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Alternating hemiplegia of childhood
Alternating hemiplegia of childhood (AHC) is a rare genetic disease which causes intermittent paralysis and progressive mental retardation starting in childhood. Additional recommended knowledge
SymptomsAHC usually occurs before four years of age and can present in either a severe or a less severe form.
EtiologySome cases of AHC are thought to be the result of a channelopathy - the gene ATP1A2 (an isoform of the sodium-potassium ATPase) has been implicated in at least one family. There is overlap with the syndrome of familial hemiplegic migraine in which the ATP1A2 gene undergoes a different mutation. TreatmentDrug therapy includes flunarizine, a calcium channel blocker. It may help to reduce the severity and duration of attacks of paralysis associated with the more serious form of alternating hemiplegia. PrognosisChildren with the benign form of alternating hemiplegia have a good prognosis. However, those who experience the more severe form have a poor prognosis because intellectual and mental capacity do not respond to drug therapy, and balance and gait problems continue. Over time, walking unassisted becomes difficult or impossible. References
Categories: Genetic disorders | Neurological disorders | Pediatrics |
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This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Alternating_hemiplegia_of_childhood". A list of authors is available in Wikipedia. |