Neurológia pre prax 4/2023

Alternating hemiplegia of childhood

Alternating hemiplegia of childhood (AHC) is a rare genetically transmitted neurological disorder that begins in infancy and presents with a wide range of neurologic symptoms. First manifestations are usually paroxysmal ocular signs and tonic/dystonic attacks. Latter, episodes of transient hemiparesis/hemiplegia on either side of body and quadriplegia develop which are typical for the disorder as well as a relief of symptoms with sleep. Delay in psychomotor development and intellectual disability are apparent and accompanied with extrapyramidal and cerebellar signs. In a half of patients seizures are present. AHC is connected with mutations in the ATP1A3 gene in the majority of cases. Differential diagnosis includes vascular, metabolic and mitochondrial disorders. Causal theraphy is not available, flunarizine is used for prophylactic treatment.

Keywords: alternating hemiplegia of childhood, ATP1A3 gene, quadriplegia, choreoathetosis, dystonia, flunarizine