TY  - JOUR
A1  - Stengel, Helena
A1  - Vural, Atay
A1  - Brunder, Anna-Michelle
A1  - Heinius, Annika
A1  - Appeltshauser, Luise
A1  - Fiebig, Bianca
A1  - Giese, Florian
A1  - Dresel, Christian
A1  - Papagianni, Aikaterini
A1  - Birklein, Frank
A1  - Weis, Joachim
A1  - Huchtemann, Tessa
A1  - Schmidt, Christian
A1  - Körtvelyessy, Peter
A1  - Villmann, Carmen
A1  - Meinl, Edgar
A1  - Sommer, Claudia
A1  - Leypoldt, Frank
A1  - Doppler, Kathrin
T1  - Anti–pan-neurofascin IgG3 as a marker of fulminant autoimmune neuropathy
JF  - Neurology: Neuroimmunology & Neuroinflammation
N2  - Objective 
To identify and characterize patients with autoantibodies against different neurofascin (NF) isoforms.

Methods 
Screening of a large cohort of patient sera for anti-NF autoantibodies by ELISA and further characterization by cell-based assays, epitope mapping, and complement binding assays.

Results 
Two different clinical phenotypes became apparent in this study: The well-known clinical picture of subacute-onset severe sensorimotor neuropathy with tremor that is known to be associated with IgG4 autoantibodies against the paranodal isoform NF-155 was found in 2 patients. The second phenotype with a dramatic course of disease with tetraplegia and almost locked-in syndrome was associated with IgG3 autoantibodies against nodal and paranodal isoforms of NF in 3 patients. The epitope against which these autoantibodies were directed in this second phenotype was the common Ig domain found in all 3 NF isoforms. In contrast, anti–NF-155 IgG4 were directed against the NF-155–specific Fn3Fn4 domain. The description of a second phenotype of anti–NF-associated neuropathy is in line with some case reports of similar patients that were published in the last year.

Conclusions 
Our results indicate that anti–pan-NF-associated neuropathy differs from anti–NF-155-associated neuropathy, and epitope and subclass play a major role in the pathogenesis and severity of anti–NF-associated neuropathy and should be determined to correctly classify patients, also in respect to possible differences in therapeutic response.
KW  - neurology
Y1  - 2019
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-202462
VL  - 6
IS  - 5
ER  -