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Distal acquired demyelinating symmetric polyneuropathy progressing to classic chronic inflammatory demyelinating polyneuropathy and response to fludarabine and cyclophosphamide
Author(s) -
Leitch Megan M.,
Sherman William H.,
Brannagan Thomas H.
Publication year - 2013
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.23629
Subject(s) - medicine , chronic inflammatory demyelinating polyneuropathy , fludarabine , cyclophosphamide , polyneuropathy , weakness , dermatology , surgery , chemotherapy , immunology , antibody
: 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