Portal de Periódicos da CAPES

Interconnections in superior temporal cortex revealed by musicogenic seizure propagation

2012; Springer Science+Business Media; Volume: 259; Issue: 10 Linguagem: Inglês

10.1007/s00415-012-6556-9

1432-1459

Irène Wang, Kazutaka Jin, Yosuke Kakisaka, Richard C. Burgess, Jorge González-Martínez, Shuang Wang, Susumu Ito, John C. Mosher, Stephen Hantus, Andreas V. Alexopoulos,

Neural dynamics and brain function

Musicogenic epilepsy (ME) is an intriguing epilepsy syndrome with estimated prevalence of 1 per million [1]. Seizures are precipitated by complex and usually highly specific stimuli. ME has been the topic of several excellent reviews [2, 3], but reports of comprehensively studied patients are scarce [1]. Notably, ME can provide insights in auditory processing and seizure-triggering mechanisms in humans. We present here a patient with non-lesional ME, whose seizure initiation and propagation shed light on interconnections within the superior temporal cortex. A 42-year-old right-handed professional composer started having seizures at 11 years. Seizures became difficult-to-control after he entered music school. Stereotyped auras were consistently triggered by songs with familiar melody and lyrics. ‘‘Happy Birthday’’ and songs by Elton John were especially provocative, whereas jazz/instrumentals had no effect. Auras consisted of buzzing or muffled sounds several times/week, evolving into dialeptic seizures with staring, unresponsiveness, and postictal dysphasia once/month. Interictal scalp-EEG showed intermittent left temporal slowing without epileptiform abnormalities. Ictal EEG localized to the left temporal region. High-resolution MRI was normal. Subtraction ictal SPECT showed discrete area of hyperperfusion within the left lateral superior temporal gyrus (STG) and contiguous supratemporal plane (Fig. 1a). Interictal FDG-PET showed small but congruent area of decreased fluoro-deoxy-glucose uptake (Fig. 1b). To further delineate the epileptogenic zone and its relationship to eloquent cortex, we performed invasive evaluation with subdural grids covering left lateral temporal and perisylvian cortex, and depth electrodes targeting Heschl’s, planum temporale and mesial temporal structures (Fig. 1c). This unique patient was also evaluated with simultaneous intracranial-EEG and MEG sampled at 1,000 Hz. For standard analysis intracranial-EEG and MEG were band-passed with low-frequency cutoff of 5 Hz, and highfrequency cutoff of 70 and 50 Hz, respectively. No epileptic spikes were found interictally on either modality. We then provoked an ictal event in the MEG suite by having the patient listen to ‘‘Happy Birthday’’ followed by his favorite Elton John song. Two minutes later he reported typical aura of muffled sound. Discrete onset (Fig. 2, red arrow) was then seen in the LPST depth electrode contacts located within planum temporale (red contacts in Fig. 1c, left), represented by sharply contoured rhythmic theta evolving into repetitive spiking with overriding paroxysmal fast activity. Similar activities (Fig. 2, blue arrow) were observed on adjacent LST depth electrode in Heschl’s gyrus (blue contacts in Fig. 1c, left) starting 1.5 s later (first propagation site). These activities Z. I. Wang Y. Kakisaka R. C. Burgess J. A. Gonzalez-Martinez S. Ito J. C. Mosher S. Hantus A. V. Alexopoulos (&) Cleveland Clinic Epilepsy Center, 9500 Euclid Avenue, Desk S-51, Cleveland, OH 44195, USA e-mail: alexopa@ccf.org