NEUROPHYSIOLOGIC EVALUATION OF LONG-TERM DESFERRIOXAMINE THERAPY IN BETA-THALASSEMIA PATIENTS

Forty patients with beta-thalassemia major (BTM), between 11 and 19 ye ars of age and maintained on long-term desferrioxamine (DFO) treatment , were examined by evoked potential and nerve conduction velocity stud ies to investigate a possible involvement of the auditory, visual, som atosensory, or peripheral nervous pathways. Pathologic findings in bra instem auditory-, visual-, and somatosensory-evoked potentials, and ne rve conduction velocity studies were demonstrated in 25%, 15%, 7.5%, a nd 25% of the patients, respectively, whereas 15% demonstrated involve ment of multiple neural pathways. Subclinical involvement of the audit ory pathway was statistically associated with higher mean daily DFO do se and longer duration of DFO therapy, whereas abnormalities regarding the somatosensory pathways were related to older age, longer mean dur ation of DFO therapy, and lower serum copper levels. Involvement of th e peripheral nervous system was related to lower serum copper levels. Multiple involvement of neural pathways was related to longer mean dur ation of DFO therapy. We conclude that risk factors related to long-te rm DFO treatment are only partly responsible for the subclinical invol vement of neural pathways demonstrated in beta-thalassemia major patie nts. (C) 1998 by Elsevier Science Inc. All rights reserved.