Ambaŭflanka Neŭropatio De La Optaj Nervoj Respondanta Al Steroidoj Ĉe Malsanulino Kiu Suferas De La Hiper Imunglobulino-Dd-Sindromo

We present the case of a young woman with hyper-IgD syndrome (HIDS) due to heterozygous V377I and I268T mutations, who developped a bilateral optic neuritis. While the CSF was normal and no NMO-antibodies where detected, she showed signs of systemic inflammation with a raised CRP. The bilateral optic neuritis responded to 5 days of high-dosage corticosteroids in due course. To our knowledge this is until now the first case of such complaints described in a patient with HIDS.