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- W3132139097 abstract "Abstract Background CIDP (chronic inflammatory demyelinating polyneuropathy) is an autoimmune disorder of peripheral nervous system, causing demyelination and even axonal degeneration. Recently, anti- Neurofascin155 (NF155) antibody-positive CIDP began to cut a figure. This is a special type of CIDP that progressively worsen and predominantly involving distal limbs. IgG4 anti- NF155 antibody-positive CIDP combining serum with CSF in clinical is very rare. The article displayed the clinical practice to evoke the diagnosis of IgG4 anti- NF155 antibody-positive CIDP in front of the disabling tremor and ataxia. We described a rare clinical disorder of IgG4 anti- NF155 antibody-positive CIDP with reviewing some of literatures. Case presentation A 49-year-old woman presented with an evaluation of weakness, numbness and a little ache of all her limbs for half a year. She revealed ataxia, intention tremor and wide-based gait. Cerebrospinal fluid (CSF) results showed the noticeably elevated protein level without pleocytosis. The findings of peripheral nerve conduction（NCV）indicated marked prolongation of distal and F-wave latencies which conformed to demyelinating polyneuropathy. And her spinal MRI illustrated a thickened right nerve root of sacrum 1-2. IgG4 NF155 antibodies of serum together with CSF were positive by indirect immunofluorescence based on NF155 transfection cells. A high-dosage mythylprednisolone pulse followed by oral prednisolone improved her symptoms. Conclusion Disabling tremor, ataxia, younger onset age, high protein levels in CSF, marked prolongation of distal and F-wave latencies, the roots or plexus of neck and lumbosacral nerves thickening on MRI, and poor response to IVIg may consider as biomarkers of IgG4 anti- NF155 antibody-positive CIDP." @default.
- W3132139097 created "2021-03-01" @default.
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- W3132139097 date "2019-05-23" @default.
- W3132139097 modified "2023-09-26" @default.
- W3132139097 title "A case of a 49-year-old woman with IgG4 anti-Neurofascin155 antibody-positive CIDP in both serum and cerebrospinal fluid" @default.
- W3132139097 doi "https://doi.org/10.21203/rs.2.9764/v1" @default.
- W3132139097 hasPublicationYear "2019" @default.
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