Abstracts

Rationale: Anterior Cingulate Epilepsy (ACE) is a diagnostic and therapeutic challenge with a broad range of nonspecific symptoms such as emotional or behavioral changes. Scalp EEG and brain MRI may not be helpful because of the deep midline location. The goal of this report is to demonstrate the utility of a multimodal approach to diagnose ACE. Methods: We retrospectively identified two pediatric patients with ACE who were initially misdiagnosed with panic disorder and referred to Comprehensive Epilepsy Center at Florida Hospital for Children. Each patient underwent comprehensive evaluation including long-term video-EEG, 3T-MRI, FDG-PET, subtraction ictal SPECT co-registered to MRI (SISCOM), and neuropsychological testing.Results: Patient #1 was a 3 year old, previously healthy female who presented with daily episodes of intense fear coupled with screaming and inconsolable crying that developed 5 weeks prior to admission. She was initially diagnosed with panic attacks by her pediatrician. Short-term EEG and 1.5T-MRI were reported as normal. Her typical episode was captured during long-term video-EEG monitoring that localized ictal onset to the right frontal area. 3T-MRI revealed High T2 signal in the right medial frontal lobe cingulate gyrus (Figure 1A). SISCOM demonstrated a significant focal hyperperfusion in right medial parafalcine frontal lobe/cingulate gyrus (Figure 1B). FDG-PET scan showed hypometabolism in bilateral medial frontal areas. Neuropsychological test scores were normal. The patient has been seizure-free since treatment with levetiracetam was initiated. Patient #2 is a 13 year old male with a history of recurrent seizures since age of 7. He was initially diagnosed with panic disorder by his primary neurologist. Seizures were characterized by a sudden frightened facial grimace followed by hyperventilation, and repetitive slapping of his wrist and walking in circles. Short-term EEG and brain MRI were reported as normal multiple times in the past. His typical episode was correlated with ictal EEG pattern arising from left frontal and midline vertex areas on long-term video-EEG monitoring (Figure 2A). Ictal SPECT and corresponding SISCOM demonstrated focal hyperperfusion in the left medial parafalcine frontal lobe (Figure 2B). Neuropsychological evaluation revealed cognitive impairments associated with frontal lobe dysfunction. He has been seizure-free for 4 months with topiramate monotherapy. Conclusions: ACE is difficult to diagnose because of diverse clinical manifestations, and can be misdiagnosed as generalized anxiety, behavioral problems, or other psychiatric disorders that may occur commonly in children. Early identification of symptoms as seizures can improve outcomes in cognition, behavior, and quality of life. However, because of the deep midline location, routine EEG and/or imaging alone may not be sufficiently sensitive for accurate diagnosis. Our findings indicate that combined multimodal approach is useful to diagnose and localize cingulate epilepsy.