Philippe Gelisse, Pierre Thomas, Raymond Padovani, Nathalie Hassan‐Sebbag, Jacques Pasquier, Pierre Genton
she particularly liked,
<i>Con Te Partiro</i>, by Andrea Boccelli. Other triggering factors were various types of music such as supermarket background music and polyphonic singing or instrumental music played by family members. The seizures had a stereotyped course: she felt anxious, tearful, then occurred slight obtundation, during which she smacked her lips and moved restlessly. There was no complete loss of consciousness, but some degree of amnesia. She never experienced a generalized tonic‐clonic seizure, but reported rare spontaneous feelings of
<i>déjà‐vu</i> that had begun at the same time as the induced seizures. There were no other spontaneous attacks; only one seizure was apparently provoked, not by music but by a loud background noise in her office. She was a music lover and a singer. Interictal EEG showed independent slow waves over the temporal regions. Several seizures with EEG localisation over the right temporal region were elicited after several minutes of exposure to music. Monoauricular stimulation with the same music produced a seizure when applied to the left ear but was ineffective when applied to the right ear. Ictal SPECT demonstrated right temporal hyperperfusion. MRI was normal. On high dose of carbamazepine, seizure frequency decreased. The addition of topiramate resulted in full seizure control. Musicogenic epilepsy is a rare form of reflex epilepsy. Pure cases, when patients do not experience unprovoked seizures, are exceptional. Our report confirms the implication of the right temporal lobe in this epilepsy. [Published with video sequence].
Athanasios Covanis, Christina Lada, Konstantinos Skiadas
reported in Rolandic epilepsy. Out of a population of 1340 children with focal afebrile seizures we studied 24 children who had emetic manifestations in at least one seizure and centrotemporal spikes in at least one EEG. They were of normal neurological status and had a follow‐up of at least two years after the last seizure. All children had sleep EEG following sleep deprivation. Two groups of patients were identified. Group A (12 patients) with EEG centrotemporal spikes only and group B (12 patients) with centrotemporal spikes and spikes in other locations. In 21 patients, ictal emetic manifestations culminated in vomiting and in three only nausea or retching occurred. The commonest presentation was ictus emeticus at onset followed by deviation of the eyes or staring, loss of contact and floppiness. In 79%, seizures occurred during sleep. Autonomic status epilepticus occurred in 37.5%. The mean age at onset was 5.3 years. Overall analysis of the clinical and EEG data points out that the vast majority of these patients primarily suffer from Panayiotopoulos syndrome. Twenty patients (83%) had ictal semiology typical of Panayiotopoulos syndrome, but five also had concurrent Rolandic symptoms and four later developed pure Rolandic seizures. The other four patients (17%) had typical Rolandic seizures with concurrent ictus emeticus. These findings suggest a link between Rolandic epilepsy and Panayiotopoulos syndrome, the two most important phenotypes of the benign childhood seizure susceptibility syndrome.
Mark E. Landau, Kevin R. Cannard
seizures. A paucity of literature is available describing the electroencephalographic (EEG) findings. We describe the clinical and EEG characteristics of a patient with genetically confirmed J‐HD. A review of previously published cases yielded EEG descriptions in only 23 patients whose disease onset was prior to the age of 32, and only 14 of these were prior to the age of 20. Epileptiform abnormalities were noted in 17 (74%), which was considerably more common than in the adult form. Generalized discharges were noted in nine, with six having polyspike and wave. The remainder had focal or multifocal epileptiform discharges. With genetic testing now available, refinement of the EEG data will be possible.
Leanne Casaubon, Bernd Pohlmann‐Eden, Houman Khosravani, Peter L. Carlen, Richard Wennberg
of long debate. Most experimental data implicate a hyperexcitable cortical generator of spike‐and‐wave activity, with the thalamus quickly recruited to sustain the generalized oscillations through a reverberating thalamocortical network. However, there is little clinical evidence to support the cortical generator hypothesis. We present video‐EEG recordings of generalized tonic‐clonic seizures in three patients with proven primary generalized epilepsy (PGE), all of whom showed a consistent pattern of lateralized seizure onset compatible with a focal frontal lobe generator.
<i>Methods :</i> Among 300 patients referred for video‐EEG monitoring for intractable epilepsy, three were found to have PGE with tonic‐clonic convulsions. All had a positive family history for epilepsy and no other epilepsy risk factors. Epilepsy onset was during adolescence (2\3) or childhood (1\3). Patients were taking 1‐4 antiepileptic drugs (AEDs) at admission, none of which was valproic acid.
<i>Results :</i> Interictal EEG showed very active, bilaterally synchronous generalized spike‐and‐wave or polyspike‐and‐wave discharges between 2.5‐4.5 Hz, maximal over the midfrontal structures symetrically in all patients. Ictal EEG showed generalized rhythmic activity without lateralization at seizure onset. Surprisingly, in all 6 recorded tonic‐clonic seizures there was a sustained (10‐15 seconds), stereotyped, clinical lateralization at onset, which took the form of a tonic "fencing posture" in one patient (two seizures) and forced head\eye\torso version in two patients (four seizures). Two patients became seizure‐free shortly after switching to valproate monotherapy. One patient refused valproate but has improved more than 90 % with a change in AEDs to lamotrigine and phenobarbital (follow‐up in all patients > 18 months).
<i>Conclusions :</i> Tonic‐clonic seizures are presumed to be generalized from onset in patients with PGE. However, video‐EEG monitoring in these patients is rarely performed and the actual clinical features of the seizures may be underappreciated. The demonstration of sustained lateralization at onset in our patients, with features clinically indistinguishable from focal onset frontal lobe seizures, is compatible with the hypothesis of a focal region of cortical hyperexcitability situated in the frontal lobes of some patients with PGE. Whether this cortical generator is autonomous or "triggered" by ascending, possibly normal, thalamocortical volleys is unresolved. [Published with video sequences].