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. [1]

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.