alternating hemiplegia of childhood
alternating hemiplegia of childhood
A rare (1:106) idiopathic condition characterised by transient attacks of hemiplegia, ranging in severity from mild weakness to complete uni- or (less commonly) bilateral paralysis.Management
• Avoid triggers—e.g., temperature changes, water exposure, bright lights, certain foods, emotional stress, physical activity.
• Flunarizine (calcium channel blocker), benzodiazepines, anti-epileptics.
Prognosis
Poor with cognitive impairment, behavioural and psychiatric disorders, and various motor defects.
Molecular pathology
Some cases have an ATP1A2 mutation.
Alternating hemiplegia of childhood, diagnostic criteria
• Onset before 18 months of age;
• Attacks of both hemiplegia on either side of the body;
• Other autonomic problems—e.g., episodic monocular nystagmus, (improper eye alignment), choreoathetosis;
• Sustained muscle contractions (dystonia);
• Mental retardation, delayed development and other neurological abnormalities;
• Symptoms disappear when sleeping;
• Episodes of bilateral hemiplegia that shift from one side of the body to the other.