The Library
Spontaneously fluctuating motor cortex excitability in alternating hemiplegia of childhood : a transcranial magnetic stimulation study
Tools
Stern, William M., Desikan, Mahalekshmi, Hoad, Damon, Jaffer, Fatima, Strigaro, Gionata, Sander, Josemir W., Rothwell, John C. and Sisodiya, Sanjay M. (2016) Spontaneously fluctuating motor cortex excitability in alternating hemiplegia of childhood : a transcranial magnetic stimulation study. PLoS One, 11 (3). e0151667. doi:10.1371/journal.pone.0151667 ISSN 1932-6203.
|
PDF
WRAP-spontaneously-fluctuating-motor-cortex-Hoad-2016.PDF - Published Version - Requires a PDF viewer. Available under License Creative Commons Attribution 4.0. Download (3633Kb) | Preview |
Official URL: https://doi.org/10.1371/journal.pone.0151667
Abstract
Background
Alternating hemiplegia of childhood is a very rare and serious neurodevelopmental syndrome; its genetic basis has recently been established. Its characteristic features include typically-unprovoked episodes of hemiplegia and other transient or more persistent neurological abnormalities.
Methods
We used transcranial magnetic stimulation to assess the effect of the condition on motor cortex neurophysiology both during and between attacks of hemiplegia. Nine people with alternating hemiplegia of childhood were recruited; eight were successfully tested using transcranial magnetic stimulation to study motor cortex excitability, using single and paired pulse paradigms. For comparison, data from ten people with epilepsy but not alternating hemiplegia, and ten healthy controls, were used.
Results
One person with alternating hemiplegia tested during the onset of a hemiplegic attack showed progressively diminishing motor cortex excitability until no response could be evoked; a second person tested during a prolonged bilateral hemiplegic attack showed unusually low excitability. Three people tested between attacks showed asymptomatic variation in cortical excitability, not seen in controls. Paired pulse paradigms, which probe intracortical inhibitory and excitatory circuits, gave results similar to controls.
Conclusions
We report symptomatic and asymptomatic fluctuations in motor cortex excitability in people with alternating hemiplegia of childhood, not seen in controls. We propose that such fluctuations underlie hemiplegic attacks, and speculate that the asymptomatic fluctuation we detected may be useful as a biomarker for disease activity.
Item Type: | Journal Article | ||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Subjects: | R Medicine > RJ Pediatrics > RJ101 Child Health. Child health services | ||||||||||||
Divisions: | Faculty of Science, Engineering and Medicine > Medicine > Warwick Medical School > Health Sciences Faculty of Science, Engineering and Medicine > Medicine > Warwick Medical School > Health Sciences > Social Science & Systems in Health (SSSH) Faculty of Science, Engineering and Medicine > Medicine > Warwick Medical School |
||||||||||||
Library of Congress Subject Headings (LCSH): | Alternating hemiplegia of childhood, Magnetic brain stimulation | ||||||||||||
Journal or Publication Title: | PLoS One | ||||||||||||
Publisher: | Public Library of Science | ||||||||||||
ISSN: | 1932-6203 | ||||||||||||
Official Date: | 21 March 2016 | ||||||||||||
Dates: |
|
||||||||||||
Volume: | 11 | ||||||||||||
Number: | 3 | ||||||||||||
Article Number: | e0151667 | ||||||||||||
DOI: | 10.1371/journal.pone.0151667 | ||||||||||||
Status: | Peer Reviewed | ||||||||||||
Publication Status: | Published | ||||||||||||
Access rights to Published version: | Open Access (Creative Commons) | ||||||||||||
Copyright Holders: | Stern et al. | ||||||||||||
Date of first compliant deposit: | 8 February 2019 | ||||||||||||
Date of first compliant Open Access: | 8 February 2019 | ||||||||||||
RIOXX Funder/Project Grant: |
|
||||||||||||
Open Access Version: |
Request changes or add full text files to a record
Repository staff actions (login required)
View Item |
Downloads
Downloads per month over past year