Abstract
BackgroundAutoantibodies targeting node of Ranvier proteins are rarely reported in China.Case presentationWe present the case of a 66-year-old Chinese man who concomitantly developed chronic inflammatory demyelinating polyneuropathy with anti-contactin-associated protein 1 antibody and bile duct hamartomas in liver, which are rarely reported in China. The man presented with chronic progressive sensory and motor symptoms, bilateral periphery facial paralysis, and protein–cell dissociation of cerebrospinal fluid. Nerve conduction study indicated demyelinating neuropathy. Enhanced magnetic resonance imaging of the liver showed diffuse intrahepatic lesions, which were considered as bile duct hamartomas in the liver. He was suspected as having chronic inflammatory demyelinating polyneuropathy and treated with intravenous immunoglobulin and prednisone. However, his condition got worse. One month later, he was diagnosed with chronic inflammatory demyelinating polyneuropathy associated with anti-contactin-associated protein 1 antibody. He received high-dose methylprednisolone, followed by standard plasma exchange and rituximab therapy. His sensory and motor manifestations were significantly improved at 1 year of follow-up.ConclusionsThis case reminds clinicians to be aware of antiparanodal antibodies, which are associated with specific phenotypes and therapeutic response.
Highlights
Autoantibodies targeting node of Ranvier proteins are rarely reported in China.Case presentation: We present the case of a 66-year-old Chinese man who concomitantly developed chronic inflammatory demyelinating polyneuropathy with anti-contactin-associated protein 1 antibody and bile duct hamartomas in liver, which are rarely reported in China
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous chronic autoimmune neuropathy characterized by chronic progressive motor and sensory deficits during a period of at least 8 weeks
Other normal or negative antibody tests included myelin basic protein (MBP), myelin oligodendrocyte glycoprotein (MOG), aquaporin-4 (AQP4), antibodies associated with paraneoplastic neurological syndromes (PNS), and antiganglioside complex antibody profiles
Summary
It appears advisable to test for antibodies against node of Ranvier proteins in subjects with CIDP resistant to intravenous immunoglobulin.
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