Evoked potentials in cerebrotendinous xanthomatosis and effect induced by chenodeoxycholic acid
M. Mondelli, A. Rossi, C. Scarpini, M. T. Dotti and A. Federico
Institute of Neurological Sciences, Medical School, University of Siena, Italy.
Evoked potentials are reported in 10 patients with cerebrotendinous
xanthomatosis, eight of whom had peripheral neuropathy. Four subjects
showed delayed N13 to N20 interpeak latencies for arm somatosensory evoked
potentials, and five showed moderately prolonged I to III and I to V
interpeak latencies of brain-stem auditory evoked potentials. Six of seven
patients showed marked delay and desynchronization of visual evoked
potentials. All five patients undergoing transcutaneous magnetic
stimulation of the motor cortex presented greatly delayed central motor
conduction time, especially of the lower limbs. After treatment with
chenodiol (750 mg/d for at least 2 years), there was a significant
improvement in nerve conduction velocities, N13 to N20 interpeak latencies,
and visual evoked potential latencies. Brain-stem auditory evoked
potentials remained unchanged.
