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Epileptic Disorders

Volume 3
Number 3,
September 2001

Synthèse

Autonomic symptoms during epileptic seizures

Christoph Baumgartner, Stefanie Lurger, Fritz Leutmezer

simple reactions to motor manifestations of seizures, but are mediated by an activation of the central autonomic network. Autonomic symptoms can be divided into cardiovascular changes, respiratory manifestations, gastrointestinal symptoms, cutaneous manifestations, pupillary symptoms, genital and sexual manifestations as well as urinary symptoms. Due to a hemispheric-specific representation of the central autonomic network, certain autonomic symptoms may provide lateralizing and sometimes localizing information on the seizure onset zone, although some of these signs may appear as a result of discharge spreading. Autonomic symptoms indicating a seizure onset in the non-dominant hemisphere include ictal vomiting and retching, spitting automatisms and ictal urinary urge. Autonomic symptoms range from subtle seizure manifestations which become apparent only during meticulous seizure analysis, to severe, sometimes life-threatening events. Cardiovascular and respiratory autonomic symptoms are discussed as the mechanisms underlying sudden unexplained death in epilepsy. When autonomic symptoms represent the sole seizure manifestation, they can pose problems for differential diagnosis of various non-epileptic conditions. Finally, autonomic seizure symptoms open a unique window on the functional organization of the central autonomic network and on brain function in general. (Published with videosequences.)

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Articles originaux

Functional MRI assessment of the hemispheric predominance for language in epileptic patients using a simple rhyme detection task

Monica Baciu, Philippe Kahane, Lorella Minotti, Annik Charnallet, Danielle David, Jean-François Le Bas, Christoph Segebarth

The findings derived from the fMRI examinations were compared with those obtained on the same patients using the Wada test, stereotactic intracerebral EEG stimulations and recordings, and/or video-EEG recordings. For the seventeen patients for whom language dominance could be assessed by means of at least one of the latter procedures, the fMRI examination provided concordant results in sixteen. In two patients, the hemispheric predominance for language could only be determined by fMRI. Nine patients underwent surgery subsequent to the fMRI examination. None of them exhibited any aphasic problems following surgery. The rhyme detection task used in the fMRI examination generates robust responses in the language areas, permits easy monitoring of the patient's task performance and can be easily undertaken by the epileptic patients. Thus, this study demonstrates that the fMRI rhyme detection paradigm is particularly well-suited for determining hemispheric language predominance in epileptic candidates for surgery.

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Neonatal seizures: background EEG activity and the electroclinical correlation in full-term neonates with hypoxic-ischemic encephalopathy. Analysis by computer-synchronized long-term polygraphic video-EEG monitoring

Luisa Comenale Pinto, Paolo Giliberti

first 48 hours of life, in our neonatal intensive care unit. In this longitudinal study, recordings lasted an average of 10.5 hours. We studied EEG ictal discharges that lacked a clinical correlate, the semeiotics, and the electroclinical correlation during seizures, by reviewing the time-synchronised, video-EEG recordings obtained during these six years. The aim of this investigation was to evaluate the relationship between background EEG activity and the electroclinical correlation or dissociation of the seizures. When background activity was depressed and undifferentiated, the seizures were characterised by the electroclinical dissociation except in instances of focal tonic seizures. With all other background patterns, seizures were invariably accompanied by the electrical correlate, the only exception being ocular signs and oral-buccal-lingual movements, which occurred both with and without paroxysmal discharges. Ocular signs and oral-buccal-lingual movements were very frequent and were observed with all patterns of background activity. Only on one occasion were they the sole clinical expression of seizures. In our patients, the electroclinical dissociation was constant only if background EEG activity was depressed and undifferentiated. The constant electroclinical dissociation of seizures was closely correlated with the type of background activity, which, in turn, is indicative of severe cerebral damage. (Published with videosequences.)

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Tonic reflex seizures of early infancy: an age-related non-epileptic paroxysmal disorder

Federico Vigevano, Maria Luisa Lispi

of life and are expressed predominantly as unusual movements. We report the clinical and EEG-polygraphic characteristics of 13 patients (nine male, four female) that we observed between 1988 and 1999, who presented non-epileptic tonic reflex seizures in the first months of life. All children had normal psychomotor development with no neurological antecedents. Onset of manifestations occurred between the first and third month of life. The seizure was characterized by diffuse tonic contraction with extension of four limbs, apnoea and cyanosis, and lasted 3-10 sec without loss of consciousness. Seizures occurred only while the child was awake and being held in a vertical position by an adult, and were triggered by movement or tactile stimulation. Ictal and interictal EEG were normal. Prognosis was benign with spontaneous remission within 2 months of onset. In our experience, this paroxysmal manifestation has not been described previously by other authors and represents a new NEPD which, in accordance with our first report of this entity, we suggest calling tonic reflex seizure of early infancy (TRSEI). (Published with videosequences.)

