Abstracts

Cingulate Gyrus Epilepsy (CGE) is a rare form of frontal lobe epilepsy whose electroclinical syndromes have not been thoroughly documented and validated. Since the early descriptions of 36 patients by Mazars and 16 by Bancaud and Talairach, only 7 cases have been published, with no general agreement as to its clinical manifestations. Bancaud emphasized intense fright, shouts, vigorous and aggressive vocalization, followed by archaic complex motor behavior while Mazars noted pseudo-absences, head nods, adversive head movements, blushing and generalized convulsive seizures as characteristic of CGE.
We studied the semiology of 4 patients whose brain imaging and intracranial recordings with strip, grid or stereotactically implanted electrodes identified lesional zones and electrographic onset of seizures in the anterior cingulate gyrus in order to: (1) describe the semiology of anterior cingulate epilepsy, and (2) compare their semiology with those previously reported by Mazars, Bancaud, and 6 case reports.
We reviewed the clinical records, CCTV-EEGs, intracranial recordings (grids or strips recordings, stereoEEGs and ECOGs), brain imaging, neuropsychological tests and neuropathology of 4 patients with anterior CGE who underwent resection of epileptogenic zones.
Brain imagings were normal in patients 1 and 4. Patient 2 had a left mesial frontal MRI hyperintensity and PET hypometabolism. Patient 3 had a calcified left cingulate cavernous angioma on MRI, left cingulate gyrus ametabolism and right temporal hypometabolism on PET, and temporal spiking on MEEG.
Neuropathology revealed discrete neuronal loss and cortical gliosis in patients 1 and 4, arachnoiditis and atrophy of mesial frontal cortex in patient 2, and a calcified cavernous angioma with gliosis in patient 3.
As described by Mazars et al, all 4 patients had a sudden stare with unresponsiveness. 2 of 4 had vocalization or grunting while 3 had tonic or clonic limb jerks. 2 of 4 had adversive head movements. All 4 had early complex motor automatisms e.g. rocking, pelvic thrusting, conducting and walking, followed by mastication and lip smacking in 2 patients. 3 of 4 had urinary incontinence and somatomotor postures. Extreme fear as described by Bancaud was observed in only 2 of 4 patients. 2 had difficulty verbally expressing themselves prior to seizures.
The clinical manifestations of anterior cingulate gyrus epilepsy are no different from the clinical manifestations of mesial frontal epilepsy involving the supplementary motor area or the mesial prefrontal cortex except for the extreme fear and vocalization considered characteristic of anterior cingulate seizures.
[Supported by: Dr. Perez-Gosiengfiao was awarded a research fellowship by the International League Against Epilepsy. Her postdoctoral fellowship in epileptology and clinical neurophysiology is supported in part by the Tan Yan Kee Foundation, Inc. ]