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.

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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:	Date Event 21 March 2016 Published 2 March 2016 Accepted
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:	Project/Grant ID RIOXX Funder Name Funder ID UNSPECIFIED Epilepsy Society http://viaf.org/viaf/311774814 UNSPECIFIED Marvin Weil Epilepsy Research Fund UNSPECIFIED UNSPECIFIED [NIHR] National Institute for Health Research http://dx.doi.org/10.13039/501100000272
Open Access Version:	Publisher

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