Abstracts

RATIONALE: Bilateral frontal polymicrogyria (BFP) is a recently described malformation of cortical development, characterized by symmetrical polymicrogyria of both frontal lobes back to precentral sulcus (Guerrini et al., 2000). The main clinical features are developmental delay, spastic quadriparesis, impaired language development, mental retardation, and epilepsy. Epilepsy can show variable ages of onset and severity. We report the electro-clinical features of two adult patients with BFP and severe drug-resistant epilepsy.
METHODS: Patient 1 was a 24-year-old female. Maternal toxoplasmosis at 3 months of pregnancy was diagnosed. The patient presented with bilateral glaucoma and cataract, mental retardation, mild bilateral pyramidal syndrome. At 10 years of age, she started to suffer from a drug-resistant epilepsy characterized by partial seizures with automatisms, tonic seizures, and frequent episodes of partial status. Patient 2 was a 30-year-old male with mild mental retardation and psychotic disturbances. At the age of 6 years, learning difficulties and behavioral disorders were noted. At 7 years, he started to suffer from daily seizures characterized by loss of consciousness, and massive tonic contraction; motor manifestations could often follow. Occasional tonic-clonic seizures could also occur. The seizures were poorly controlled by antiepileptic treatment. Both patients underwent awake and sleep video-EEG/polygraphic recording, computerized video-EEG monitoring, brain MRI, interictal SPECT (in patient 1).
RESULTS: In both patients, interictal EEG was characterized by diffuse, with frontal predominance, paroxysmal activities; in patient 1, multifocal (left frontal and asyncronous bilateral temporal) spikes were present as well. In patient 1, ictal video-EEG showed seizures characterized by a massive tonic contraction, followed by left head version and mild pedaling movement of the lower limbs; in the EEG, an initial diffuse flattening was observed, followed by diffuse rhythmic spike activity, with maximal amplitude over the fronto-central regions, then anteriorly predominant spike-waves complexes appeared. In patient 2, video-EEG allowed the recording of seizures with a diffuse tonic contraction followed by hypermotor phenomena. Ictal EEG showed an initial diffuse slow wave complex, that preceded a low amplitude fast rhythmic activity that was followed by diffuse slow spikes and waves abnormalities. In both patients, brain MRI showed BFP, that in patient 1 was associated with cerebellar vermis agenesia. In patient 1, interictal SPECT showed by bilateral frontal hypofixation.
CONCLUSIONS: The electro-clinical features of epilepsy associated with BFP has been not clearly described yet. Indeed, a spectrum of epileptic disorder has been reported. Both adult cases that we observed showed similar electro-clinical epileptic features, suggesting the involvement of frontal structures. Neurologically, they differed from the data of the literature for the mild (patient 1) or absent (patient 2) motor impairment.