G. Capovilla, G. Rubboli, F. Beccaria, M.E. Lorenzetti, A. Montagnini, C. Resi, E. Gardella, A. Gambardella, A. Romeo, C.A. Tassinari
of the face or neck. All patients underwent repeated and prolonged split-screen video-polygraphic EEG recordings. The polygraphic recordings and clinical correlations of the absence seizures were analysed. All patients presented with multi-quotidian, typical absence seizures. During the absences, the patients could show mild, rhythmic, myoclonic jerks involving facial areas (eyebrows, nostrils, perioral region, chin) or neck muscles (sternocleidomastoideus), with the same frequency as the spike-wave complexes. Polygraphic tracings demonstrated that the myoclonias were correlated to the spike component. Clinically, all patients showed a benign course, with complete seizure control under antiepileptic treatment. In the follow-up, 7 patients withdrew from treatment without relapse.
We conclude that all our patients showed an electroclinical picture consistent with CAE. The occurrence of myoclonic manifestations of the face or neck associated with the absences did not influence the benign course of their disease. The electroclinical features observed in our group of patients differentiates our cases both from epilepsy with myoclonic absences and from absences with perioral myoclonia (with Video).
C.P. Panayiotopoulos, C.D. Ferrie, M. Koutroumanidis, S. Rowlinson, S. Sanders
tonic clonic seizures and frequently absence status epilepticus. We report an 11 year-old normal girl with frequent episodes of absence status and no other overt clinical manifestations. However, appropriate video-EEG recordings documented that she had frequent absence seizures that were so mild as to escape recognition by her and the parents. These consisted of mild impairment of cognition and eyelid fluttering during brief generalised discharges of spike/multiple spike and slow waves. No further seizures occurred and the EEG normalised after appropriate drug treatment. Thus, it appears that this syndrome of phantom absences and absence status may start much earlier, in late childhood. Appropriate video-EEG documentation is needed for the recognition of these patients that may be more common than it appears from the few published cases (with Video).
K. Krakow, L. Lemieux, D. Messina, C.A. Scott, M.R. Symms, J.S. Duncan, D.R. Fish
B.V. Vaughn, E. Bernard, S. Lannon, B. Mann, O’N.F. D’Cruz, W. Shockley, A. Passanante
placement of the stimulator leads. We have developed two intraoperative techniques that improve correct identification of the vagus nerve. Both of these techniques utilize the co-localization of the recurrent laryngeal nerve with the vagus nerve. For patients undergoing stimulator placement with regional and local anesthesia, the stimulator current intensity is increased until alteration of voice can be confirmed with a voice test. Patients undergoing general anesthesia can also be tested by direct stimulation of the isolated vagus nerve. Utilizing visualization of the larynx and vocal cords via fiberoptic endoscopy, direct stimulation of the vagus nerve will produce a contraction of the left lateral wall of the larynx and tightening of the left vocal cord. Neither of these procedures produce any untoward effects for the patients. We have found these methods improve our ability to confirm correct placement of the stimulator with minimal increase in operative time (with Video).
J.S. Sieratzki, G.A. Calvert, M. Brammer, A. David, B. Woll
usually subsides at puberty, a severe communication impairment often persists. Here we report on a detailed study of a 26-year old, left-handed male, with onset of LKS at age 5 years, who is aphasic for English but who learned British Sign Language (BSL) at age 13. We have investigated his skills in different language modalities, recorded EEGs during wakefulness, sleep, and under conditions of auditory stimulation, measured brain stem auditory-evoked potentials (BAEP), and performed functional MRI (fMRI) during a range of linguistic tasks. Our investigation demonstrated severe restrictions in comprehension and production of spoken English as well as lip-reading, while reading was comparatively less impaired. BSL was by far the most efficient mode of communication. All EEG recordings were normal, while BAEP showed minor abnormalities. fMRI revealed: 1) powerful and extensive bilateral (R > L) activation of auditory cortices in response to heard speech, much stronger than when listening to music; 2) very little response to silent lip-reading; 3) strong activation in the temporo-parieto-occipital association cortex, exclusively in the right hemisphere (RH), when viewing BSL signs. Analysis of these findings provides novel insights into the disturbance of the auditory speech cortex which underlies LKS and its diagnostic evaluation by fMRI, and underpins a strategy of restoring communication abilities in LKS through a natural sign language of the deaf (with Video)
A. Biraben, M. Beaussart, E. Josien, M. Pestre, J.F. Savet, J.L. Schaff, D. Tourniaire, M. Sevestre, J. Renault-Djouadi