Alternating hemiplegia of childhood: New diagnostic options

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.

Alternating Hemiplegia of Childhood with ATP1A3 Gene Mutation- A Case Report

“…Alternating episodes of hemiplegia occur but sometimes weakness may extend to other side or may occur bilaterally from the very beginning. 6 Reported patient also presented with neurological manifestation in early infancy and had typical alternating episodes of weakness and sometimes bilateral weakness. During the episodes of weakness consciousness usually remains normal and symptoms disappear upon sleep.…”

More Than a Decade of Misdiagnosis of Alternating Hemiplegia of Childhood with Catastrophic Outcome

“…Writhing movements that suggest choreoathetosis could also be an associated feature. Mental slowing occurs early in the disease course, while mental regression and persistent neurological abnormalities could follow late in the course of the disease [ 7 ].…”

“…In some children, motor dysfunctions caused wheelchair-dependency, but others were able to have an independent life in adulthood. As patients become older, hemiplegic spells and abnormal ocular movements become less common and hypotonia less severe [ 7 ].…”

A novel de novo mutation inATP1A3and childhood-onset schizophrenia

“…AHC (Heinzen et al 2012; Ishii et al 2013) is characterized by paroxysmal eye movements with onset in the first few months of life and paroxysmal focal dystonia and flaccid alternating hemiplegia with onset before 18 mo (Gergont and Kaciński 2014). Symptoms of AHC are often episodic and may be triggered by physical stressors such as extreme change in body temperature, fatigue, and infection, as well as psychological or emotional stressors (Brashear et al 2007).…”