominant chronic inflammatory demyelinating polyneuropathy.