Acute deterioration of Charcot-Marie-Tooth disease IA (CMT IA) following 2mg of vincristine chemotherapy

Background: Severe up to life-threatening neuropathy has been observed in p atients with hereditary neuropathies receiving vincristine. Case report: A 52-year-old female painter suffering from high-grade non-Hod gkin's lymphoma (stage IVB) was treated with a total of 4 mg of vincristine during two courses of CHOP chemotherapy (cyclophosphamide, vincristine, ad riamycin, prednisone). At onset of treatment no neurological problems were reported. There was good lymphoma response to chemotherapy. At the same tim e, however, the patient gradually developed dysphagia, dysarthria, muscular weakness of both lower and upper extremities, areflexia, paraesthesia of t he fingertips and bilateral sensory impairment of feet and lower legs. Thes e symptoms continually worsened over a period of seven weeks until she was unable to walk or to perform her work. Electrophysiological studies showed peripheral axonal and demyelinative sensorimotor neuropathy in correlation to histological findings. Molecular analysis revealed 17p11.2 duplication t ypical for Charcot-Marie-Tooth disease IA. While continuing chemotherapy wi thout the use of vincristine the patient's neurologic symptoms slowly recov ered within six months. Conclusion: Prior to administration of vincristine family and patient histo ry as well as physical examination should be performed carefully to look fo r underlying hereditary neuropathy. For those patients with a clinical hist ory or symptoms suggestive for CMT nerve conduction velocity studies and on an individual base even molecular genetic analysis are neccessary to preve nt serious neurologic complications.