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Familial mesial temporal lobe epilepsy (MRI)

Coronal T1-IR and T2-weighted images of three 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 three individuals. Patient III-3 is a 50-year-old man who had focal impaired awareness seizures with rare focal to bilateral tonic-clonic seizures since the age of 4. He has not received medication for several years and has sporadic focal aware 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 focal impaired awareness seizures, occasionally evolving with focal to bilateral tonic-clonic seizures, at the age of 9. She has frequent seizures that are 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 had only had a few seizures in her life. The first focal impaired awareness seizure with bilateral tonic-clonic seizure was at 8 years old. She never took antiseizure 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.)