Abstract
Evoked potential testing was done in 34 adolescent patients with hyperphenylalaninemia (21 type 1, 8 type 2, 4 type 3, 1 DHPR deficiency) and in 35 control persons. In all but 4 of the type 1 and 2 patients the dietary treatment had been started during the first 12 weeks of life. In 22 the diet had been terminated at the age of 10.1 +1.6 years (range 6 - 13 years). 8 patients were still on diet at the time of this investigation. In the pattern reversal VEP a prolongation of the latency of wave P100 was found (104.3 ± 6.2 ms vs. 99.3 ± 3.4 ms, p<0.001). This prolongation correlated significantly with the quality of diet during the first 10 years of life. Controling for the quality of diet no influence of the age at which the diet was terminated was demonstrated. There were no correlations between the VEP findings and the Phe concentration at later ages. Click-evoked BAEPs were normal with regard to the latency and shape of waves I through V. The only difference between patients and control persons was an increase of the interear difference of the interwave lateny I-V.
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Korinthenberg, R., Ullrich, K. & Füllenkemper, F. 13 VISUAL AND AUDITORY EVOKED POTENTIALS IN ADOLESCENTS WITH HYPERPhENYLALANINEMIA. Pediatr Res 24, 263 (1988). https://doi.org/10.1203/00006450-198808000-00039
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DOI: https://doi.org/10.1203/00006450-198808000-00039