Perulli, M;
Poole, J;
Di Lazzaro, G;
D'Ambrosio, S;
Silvennoinen, K;
Zagaglia, S;
Jimenez-Jimenez, D;
... Balestrini, S; + view all
(2021)
Non-Stationary Outcome of Alternating Hemiplegia of Childhood into Adulthood.
Movement Disorders Clinical Practice
10.1002/mdc3.13388.
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Abstract
Background Although described as non-progressive, alternating hemiplegia of childhood (AHC) can display a sudden deterioration, anecdotally reported mainly in childhood. Outcome in adulthood is uncertain. Objectives Aim of this study is to describe the long-term follow-up of neurological function in adults with AHC. Methods Seven adults with AHC were included in this retrospective single-center study. Clinical history and previous investigation data were gathered from the review of medical records. Video-documented neurological examination was performed at the last follow-up visit in four out of the seven reported indivisuals. Results Over a median follow-up of 16 years, neurological outcome and trajectories were heterogeneous. All individuals showed new neurological signs or symptoms. Three experienced a serious irreversible neurological deterioration after prolonged quadriplegic episodes and/or status epilepticus in their second or third decade. One patient died at age 29. Conclusions This video-series suggests that AHC in adulthood is not stationary; larger cohorts are needed to identify genotype–phenotype correlations and clinically useful outcome predictors.
| Type: | Article |
|---|---|
| Title: | Non-Stationary Outcome of Alternating Hemiplegia of Childhood into Adulthood |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1002/mdc3.13388 |
| Publisher version: | https://doi.org/10.1002/mdc3.13388 |
| Language: | English |
| Additional information: | This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third-party material in this article are included in the Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/ |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Clinical Neurology, Neurosciences & Neurology, alternating hemiplegia of childhood, adult, movement disorders, regression, ATP1A3, MUTATIONS, ENCEPHALOPATHY, CHILDREN |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Experimental Epilepsy |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10141676 |
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