Abstracts

Rationale: Congenital bilateral perisylvian syndrome (CBPS) is a neurological syndrome characterized by pseudobulbar palsy, cognitive deficits and bilateral perisylvian polymicrogyria. Epileptic syndromes in CBPS are often variable and the seizures are poorly controlled in 65%. Seizure reduction was accomplished by corpus callosotomy in three cases of medically refractory epilepsy secondary to CPBS. Methods: All three cases had been aggressively treated by epileptologists with multiple anti-epileptic drugs (AEDs). However, most of the AEDs did not give patients any remarkable benefits in seizure reduction. Then corpus callosotomy was carried out for these three cases. Results: [Case 1] A 42-year-old woman with moderate mental retardation was referred to our epilepsy center because of frequent seizures that seriously affected her daily life. Drop attacks with tonic seizure started at the age of nine and atypical absence came to her when she was fifteen years old. Epileptologists tried many kinds of AEDs to control her seizure events. However, AEDs did not work well for her. An MRI demonstrated typical bilateral perisylvian polymicrogyria. Long-term video-EEG monitoring was also performed and the final diagnosis of Lennox-Gastaut syndrome was made. Then the patient underwent anterior four fifths corpus callosotomy and showed satisfactory outcome, especially in drop attacks and periodic spasms. [Case 2] A mentally retarded 31-year-old man with CBPS was referred to our hospital for intractable epilepsy. An exam showed left sided hemiparesis and mild dysphagia. The patient demonstrated drop attacks of generalized tonic seizures. Then complete corpus callosotomy was performed and gave the patient seizure reduction. Adjustment of AEDs is necessary because he still has frequent simple partial seizures. [Case 3] A 13-year-old girl had been treated by pediatric epileptologists at our epilepsy center. She has left sided hemiparesis and pseudobulbar palsy, which were found when she was an infant. Her tonic spasms started at the age of nine. She fell down with these tonic spasms. An MRI also demonstrated typical bilateral perisylvian polymicrogyria. She underwent anterior four fifths corpus callosotomy, which demonstrated significant seizure reduction. Although she still has tonic spasms, she does not have clusters of the spasms at the moment. Conclusions: Corpus callosotomy was effective for seizure reduction, particularly in drop attacks secondary to CBPS. However, these patients are not completely seizure free at present. Some of the further investigations were carried out in these patients using dense-array EEG, which showed possible foci in two of the three patients. Then we will make a plan for resective surgery or vagus nerve stimulation eventually.