Evoked potentials in children with Wilson's disease.
We assessed multimodal evoked potentials (EPs) in 13 children with newly diagnosed neurologically symptomatic Wilson's disease (WD) and in their first degree symptom-free relatives, consisting of seven presymptomatic and 15 asymptomatic siblings and 22 asymptomatic parents. EP abnormalities of at least one modality and one side stimulation were observed in 38.5% of patients, 42.9% of presymptomatic siblings, 21.4% of asymptomatic siblings and 18.2% of parents. Patients tended to have more prolonged central latencies of EPs. However, the left I-V interpeak brainstem auditory EP latency difference was the only one to reach at the statistical significance (P = 0.001). Abnormal VEP P100 latency was detected more frequently in presymptomatic siblings than those in asymptomatic ones (42.9% vs 7.1%, P = 0.049). In all relatives, other diagnostic tests including electroencephalography, electromyography and head magnetic resonance imaging (MRI) for subclinical nervous system involvement and Kayser-Fleischer rings examination yielded normal results. In pre/asymptomatic siblings, genetic and biochemical studies may aid to initiate treatment prior to the development of permanent tissue damage. Our results indicate that abnormal EPs may signal unique pathological finding in some subjects. Importantly, these abnormalities occur earlier than Kayser-Fleischer rings and MRI lesions. In early stages of WD, EP recordings may, therefore, be used to help decide on treatment initiation and treatment efficacy evaluation. Moreover, EP recordings can readily be added to family screening studies.
Department of Pediatric Neurology, Hacettepe University Hospitals, Ankara, Turkey. mtopcu@gen.hun.edu.tr
Meral
M
Topcu
Mehmet Akif
MA
Topcuoglu
Gulsen
G
Kose
Gulay
G
Nurlu
Guzide
G
Turanli
D000293:Adolescent; D000328:Adult; D002648:Child; D004569:Electroencephalography; D004576:Electromyography; D005071:Evoked Potentials; D016057:Evoked Potentials, Auditory, Brain Stem; D005073:Evoked Potentials, Somatosensory; D005074:Evoked Potentials, Visual; D005260:Female; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000503:physiopathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male