KEGG   DISEASE: H00998Help
Entry
H00998                      Disease                                

Name
Alternating hemiplegia of childhood
Description
Alternating hemiplegia of childhood (ACH) is a rare disorder with onset before 18 months of age. The earliest manifestations of AHC are recurrent bilateral attacks of hemiplegia lasting minutes to days, abnormal ocular movements, and autonomic disturbances. A characteristic feature of AHC is the disappearance of all abnormalities when the child falls asleep.
Category
Nervous system disease
BRITE hierarchy
Pathway
Aldosterone-regulated sodium reabsorption
Endocrine and other factor-regulated calcium reabsorption
Proximal tubule bicarbonate reclamation
Gene
ATP1A2 [HSA:477] [KO:K01539]
Env factor
Exposure to cold
Emotional stress
Fatigue
Bathing
Hyperthermia/hypothermia
Upper respiratory infection
Drug
Flunarizine [DR:D07971]
Comment
Hemiplegic migraine (H00775) and alternating hemiplegia of childhood (AHC) share some clinical features.
Other DBs
ICD-10: 
MeSH: 
OMIM: 
Reference
PMID:8496742 (description)
  Authors
Bourgeois M, Aicardi J, Goutieres F
  Title
Alternating hemiplegia of childhood.
  Journal
J Pediatr 122:673-9 (1993)
Reference
PMID:17880649 (description, drug, env_factor)
  Authors
Neville BG, Ninan M
  Title
The treatment and management of alternating hemiplegia of childhood.
  Journal
Dev Med Child Neurol 49:777-80 (2007)
Reference
PMID:15286158 (description, gene)
  Authors
Bassi MT, Bresolin N, Tonelli A, Nazos K, Crippa F, Baschirotto C, Zucca C, Bersano A, Dolcetta D, Boneschi FM, Barone V, Casari G
  Title
A novel mutation in the ATP1A2 gene causes alternating hemiplegia of childhood.
  Journal
J Med Genet 41:621-8 (2004)
Reference
PMID:14667076 (description)
  Authors
Kanavakis E, Xaidara A, Papathanasiou-Klontza D, Papadimitriou A, Velentza S, Youroukos S
  Title
Alternating hemiplegia of childhood: a syndrome inherited with an autosomal dominant trait.
  Journal
Dev Med Child Neurol 45:833-6 (2003)
Reference
PMID:18498393 (description)
  Authors
de Vries B, Stam AH, Beker F, van den Maagdenberg AM, Vanmolkot KR, Laan L, Ginjaar IB, Frants RR, Lauffer H, Haan J, Haas JP, Terwindt GM, Ferrari MD
  Title
CACNA1A mutation linking hemiplegic migraine and alternating hemiplegia of childhood.
  Journal
Cephalalgia 28:887-91 (2008)

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