FV 1016. Antibodies against Neurofascin Are Associated with Severe und Chronic Forms of Guillain–Barré’s Syndrome

Background: GuillainBarré’s syndrome (GBS) is an acute neuropathy typically characterized by diffuse limb weakness, whereas neurological presentation and symptoms between cases are considerably diverse in addition to response to treatment and course of the disease with some progressing to chronic forms. In a subgroup of adults with a severe form of GBS/CIDP neurofascin antibodies have been detected.

Aims: To screen serum of children with GBS/CIDP for different autoantibodies against gangliosides and neurofascin 155 (NF).

Question: Are neurofascin antibodies detectable in children with GBS/CIDP?

Methods: Children with a history GBS were included in the study. From the majority of children a complete clinical dataset including CSF and NCS studies and course of the disease) was available in addition to serum samples from the initial manifestation or obtained during the further course of the disease. Serum samples were screened for autoantibodies (abs) against different antigens (e.g., neurofascin 155, contactin 1, gangliosides such as GM1, GD1a, GD1b, GQ1b) using ELISAs and cell based assays.

Results: Forty-nine children were referred from different hospitals in Germany/Austria between 2010 and 2018 with a diagnosis of GBS (AMAN [n = 3], MFS [n = 11], AIDP [n = 35]). Median age range was 11 years (1–18 years). Four children continued to have a CIDP. Our preliminary results further showed that in 2/49 children neurofascin 155 abs were detected both of whom had a long and protracted disease course requiring different forms of immunomodulation. Seven children had GM1-, three GD1a, and six had GD1b IgG abs.

Conclusion: In a small subgroup of children with a severe GBS and a subsequent chronic course, NF abs were found. Adult studies suggest that these patients should be treated with rituximab.