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Somatosensory evoked potentials with high cortical amplitudes: clinical data in 31 children
B Schmitt1, L Thun-Hohenstein, L Molinari
1Department of Clinical Neurophysiology and Pediatric Neurology, University Children's Hospital, Zürich, Switzerland.
Insights
Enhanced somatosensory evoked potentials (SEP) amplitudes were observed in children with neurologic disorders. These enhanced SEP amplitudes are a useful diagnostic criterion for neuronal ceroid lipofuscinosis.
Area of Science:
- Neuroscience
- Clinical Neurology
Background:
- Somatosensory evoked potentials (SEP) are used in diagnosing pediatric neurologic disorders.
- Enhanced SEP amplitudes can indicate underlying neurological conditions.
Purpose of the Study:
- To investigate the diagnostic value of enhanced SEP amplitudes in children with various neurologic disorders.
- To determine if enhanced SEP amplitudes are specific to certain conditions, particularly neuronal ceroid lipofuscinosis (NCL).
Main Methods:
- Recorded SEP in 282 children with neurologic disorders between 1989 and 1993.
- Analyzed N20/P25/N35 amplitudes and cervico-cortical conduction times.
- Compared findings to a control group and performed follow-up recordings.
Main Results:
- Thirty-one children exhibited enhanced SEP amplitudes.
- All patients with NCL showed enhanced cortical SEPs; five also had prolonged cervico-cortical conduction times.
- Enhanced SEPs were also found in heterogeneous disorders and hemiparesis, indicating non-specificity.
Conclusions:
- Enhanced SEP amplitudes are generally unspecific indicators of neurologic disorders.
- However, enhanced SEP amplitudes may serve as a valuable diagnostic criterion for NCL.
- Prolonged cervico-cortical conduction times were predominantly observed in NCL patients.
Abstract:
Between 1989 and 1993, somatosensory evoked potentials (SEP) were recorded as part of the diagnostic work-up in 282 children with different neurologic disorders. In thirty-one children with N20/P25/N35 amplitudes were enhanced compared to our control group (highest amplitude 14.1 microV). Four children had amplitudes > 40 microV ("giant"), fifteen between 20-39.9 microV ("elevated") and twelve between 14-19.9 microV ("borderline"). Enhanced cortical SEPs were seen in all patients with neuronal ceroid lipofuscinosis (5 late-infantile NCL > 20 microV, 1 juvenile NCL 14.7 microV). In addition, five of six NCL children showed bilaterally prolonged cervico-cortical conduction times, otherwise only seen in a 4-month-old child following hypoxia. "Borderline" and "elevated" SEPs occurred in patients with heterogeneous neurologic disorders. Follow-up recordings showed inconsistent results: seven children had amplitudes > 14 microV in all recordings, six only at the first examination, and six only at follow-up. In six children with hemiparesis enhanced SEPs were recorded over both (n = 2) or only over the unaffected hemisphere (n = 4). Myoclonic seizures were observed only in five children with NCL. Similar to other SEP parameters, enhanced amplitudes are an unspecific indicator of an ongoing neurologic disorder. However, in neuronal ceroid lipofuscinosis, enhanced SEP amplitudes may be a useful diagnostic criterium.