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hemiplegia alterans (alternating hemiplegia)
Etiology: idiopathic
Epidemiology:
- develops in childhood, generally in 1st 4 years of age
Genetics:
- associated with defects in ATP1A2
Clinical manifestations:
1) recurrent episodes of hemiplegia
2) paralysis can affect eye movements, limbs, or facial muscles
3) mental retardation
4) balance & gait difficulties
5) hyperhidrosis
6) changes in body temperature
7) sleep helps in the recovery from the periods of paralysis but the paralysis can recur upon waking
8) variant form
a) occurs primarily at night, when a child awakens
b) related to migraine
c) no other mental or neurological impairments
9) shares features with familial hemiplegic migraine 2
10) typically distinguished from familial hemiplegic migraine by
a) infantile onset of symptoms
b) high prevalence of associated neurological deficits that become increasingly obvious with age
Management:
1) flunarizine may reduce the severity & duration of attacks of paralysis
2) prognosis
a) good prognosis with variant form
b) more severe form associated with poor prognosis
1] mental retardation not responsive to drug therapy
2] balance and gait problems persist
3] over time, walking unassisted becomes difficult or impossible
Related
hemiplegia (HP)
General
developmental disorder
genetic disease of the central nervous system
Database Correlations
OMIM 104290
References
- NINDS Alternating Hemiplegia Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Alternating-Hemiplegia-Information-Page
- OMIM :accession 104290