2017 Volume 33 Issue 4 Pages 537-541
In 2015, several studies about CIDP with antibodies to paranodal proteins were reported. Of the 533 patients with CIDP, 13 (2.4%) had IgG4 antibody to contacin–1. Three of 13 patients showed subacute symptom onset. All of the patients presented with sensory ataxia. Six of 10 anti–contactin–1 antibody–positive patients had poor response to IVIg, whereas 8 of 11 antibody–positive patients had good response to corticosteroids. The clinical features of CIDP with anti–neurofascin–155 antibody were reported. Anti–neurofascin–155 IgG4 antibodies were detected in 18% (9/50) of CIDP patients. Anti–neurofascin–155 antibody–positive CIDP patients had younger onset age (p＜0.0001), higher frequency of drop foot (p＝0.0242), tremor (p＝0.03), and DADS phenotype (p＝0.0014). Greater cervical root diameter on MRI neurography (p＝0.002) and higher CSF protein levels (p＜0.0001) were observed. Improvement of polyneuropathy following autologous stem cell transplantation (ACST) for sixty patients with POEMS syndrome was reported. After ACST, the Neuropathy Impairment Score improved from 66 to 48 points at 12 months and to 30 points at most recent follow–up (p＜0.0001). The mRS score improved from 3 to 1.5 (p＜0.0001). VEGF levels decreased from 452 to 63.5pg/mL (p＜0.0001). The ulnar CMAP improved from 4.3 to 7.6mV (p＜0.0001) and CV improved from 34 to 51m/s (p＜0.0001). The clinicopathologic features of 18 patients with folate–deficiency neuropathy were reported. Those patients presented with low serum folate levels but normal blood thiamine and serum cobalamin levels in the absence of chronic alcoholism. Folate–deficiency neuropathy was characterized by slowly progressive polyneuropathy with predominant involvement of the lower extremities, with a tendency to manifest as sensory rather than motor neuropathy and predominant deep rather than superficial sensory loss, which was different from thiamine–deficiency neuropathy.
