Severe vincristine neuropathy in Charcot-Marie-Tooth disease type 1A

Cancer. 1996 Apr 1;77(7):1356-62. doi: 10.1002/(SICI)1097-0142(19960401)77:7<1356::AID-CNCR20>3.0.CO;2-#.


Background: A general predisposition for vincristine-related neuropathy has been observed in persons with a family history of hereditary neuropathies.

Methods: In a retrospective case series, we investigated the possible association between the DNA rearrangement found in patients with Charcot-Marie-Tooth Disease Type 1A (CMT1A) and susceptibility to the neurotoxicity of vincristine. In selected patients and family members, we performed electrodiagnostic studies and analyzed DNA samples for 17p11.1-12 duplication associated with CMT1A.

Results: We describe three families with autosomal dominant CMT1, among whom a family member with a neoplastic disease suffered rapid onset, severe neuropathy after receiving initial doses of vincristine as a part of a routine chemotherapy protocol. All three families had at least one affected family member with 17p11.2-12 duplication.

Conclusions: These cases show that 17p11.2-12 duplication predisposes patients to severe neurotoxicity from vincristine and that this drug should be avoided with patients with CMT1A. It is therefore essential to obtain a detailed family history for all oncology patients to screen for possible hereditary neuropathies. In patients with unexplained or preexisting familial neuropathy, testing for 17p11.2-12 duplication should be carried out prior to initiating vincristine therapy. Patients with other hereditary neuropathies may also be at risk for severe neurotoxic reactions.

Publication types

  • Case Reports
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Charcot-Marie-Tooth Disease / genetics*
  • Charcot-Marie-Tooth Disease / physiopathology*
  • Child
  • Chromosomes, Human, Pair 17
  • DNA / genetics
  • Disease Susceptibility
  • Electrophysiology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Nervous System Diseases / chemically induced*
  • Nervous System Diseases / diagnosis
  • Pedigree
  • Pregnancy
  • Retrospective Studies
  • Vincristine / adverse effects*


  • Vincristine
  • DNA