Studies of multimodal evoked potentials in treated phenylketonuria: the pattern of vulnerability.
We performed studies of multimodal evoked potentials and peripheral sensory and motor nerve conductions in 33 early and 6 late treated patients with phenylketonuria. The studies revealed the following picture: 1. In 27% of early treated patients latencies of visual evoked potentials were increased. The cause for these changes in unknown. 2. Nerve conduction studies showed the presence of a minor sensory neuropathy which in rare cases may also affect peripheral motor nerves. This neuropathy did not have features of a central-peripheral distal axonopathy which argues against a toxic/nutritional causation. 3. Deficits in the central sensory, motor, and auditory pathways were present, but rare in early treated patients. If the results of electrophysiological studies reported by different groups are compared, the emerging picture is very similar and the majority of the-minor-differences is likely to be explained by technical aspects.
Ludolph
A C
AC
Department of Neurology, Humboldt University, Berlin, Germany.
Vetter
U
U
Ullrich
K
K
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D005071:Evoked Potentials; D005072:Evoked Potentials, Auditory; D005073:Evoked Potentials, Somatosensory; D005074:Evoked Potentials, Visual; D005260:Female; D006801:Humans; D008297:Male; D010661:Phenylketonurias / Q000178:diet therapy / Q000503:physiopathology*