Botulinum toxin A improves involuntary limb movements in Rasmussen syndrome

Rasmussen syndrome (RS) is a focal, progressive, cortical inflammation affecting one cerebral hemisphere that usually presents with epilepsia partialis continua (EPC) that is resistant to antiepileptic drugs (AEDs). The encephalitis is often associated with ipsilateral cerebral hemisphere and basal ganglia atrophy.1 Patients can develop involuntary movements, including myoclonus, dystonia, and athetosis, usually in association with EPC.2 The encephalitis is probably autoimmune mediated, although the pathogenesis of the atrophy is unknown.3 We report a man with established RS who developed EPC and painful dystonia that improved with botulinum toxin A (BTX-A). We described previously this 43-year-old, left-handed man.4 He sought treatment in 1989 for EPC followed by neurologic and cognitive deficits. The following therapies had no sustained effect: multiple AEDs, right corticectomy in 1990, infusions of sodium valproate or benzodiazepines into the corticectomy cavity in 1994, and right frontoparietal subcortical transection in 1995. Immunomodulatory therapy started in 1996, and his condition improved, although he continued to have intermittent bouts of left upper limb myoclonic jerking.4 He was stable until 2002 …