Alternating hemiplegia of childhood: a syndrome inherited with an autosomal dominant trait
Article first published online: 13 FEB 2007
Developmental Medicine & Child Neurology
Volume 45, Issue 12, pages 833–836, December 2003
How to Cite
Kanavakis, E., Xaidara, A., Papathanasiou-Klontza, D., Velentza, S. and Youroukos, S. (2003), Alternating hemiplegia of childhood: a syndrome inherited with an autosomal dominant trait. Developmental Medicine & Child Neurology, 45: 833–836. doi: 10.1111/j.1469-8749.2003.tb00899.x
- Issue published online: 13 FEB 2007
- Article first published online: 13 FEB 2007
- Accepted for publication 30th May 2003.
Alternating hemiplegia of childhood is a rare disorder characterized by recurrent attacks of hemiplegia affecting either side of the body, oculomotor and autonomic disturbances, movement disorders, and progressive cognitive impairment. We report on one family with autosomal dominant alternating hemiplegia. The disorder was first recognized in a 9-year-old child, the third son of the family, who presented with learning disability,* tonic-clonic seizures, dystonic attacks, and episodes of alternating hemiplegia starting at the age of 21/2 years. His mother and three brothers had similar symptoms. The maternal uncle, who has learning disability, had experienced multiple dystonic attacks. Tests performed on the family, including computerized tomography, magnetic resonance imaging, and magnetic resonance angiography of the brain as well as metabolic evaluation, were normal. Cytogenetic analysis was normal and mitochondrial DNA analysis revealed no deletions or mutations in the four affected family members and the grandmother. An autosomal dominant mode of inheritance is suggested by the fact that both sexes are affected in two generations.