Alternating hemiplegia of childhood: An electroclinical study of sleep and hemiplegia.

Josephine Poole, Sara Zagaglia, Rita Demurtas, Fiona Farrell, Matthew C Walker, Sanjay M Sisodiya, Simona Balestrini, Umesh Vivekananda

Journal: PloS one 2022;17(9):e0268720

PMID: 36178910

Abstract

OBJECTIVE

Alternating Hemiplegia of Childhood (AHC) is characterised by paroxysmal hemiplegic episodes and seizures. Remission of hemiplegia upon sleep is a clinical diagnostic feature of AHC. We investigated whether: 1) Hemiplegic events are associated with spectral EEG changes 2) Sleep in AHC is associated with clinical or EEG spectral features that may explain its restorative effect.

METHODS

We retrospectively performed EEG spectral analysis in five adults with AHC and twelve age-/gender-matched epilepsy controls. Five-minute epochs of hemiplegic episodes and ten-minute epochs of four sleep stages were selected from video-EEGs. Arousals were counted per hour of sleep.

RESULTS

We found 1) hemispheric differences in pre-ictal and ictal spectral power (p = 0.034), during AHC hemiplegic episodes 2) 22% reduced beta power (p = 0.017) and 26% increased delta power (p = 0.025) during wakefulness in AHC versus controls. There were 98% more arousals in the AHC group versus controls (p = 0.0003).

CONCLUSIONS

There are hemispheric differences in spectral power preceding hemiplegic episodes in adults with AHC, and sleep is disrupted.

SIGNIFICANCE

Spectral EEG changes may be a potential predictive tool for AHC hemiplegic episodes. Significantly disrupted sleep is a feature of AHC.

Address: Department of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, London, United Kingdom.; Chalfont Centre for Epilepsy, Chalfont St. Peter, United Kingdom.; Neurology and Stroke Unit, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.; Children's Hospital A. Meyer, University of Florence, Paediatric Neurology, Neuroscience Department, Florence, Italy.
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