Abstracts

Rationale: Coexistence of idiopathic generalized and focal epilepsies is exceptionally reported. When it occurs, it is unclear whether the two conditions are independent, or the expression of one alters the natural history of the other. Methods: We present a case of juvenile myoclonic epilepsy (JME) followed by late-onset temporal lobe epilepsy. Results: A 49-year-old woman was referred to our epilepsy center at the age of 30. She had a strong family history of epilepsy in her mother, half-sister, and son. She denied a history of febrile seizures, head trauma, or CNS infections. Her seizures had initially been recognized at 14 years of age, and matched the description of myoclonic, typical absence, and generalized-onset tonic-clonic seizures. Her epilepsy has been poorly controlled over the years. On average, she has had daily myoclonic seizures and 1-4 generalized convulsive events a month for most of her recorded history. The diagnosis of JME was documented by several video-EEG monitoring studies between 33 and 35 years of age. She was recorded myoclonias consisting mainly of proximal arm jerks; and generalized convulsive seizures, which were clinically nonlateralizing. These seizures were associated at onset with generalized polyspike or spike-and-wave discharges that were maximal over the frontal regions and variably asymmetric. Interictally, there was a normal background punctated by generalized discharges of similar morphology, and occasional bifrontal spikes. When she was 43, her family noted episodes of jumbled speech and prolonged staring. An EEG showed novel, temporal lead in interictal spikes preceding bursts of generalized discharges. An MRI brain was suggestive of right mesial temporal lobe sclerosis. Subsequently, continuous video-EEG monitoring off medications at the age of 48 disclosed prominent left temporal intermittent rhythmic delta (TIRDA), and abundant bilaterally independent anterior temporal spikes. Bifrontal spikes were noted rarely, and there were no generalized discharges. Several complex partial seizures during which the patient looked dazed, and displayed semipurposeful activity were recorded. Electrographically, these seizures were associated with an apparent right temporal onset in the setting of ongoing left TIRDA activity. No generalized seizures were noted. On later clinic visits, the patient reported only occasional myoclonic seizures compared with past years. She currently undergoes presurgical evaluation for medically intractable temporal lobe epilepsy. Conclusions: This is a rare case of JME coexisting with temporal lobe epilepsy in the same patient. A shared epileptic network is suggested by the appearance of lead in temporal spikes associated with generalized epileptiform discharges, followed in time by TIRDA, temporal interictal spikes and late-onset temporal lobe seizures. In this case, the two conditions appear to be related.