A Severe Course of Relapsing-Remitting Acute-Onset Chronic Inflammatory Demyelinating Polyneuropathy in a Young Man

• Main Authors: Anna P. Patnaik, Joseph Mininni, Neil C. Porter, Nicholas A. Morris
• Format: Article
• Language: English
• Published: Karger Publishers 2021-02-01
• Series: Case Reports in Neurology
• Subjects: chronic inflammatory demyelinating polyneuropathy; guillain-barré syndrome; demyelinating polyneuropathy
• Online Access: https://www.karger.com/Article/FullText/511956

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.