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Neurophysiologic Study of β-Thalassemia Patients

Department of Paediatrics, University of Hong Kong, Queen Mary Hospital, Hong Kong

A. Li, MBBS, FRCP(Edin)

Department of Paediatrics, University of Hong Kong, Queen Mary Hospital, Hong Kong

A.C.W. Lee, MBBS, MRCP(UK)

Department of Paediatrics, University of Hong Kong, Queen Mary Hospital, Hong Kong

Neurophysiologic investigations were performed in 34 Chinese patients with β-thalassemia major maintained on long-term desferrioxamine treatment to look for subclinical toxicity in the auditory, visual, peripheral, or central neural pathways. In the auditory pathway study, four patients (12%) had mild sensorineural hearing impairment. Two patients (6%) had increased P 100 latencies in the visual evoked potential study, and nine patients (26%) had abnormal electroretinogram results. All had normal electrooculograms. Ophthalmoscopic examination was abnormal in three patients (9%), and three (9%) had a visual field defect. In the peripheral or central nervous pathways, seven patients (21%) had sensory neuropathy, of which three cases were probably related to diabetes mellitus. All had normal motor conduction velocities. Four patients (12%) had increased cortical latencies of median or posterior tibial somatosensory evoked potential. Abnormalities in multiple neural pathways were seen in four patients (12%). There was a significant association between subclinical toxicity to the peripheral or central nervous systems and serum ferritin level (P < .03) and the presence of diabetes mellitus (P < .002). There was no significant relationship between the age, dosage, or duration of desferrioxamine used and the increased risk of neurotoxicity to the auditory, visual, peripheral, or central nervous systems. There was also no association between the risk of neurotoxicity and the serum zinc, copper, or fructosamine levels. (J Child Neurol 1993;8:330-335).

Journal of Child Neurology, Vol. 8, No. 4, 330-335 (1993)
DOI: 10.1177/088307389300800407


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