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Alternating hemiplegia of childhood: New diagnostic options
Affiliations- Department of Neurology of Children and Youth, Jagiellonian University, Collegium Medicum, Krakow, Poland
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Abstract
A syndrome of alternating hemiplegia of childhood (AHC) is a rare disorder first presented in 1971. AHC is characterized by transient episodes of hemiplegia affecting either one or both sides of the body. Age of onset is before 18 months and the common earliest manifestations are dystonic or tonic attacks and nystagmus. Hemiplegic episodes last minutes to days and the frequency and duration tend to decrease with time. Motor and intellectual development is affected, deficits may also develop later. Epileptic seizures occur in some patients. Neuroimaging of the brain usually reveals no abnormalities. The variability of individual clinical presentations and evolution of symptoms have made diagnosis difficult. Therefore the problems of misdiagnosis could account for the low prevalence of this syndrome. This paper hopes to present actual data on AHC, especially of the results of genetic research and new diagnostic tools.
Abstract
A syndrome of alternating hemiplegia of childhood (AHC) is a rare disorder first presented in 1971. AHC is characterized by transient episodes of hemiplegia affecting either one or both sides of the body. Age of onset is before 18 months and the common earliest manifestations are dystonic or tonic attacks and nystagmus. Hemiplegic episodes last minutes to days and the frequency and duration tend to decrease with time. Motor and intellectual development is affected, deficits may also develop later. Epileptic seizures occur in some patients. Neuroimaging of the brain usually reveals no abnormalities. The variability of individual clinical presentations and evolution of symptoms have made diagnosis difficult. Therefore the problems of misdiagnosis could account for the low prevalence of this syndrome. This paper hopes to present actual data on AHC, especially of the results of genetic research and new diagnostic tools.
Keywords
Alternating hemiplegia, Children, ATP1A3
About this articleTitle
Alternating hemiplegia of childhood: New diagnostic options
Journal
Neurologia i Neurochirurgia Polska
Issue
Article type
Review Article
Pages
130-135
Page views
545
Article views/downloads
644
DOI
10.1016/j.pjnns.2013.05.003
Bibliographic record
Neurol Neurochir Pol 2014;48(2):130-135.
Keywords
Alternating hemiplegia
Children
ATP1A3
Authors
Aleksandra Gergont
Marek Kaciński
