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Distal acquired demyelinating symmetric polyneuropathy progressing to classic chronic inflammatory demyelinating polyneuropathy and response to fludarabine and cyclophosphamide

Authors

  • Megan M. Leitch MD,

    1. Department of Neurology, Neurological Institute, Columbia University, 710 W 168th Street, Box 163, New York, New York 10032 USA
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  • William H. Sherman MD,

    1. Department of Medicine, Columbia University, New York, New York 10032 USA
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  • Thomas H. Brannagan III MD

    Corresponding author
    1. Department of Neurology, Neurological Institute, Columbia University, 710 W 168th Street, Box 163, New York, New York 10032 USA
    • Department of Neurology, Neurological Institute, Columbia University, 710 W 168th Street, Box 163, New York, New York 10032 USA
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Abstract

Introduction: Distal acquired demyelinating symmetric polyneuropathy (DADS) is proposed as a distinct entity from classic chronic inflammatory demyelinating polyneuropathy (CIDP). Methods: We report a 58-year-old woman with DADS that progressed to a severe case of classic CIDP. Results: She had distal numbness and paresthesias, minimal distal weakness and impaired vibratory sensation. She had anti-MAG antibodies, negative Western blot, and lacked a monoclonal gammopathy. There were prolonged distal motor latencies. She remained stable for 6 years until developing proximal and distal weakness. Nerve conduction studies showed multiple conduction blocks. She developed quadriparesis despite first-line treatment for CIDP. She was started on cyclophosphamide and fludarabine. Twenty-five months after receiving chemotherapy, she had only mild signs of neuropathy off all immunotherapy. Conclusions: DADS may progress to classic CIDP and is unlikely to be a separate disorder. Fludarabine and cyclophosphamide may be effective for refractory CIDP. Muscle Nerve, 2013

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