A 13-year-old girl with Rasmussen syndrome diagnosed at 5 years, and right-sided seizures refractory to various therapies, including hemispherectomy, responded to thalidomide and is reported from Belgrade, Yugoslavia. Initial control with carbamazepine, valproate and clonazepam was followed by relapse and a more extensive right hemiparesis. Despite resection of the cortical epileptogenic zones in the left hemisphere at 9 years of age, and trials of different anticonvulsants, acyclovir, g-globulin, iv methylprednisolone, oral prednisone, and plasma exchange, seizure frequency increased. MRI showed atrophy and cystic changes in the left hemisphere with compensatory dilatation of the lateral ventricle. Thalidomide (300 mg/day) was added to a midazolam iv drip, and partial seizure control was sustained. For the past 3 years, her seizures have been less intense and less frequent and have not interfered with everyday activities. Treatment includes thalidomide 5 mg/kg, valproate 30 mg/kg, clonazepam and piracetam. Apart from occasional leukopenia, no other adverse effects are reported. 
COMMENT. Thalidomide may be considered as an alternative treatment for refractory and incapacitating seizures in Rasmussen syndrome, when more conventional therapies have failed. One previous report found thalidomide effective in a 7-year-old male with Rasmussen syndrome whose seizures were associated with high levels of CSF tumor necrosis factor a. (Ravenscroft A et al. Brain Dev 1998;20:398). The antiepileptic effect may be related to inhibition of the tumor necrosis factor and boosting of the immune response. Apart from the high teratogenicity, a sensory neuropathy is the major adverse complication of thalidomide therapy.