Alternating hemiplegia of childhood

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Alternating hemiplegia of childhood (AHC) is a rare genetic disease which causes intermittent paralysis and progressive mental retardation starting in childhood.

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Alternating hemiplegia of childhood
OMIM 104290
DiseasesDB 33595
eMedicine neuro/219 

Overview

Historical Perspective

Classification

Pathophysiology

Etiology

Some 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.

Causes

Differentiating Alternating hemiplegia of childhood from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural history

Complications

Prognosis

Children 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.

Diagnosis

Diagnostic Criteria

History and Symptoms

AHC usually occurs before four years of age and can present in either a severe or a less severe form.

  • The less severe form has a good prognosis and is indicated by episodes occurring primarily at night, and can often be related to migraines. There is no neurological impairment in this form of AH.

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Drug 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.

Medical Therapy

Surgery

Prevention

Sources

References

nl:Alternating Hemiplegia of Childhood



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