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Europa Medicophysica 2000 June;35(2):55-60

Copyright © 2000 EDIZIONI MINERVA MEDICA

language: English

Electrophysiological evaluation of the peripheral and central pathways in patients with achondroplasia before and during a lower-limb lenghtening procedure

Polo A. ¹, Zambito A. ², Antoniazzi F. ³, Simeone M. ³, Aldegheri R. ⁴

¹ Department of Neurological and Visual Sciences, University of Verona, Italy; ² Division of Orthopaedic Rehabilitation, University of Verona, Italy; ³ Department of Paediatrics, University of Verona, Italy; ⁴ Institute of Orthopaedics, University of Verona, Italy

In ­this ­paper we ­review the spec­trum of spi­nal and periph­er­al ­nerve involve­ment sec­on­dary to achon­dro­pla­sia. Alongside con­ven­tion­al and com­pu­ter­ised imag­ing tech­niques, elec­tro­phys­io­log­i­cal inves­ti­ga­tion may rep­re­sent a use­ful, non-inva­sive ­approach in ­this clin­i­cal set­ting. Somatosensory ­evoked poten­tials (SEPs) and mag­net­ic stim­u­la­tion are val­u­able ­tools for stud­y­ing spi­nal ­cord func­tion. Neurophysio-logical abnor­mal­ities ­show a ­good cor­re­la­tion ­with the ­lesion lev­el. Imaging tech­niques indi­cate ­that mul­ti­ple mal­for­ma­tion can ­affect the ­patient at the ­same ­time and SEPs ­help to deter­mine the ­main ­site of involve­ment. Interestingly, ­these tech­niques are ­more sen­si­tive ­than clin­i­cal eval­u­a­tion in doc­u­ment­ing neu­ro­log­i­cal impair­ment in ­patients ­with achon­dro­pla­sia ­prior to the man­i­fes­ta­tion of unmis­tak­able ­signs. Callotasi has ­became a wide­ly ­used and accept­ed pro­ce­dure for ­limb length­en­ing. Extensive length­en­ing can be safe­ly per­formed in ­patients ­with achon­dro­pla­sia ­once neu­ro­log­i­cal impair­ment has ­been ­ruled out. In our expe­ri­ence, the pres­ence of elec­tro­phys­io­log­i­cal abnor­mal­ities ­calls for a com­pre­hen­sive sur­gi­cal re-eval­u­a­tion of the tra­di­tion­al pro­ce­dure, and some­times exclu­sion of ­patients. Peripheral ­nerve involve­ment may ­occur dur­ing ­limb length­en­ing, and con­tin­u­ous ­nerve mon­i­tor­ing pro­vides use­ful ­insights ­into the pathoph­y­sio­lo­gy of ­nerve dam­age.