Aggressive Acquired Demyelinating Neuropathy Caused by NF-155: Initially Treatment-Resistant

Abstract

Objectives: Anti–neurofascin-155 IgG4 (NF-155) antibody disease has previously been associated with a subset of patients with chronic inflammatory demyelinating polyradiculoneuropathy. We report a case of NF-155 positive polyneuropathy that initially presented as an acute inflammatory demyelinating polyradiculoneuropathy. The patient responded appropriately to treatment but subsequently progressed over a 3-month period, resulting in quadriplegia, areflexia, and oculobulbar paralysis. Methods: Case report and literature review. Results: A 40-year-old male presented with acute bilateral arm and thigh weakness, areflexia, and distal sensory loss. Treatment with intravenous immunoglobulin (IVIg) for acute acquired demyelinating neuropathy resulted in initial improvement but subsequent decline. Lack of response to additional IVIg and plasmapheresis (PLEX) prompted testing for NF-155. Treatment with rituximab and steroids resulted in virtually complete recovery. Conclusions: Early testing for nodal and paranodal proteins is indicated in patients who present with acute acquired demyelinating neuropathy but fail to respond to conventional treatments, such as IVIg or PLEX. Identification of nodal and paranodal antibodies should prompt treatment with rituximab and steroids to increase likelihood of recovery.

Autoimmune nodopathy

“…A certain degree of treatment responsiveness is anticipated in cases involving pathogenic autoantibodies other than IgG4, such as IgG1 or IgG3 54,57,58 . However, this response may transition to a nonresponsive state, often accompanied by a switch to the IgG4 subclass.…”