Evoked Potentials in Neuronal Ceroid Lipofuscinosis
- 1 January 1979
- journal article
- research article
- Published by S. Karger AG in European Neurology
- Vol. 18 (4), 234-242
- https://doi.org/10.1159/000115082
Abstract
Nerve conduction, EEG, visual evoked potentials, electroretinograms and somatosensory evoked potentials were investigated in 3 children with the Bielschowsky-Jansky-type and in 1 child diagnosed as Spielmeyer-Vogt-type of neuronal ceroid lipofuscinosis. Electroretinograph-ic responses were abolished in all of them. Electroencephalograms showed high amplitude, irregular delta-theta activity and spike- or polyspike-wave discharges without localized preponderance. As a characteristic feature for the Bielschowsky-Jansky type grossly enlarged evoked responses to single light flashes were recorded. Somatosensory evoked potentials were increased in amplitude in 2 patients. Myoclonic jerks of the pyramidal type could be elicited by electrical stimuli to the median nerve. The possibility to differentiate certain neurometabolic disorders of childhood by simple electrophysiological parameters is discussed.Keywords
This publication has 1 reference indexed in Scilit:
- So-called neuronal ceroid lipofuscinosis: Neurophysiological studies in 60 childrenJournal of Neurology, Neurosurgery & Psychiatry, 1977