PT - JOURNAL ARTICLE AU - Belbezier, Aude AU - Joubert, Bastien AU - Montero-Martin, Gonzalo AU - Fernandez-Vina, Marcelo AU - Fabien, Nicole AU - Rogemond, Véronique AU - Mignot, Emmanuel AU - Honnorat, Jérôme TI - Multiplex family with GAD65-Abs neurologic syndromes AID - 10.1212/NXI.0000000000000416 DP - 2018 Jan 01 TA - Neurology - Neuroimmunology Neuroinflammation PG - e416 VI - 5 IP - 1 4099 - http://nn.neurology.org/content/5/1/e416.short 4100 - http://nn.neurology.org/content/5/1/e416.full SO - Neurol Neuroimmunol Neuroinflamm2018 Jan 01; 5 AB - Objective: Neurologic autoimmune syndromes associated with anti–glutamate acid decarboxylase 65 antibodies (GAD65-Abs) are rare and mostly sporadic.Methods: We describe a niece and her aunt with GAD65-Abs neurologic syndromes. High-resolution HLA typing of Class I and Class II alleles was performed using next-generation sequencing.Results: The proband had cerebellar ataxia and probable limbic encephalitis features, whereas her niece had stiff-person syndrome. Both had a high titer of GAD65-Abs in serum and CSF and showed signs of inflammation in CSF. Both affected members carried the same rare recombinant DRB1*15:01:01∼DQA1*01:02:01∼DQB1*05:02:01 haplotype, which may or may not be involved in disease susceptibility. Of interest, other unaffected members of the family either had the same HLA haplotype but normal serum GAD65-Abs or had different HLA types but a high titer of serum GAD65-Abs without neurologic symptoms, suggesting cumulative effects.Conclusions: This unique association strengthens the concept that hereditary factors, possibly including specific HLA haplotypes, play a role in neurologic syndromes associated with GAD65-Abs.GAD=glutamic acid decarboxylase; IgG=immunoglobulin G; SPS=stiff-person syndrome; T1DM=type 1 diabetes mellitus; TG=antithyroglobulin; TPO=antithyroperoxidase