Short-latency somatosensory-evoked potentials demonstrate cortical dysfunction in patients with Angelman syndrome
| Autor: | Hideaki Shiraishi, Kiyoshi Egawa, Shinji Saitoh, Naoko Asahina |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
VPA
Valproic acid CZP Clonazepam Somatosensory system lcsh:RC346-429 03 medical and health sciences 0302 clinical medicine Neurodevelopmental disorder Angelman syndrome Somatosensory-evoked potentials GABARs GABAA receptor subunit genes medicine UBE3A SEFs Somatosensory-evoked fields 030212 general & internal medicine Latency (engineering) lcsh:Neurology. Diseases of the nervous system SSEPs Short-latency somatosensory-evoked potentials medicine.diagnostic_test GABAergic systems business.industry AS Del AS patients with a deletion in 15q11-q13 Neurodevelopmental disorders Magnetoencephalography medicine.disease AS Angelman syndrome Neurology Somatosensory evoked potential GABAergic Original Article business CCT Central conduction time Neuroscience 030217 neurology & neurosurgery |
| Zdroj: | eNeurologicalSci eNeurologicalSci, Vol 22, Iss, Pp 100298-(2021) |
| ISSN: | 2405-6502 |
| Popis: | Background Angelman syndrome (AS) is neurodevelopmental disorder, causal gene of which is maternally expressed UBE3A. A majority of patients results from the large deletion of relevant chromosome which includes GABAA receptor subunit genes (GABARs) as well as UBE3A (AS Del). We previously reported aberrantly desynchronized primary somatosensory response in AS Del by using magnetoencephalography. The purpose of this study is to estimate cortical and subcortical involvement in the deficit of primary somatosensory processing in AS. Methods We analyzed short-latency somatosensory-evoked potentials (SSEPs) in 8 patients with AS Del. SSEPs were recorded on a 4-channel system comprising of two cortical electrodes which were placed on the frontal and centro-parietal areas. The peak and onset latency of each component were measured to compare latency and interval times. Results The first-cortical peak latency (N20, P20), and N13-N20 peak interval times were significantly prolonged in AS Del compared to healthy controls. In contrast, there was no difference in latencies between subcortical components up to N20 onset or for N11-N20 onset interval times. Conclusion Highly desynchronized first-cortical SSEP components and normal latencies of subcortical components indicated cortical dysfunction rather than impairment of afferent pathways in AS Del patients, which might be attributed to GABAergic dysfunction due to loss of UBE3A function and heterozygosity of GABARs Highlights • Somatosensory-evoked potentials (SEPs) were evaluated in Angelman syndrome (AS). • All subjects had a 15q11-13 deletion, which includes the GABAA receptor subunit genes. • The duration of the first-cortical SEP components was significantly prolonged. • Latencies between subcortical components were comparable to controls. • Desynchronized cortical response suggests GABAergic dysfunction in AS with deletion. |
| Databáze: | OpenAIRE |
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