ABSTRACT

Congenital bilateral perisylvian syndrome (CBPS) is a congenital neuronal migration disorder characterized by epilepsy, pseudobulbar palsy, cognitive deficits and bilateral perisylvian abnormalities at brain imaging. We report a case of a 44-year-old male admitted with refractory seizures and drop attacks and diagnosed with CBPS after detailed history, neurological examination and magnetic resonance imaging (MRI) findings. The patient had been suffering from seizures since the age of 13. He had generalized tonic-clonic seizures once a month and tonic/ atonic drop attacks repeating daily in spite of antiepileptic drug therapy. Neurological examination showed pyramidal signs, predominantly on the right side, pseudobulbar paresis and mild cognitive impairment. Interictal electroencephalogram (EEG) revealed bilateral frontotemporal sharp-slow wave discharges. MRI revealed bilateral perisylvian local cortical thickening and polymicrogyria. Patients with refractory seizures should undergo detailed investigation, including brain imaging for the possibility of having a specific syndrome. Intractable seizures, pseudobulbar signs and mental retardation suggest the diagnosis of CBPS. Brain MRI plays an important role in the diagnosis of this syndrome.

Keywords: Congenital bilateral perisylvian syndrome, drop attack, epilepsy, polymicrogyria