Non-Stationary Outcome of Alternating Hemiplegia of Childhood into Adulthood

Domenica Immacolata Battaglia, Marco Perulli, Josephine Poole, Sasha D'Ambrosio, Katri Silvennoinen, Sara Zagaglia, Diego Jiménez-Jiménez, Sanjay M. Sisodiya, Simona Balestrini

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review

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.
Lingua originaleEnglish
pagine (da-a)206-211
Numero di pagine6
RivistaMovement Disorders Clinical Practice
Volume9
DOI
Stato di pubblicazionePubblicato - 2022

Keywords

  • ATP1A3
  • adult
  • alternating hemiplegia of childhood
  • movement disorders
  • regression

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