Alternating hemiplegia of childhood is a rare disorder characterized b
y episodic paroxysmal attacks of neurologic dysfunction and has histor
ically been thought to represent a migraine equivalent, an unusual for
m of epilepsy, or a movement disorder. Medical treatment with a variet
y of anticonvulsants and migraine agents is largely unsuccessful, and
progressive mental deterioration is universally expected. Despite exte
nsive laboratory studies, the pathophysiologic basis of this condition
has thus far eluded identification. Recently, an international worksh
op was held to generate testable hypotheses regarding the pathophysiol
ogy of alternating hemiplegia of childhood. This paper summarizes the
major proceedings of that workshop, and hopes to stimulate further int
erest in elucidating the molecular and cellular mechanisms underlying
this unusual disorder.