asymptomatic CMT 1A and vincristine neuropathy

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Abstract from Pediatr Blood Cancer. 2004 Oct;43(5):606.

Vincristine neuropathy: Neurophysiological and genetic studies in a

case of Wilms tumor.

Schiavetti A, Frascarelli M, Uccini S, Novelli A.

Department of Pediatrics, University of Rome " La Sapienza, " Rome,

Italy.

We report a 10-year-old female with Wilms tumor (WT) who developed

severe neuropathy after the fifth weekly dose of vincristine. The

girl was previously asymptomatic and the family history was negative

for inherited neuropathies. Neurophysiological studies and

electrodiagnostic findings were suggestive of a axonal neuropathy

with greater motor than sensory characteristics not typical of

Charcot-Marie-Tooth (CMT) Type 1A. Genetic studies were performed in

view of the degree of neurotoxicity. Duplication of 17p11.2 was found

that supported the diagnosis of CMT Type 1A. The patient is alive

without disease and with minimal weakness of the lower extremities

after 42 months. Neurophysiological studies, repeated at 8 and 24

months, were negative. Although the association of asymptomatic CMT

and vincristine neuropathy has been previously reported, the present

case is of note because the reversible neuropathy occurred after five

doses of vincristine, suggesting that possible more people suffering

vincristine neurotoxicity may have underlying and asymptomatic CMT.