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.

Perulli, M., Poole, J., Di Lazzaro, G., D'ambrosio, S., Silvennoinen, K., Zagaglia, S., Jimenez-jimenez, D., Battaglia, D., Sisodiya, S. M., Balestrini, S., Non-Stationary Outcome of Alternating Hemiplegia of Childhood into Adulthood, <<MOVEMENT DISORDERS CLINICAL PRACTICE>>, 2022; 9 (2): 206-211. [doi:10.1002/mdc3.13388] [http://hdl.handle.net/10807/197164]

Non-Stationary Outcome of Alternating Hemiplegia of Childhood into Adulthood

Perulli, M.;Battaglia, D.;
2022

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.
Inglese
Perulli, M., Poole, J., Di Lazzaro, G., D'ambrosio, S., Silvennoinen, K., Zagaglia, S., Jimenez-jimenez, D., Battaglia, D., Sisodiya, S. M., Balestrini, S., Non-Stationary Outcome of Alternating Hemiplegia of Childhood into Adulthood, <<MOVEMENT DISORDERS CLINICAL PRACTICE>>, 2022; 9 (2): 206-211. [doi:10.1002/mdc3.13388] [http://hdl.handle.net/10807/197164]
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/10807/197164
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