Topiramate: A new Agent for Patients with Alternating Hemiplegia of Childhood

Abstract

Alternating hemiplegia of childhood is a rare syndrome characterized by the onset, before 18 months of age, of frequent attacks of alternating paralysis. Here we report the efficacy of topiramate in four patients with alternating hemiplegia of childhood (AHC) that did not respond to flunarizine, as well as in two newly diagnosed patients. Following treatment with topiramate, the frequency and duration of hemiplegic attacks significantly improved in all patients. Additional symptoms such as seizures, migraine, involuntary movements, autonomic symptoms, and impaired mental development also improved. Topiramate is worth trying when treating patients with AHC as a first trial, or a substitute for flunarizine once the latter agent loses effect.

Alternating Hemiplegia of Childhood in Chinese Following Long-Term Treatment With Flunarizine or Topiramate

“…Topiramate has been shown to reduce the frequency and severity of the paroxysmal hemiplegic episodes [5]. Our previous study also reported a similar positive effect of topiramate on Chinese Children with AHC [6]. In our study, three patients who were responsive to topiramate therapy showed favorably amelioration of hemiplegic and dystonic episodes.…”

“…Though standard therapy for AHC remains unclear with evidence suggesting no improvement for most patients treated with empiric drugs, flunarizine, an unselective blocker of voltage-dependent calcium channels, has been widely recommended for AHC treatment [3,4]. Topiramate, an agent effective in the prophylactic treatment of epilepsy and migraine, has also been reported to be a promising therapy drug for patients of AHC [5,6].…”

Alternating Hemiplegia of Childhood: Early Characteristics and Evolution of a Neurodevelopmental Syndrome

“…Proven therapies for amelioration of episode frequency and duration are extremely limited, and the long-term impact of apparently even the most broadly effective agent, flunarizine, is essentially unknown. [36][37][38][39][40] Even those medications considered to be most useful by clinicians and families, including flunarizine and the benzodiazepines, have yet to be evaluated in prospective, randomized, placebocontrolled trials. 7,41-43 Thus, at present, there is little to no justification for the uniform initiation of any particular therapeutic agent for all patients with AHC.…”

The Expanding Spectrum of Neurological Phenotypes in Children With ATP1A3 Mutations, Alternating Hemiplegia of Childhood, Rapid-onset Dystonia-Parkinsonism, CAPOS and Beyond

“…69,70 Like flunarizine, reports of benefit have been largely anecdotal. 16 It too blocks voltage-dependent Na + channels; in addition, it is an antagonist of non-N-methyl D-aspartate glutamate and calcium channel receptors and is a carbonic anhydrase inhibitor.…”