Familial mesial temporal lobe epilepsy (MRI)

Coronal T1-IR and T2-weighted images of 3 siblings (III-4 and III-5 are dizygotic twins) with familial mesial temporal lobe epilepsy, from the family represented in the pedigree. Hippocampal atrophy with hyperintense T2 signal and abnormal hippocampal internal structure is identified in these 3 individuals. Patient III-3 is a 50-year-old man who had complex partial seizures with rare secondary generalization since age 4 years and has not received medication for several years. He has sporadic partial seizures only when he drinks alcohol. His MRI shows left hippocampal atrophy. Patient III-4 is a 47-year-old woman who had a simple febrile seizure when she was 2 years old. She started having complex partial seizures with occasional secondary generalization at age 9 years. She has frequent seizures, refractory to medication, and her MRI shows bilateral hippocampal atrophy. Patient III-5 is a 47-year-old woman, the twin sister of patient III-3, who has only had a few seizures in her life. The first complex partial seizure with secondary generalization was at 8 years. She never took anti-epileptic medications, and her sporadic seizures are associated with major stress or alcohol intake. Her MRI showed bilateral hippocampal atrophy with left side predominance. (Reproduced with permission from Neurology 2003;60(3):405-9.)