Non-Stationary Outcome of Alternating Hemiplegia of Childhood into Adulthood

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.

Automated summary

This study followed up on 7 adults with alternating hemiplegia of childhood (AHC) and found heterogeneous neurological outcomes over a median of 16 years. Three patients experienced serious irreversible neurological deterioration after prolonged quadriplegic episodes and/or status epilepticus in their second or third decade. Further research with larger cohorts is needed to identify genotype-phenotype correlations and clinically useful outcome predictors.