Patnaik Anna P, Mininni Joseph, Porter Neil C, Morris Nicholas A
Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Department of Pathology, University of Maryland School of Medicine, Baltimore, Maryland, USA.
Case Rep Neurol. 2021 Feb 4;13(1):73-77. doi: 10.1159/000511956. eCollection 2021 Jan-Apr.
Acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) is an immune mediated neuropathy characterized by progressive weakness and sensory impairment lasting over 2 months. Guillain-Barré-Strohl syndrome (GBS) is an immune mediated polyneuropathy with a similar presentation often over less than 4 weeks. While some have argued for the existence of recurrent GBS, most classify the syndrome as a form of relapsing-remitting CIDP. However, there are cases of GBS with treatment-related fluctuations that must be distinguished from A-CIDP as patients with A-CIDP require long-term immunotherapy. In this case report, we discuss a patient with multiple relapses over 3 years, who is more likely to have A-CIDP. His ganglioside profile, which has rarely been reported in A-CIDP, included high concentrations of anti-GM1, anti-GD1a, and anti-GD1b antibodies, which may account for his severe disease course.
急性起病慢性炎性脱髓鞘性多发性神经病（A-CIDP）是一种免疫介导的神经病，其特征为进行性肌无力和感觉障碍持续超过2个月。吉兰-巴雷-施特罗尔综合征（GBS）是一种免疫介导的多发性神经病，临床表现相似，通常病程少于4周。虽然有些人主张复发性GBS的存在，但大多数人将该综合征归类为复发缓解型CIDP的一种形式。然而，存在GBS伴有与治疗相关波动的情况，必须与A-CIDP相区分，因为A-CIDP患者需要长期免疫治疗。在本病例报告中，我们讨论了一名在3年中多次复发的患者，他更可能患有A-CIDP。他的神经节苷脂谱在A-CIDP中很少被报道，包括高浓度的抗GM1、抗GD1a和抗GD1b抗体，这可能解释了他严重的病程。
