title: Distal acquired demyelinating symmetric polyneuropathy progressing to classic chronic inflammatory demyelinating polyneuropathy and response to fludarabine and cyclophosphamide.
abstract: BACKGROUND
  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.
authors.affiliation: Department of Neurology, Neurological Institute, Columbia University, 710 W 168th Street, Box 163, New York, New York 10032 USA.
authors.forename: Megan M
authors.initials: MM
authors.lastname: Leitch
authors.forename: William H
authors.initials: WH
authors.lastname: Sherman
authors.forename: Thomas H
authors.initials: TH
authors.lastname: Brannagan
mesh_terms: D003520:Cyclophosphamide / Q000627:therapeutic use*; D018450:Disease Progression; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008875:Middle Aged; D009431:Neural Conduction / Q000502:physiology; D010525:Peripheral Nerves / Q000503:physiopathology; D011115:Polyneuropathies / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D016896:Treatment Outcome; D014740:Vidarabine / Q000031:analogs & derivatives* / Q000627:therapeutic use
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D014740:Vidarabine; C024352:fludarabine