Department of Neurology, Centro Hospitalar Universitário de São João, E.P.E., Porto, Portugal; Department of Clinic Neurosciences and Mental Health, Faculty of Medicine of University of Porto, Porto, Portugal.
Department of Anatomic Pathology, Centro Hospitalar Universitário de São João, E.P.E., Porto, Portugal.
J Neuroimmunol. 2019 Sep 15;334:576997. doi: 10.1016/j.jneuroim.2019.576997. Epub 2019 Jun 19.
We describe the case of a 69-year-old man who presented with symptoms of headache and severe vision loss due to G4 immunoglobulin (IgG4) hypertrophic pachymeningitis (HP). The patient was initially responsive to corticotherapy, but vision loss progressed when steroid therapy was first tapered. No improvement was noticed with intravenous rituximab. The patient showed clinical and radiological improvement after intrathecal rituximab, which can be an efficacious alternative treatment option.
我们描述了一例 69 岁男性患者,因 G4 免疫球蛋白(IgG4)肥厚性硬脑膜炎(HP)出现头痛和严重视力丧失的症状。该患者最初对皮质激素治疗有反应,但当开始逐渐减少激素治疗时,视力丧失进展。静脉注射利妥昔单抗后未见改善。鞘内注射利妥昔单抗后患者的临床和影像学均有改善,这可能是一种有效的替代治疗选择。