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Is the left cerebral hemisphere more prone to epileptogenesis than the right?

Mark D. Holmes, Carl B. Dodrill, Roman L. Kutsy, George A. Ojemann, John W. Miller

epilepsy, as documented by long-term EEG-video monitor studies. We identified those with interictal epileptiform patterns on EEG confined to one hemisphere, those with clinical seizures that arose only from one side, and individuals with neuropsychological deficits lateralizing more to one cerebral hemisphere than the other. These data were then related to the handedness of the subjects. Results. For left-handed patients, interictal discharges were significantly more likely to be confined to the left side, seizures more frequently arose from only the left hemisphere, and neuropsychological deficits lateralized more often to the left brain. In contrast, for right-handed subjects, there were no significant differences in unilateral localization of either interictal discharges or of seizure onsets, nor did neuropsychological deficits lateralize more often to one side or the other. Although, as a group, left-handers had an earlier age of onset of epilepsy than right-handers, the lateralizing EEG and neuropsychological patterns seen in left-handers were not related to age of onset of epilepsy, febrile seizures, family history of either epilepsy or left-handedness, or other risk factors. Conclusion. In localization-related epilepsy, there is a significant association of left-handedness with left hemispheric lateralization of epileptiform EEG patterns and neuropsychological deficits. These findings do not clearly reflect a greater left than right cerebral hemispheric vulnerability to epileptogensis, except possibly in left-handers.

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Symptomatic epilepsy with facial myoclonus triggered by language

Maria Paola Canevini, Aglaia Vignoli, Vincenzo Sgro, Elena Zambrelli, Ada Piazzini, Nadia Colombo, Raffaele Canger

language. At age 57, the patient first noted that he would begin to stutter when delivering lectures at conferences. The stuttering would worsen if he continued talking. The video-polygraphic EEG recording shows brief paroxysms of spikes and polyspikes, followed by a slow wave, more evident in the left fronto-temporal region. The myoclonic jerks originating from the submental area correlate with EEG abnormalities. Clinically, these jerks determined a form of stuttering. The triggering factors were reading, speaking and listening to spoken language. This case had several characteristic features: facial myoclonus was the only seizure type experienced by the patient; the seizures and language impairment had a very late onset ­ about 50 years after the traumatic event that produced a dramatic lesion in the left fronto-polar region. (Published with videosequences.)

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Drug-induced nonconvulsive status epilepticus with low dose of tiagabine

Sabine Fitzek, Stefan Hegemann, Dieter Sauner, Dominikus Bönsch, Clemens Fitzek

status epilepticus under tiagabine suggested a specific role of dose levels, since in these patients symptoms occurred mostly at 40 mg/day or higher. We report a case of complex partial status epilepticus in a patient receiving a low dose of tiagabine and review all 11 case reports of nonconvulsive status epilepticus in patients on tiagabine, with regard to daily doses. Our analysis suggests an individual risk threshold of unknown aetiology.

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Efficacy of lamotrigine add-on therapy in severe partial epilepsy in adults with drop seizures and secondary bilateral synchrony on EEG

Francesca Bisulli, Agostino Baruzzi, Anna Rosati, Roberto Riva, Patrizia Avoni, Angelina Cerullo, Paolo Tinuper

on EEG. Methods: We carried out a single-center, open-label, prospective study on a restricted group of patients experiencing an EDA frequency of at least one/month during the previous year regardless of multiple antiepileptic drug (AED) trials. Study design consisted of three phases: a 3-month baseline period, a 4-month period in which LTG was titrated and a 9-month maintenance dose observational period. LTG add-on therapy depended on valproate (VPA) association, with a maximum of 200 mg/day with VPA and 600 mg/day in the absence of VPA. Every three months, patients underwent clinical, hematological and EEG evaluation including plasma level of AEDs. To assess the efficacy of LTG add-on therapy, patients were required to keep a detailed seizure diary throughout the study. Results: Fourteen patients (nine men and five women), aged from 21 to 51, were included in the study. All of them had complex partial seizures (CPS), besides EDA, and half of them had secondarily generalized seizures (SGS). Two of the 14 patients had to stop LTG due to side effects, although one of them was seizure-free after LTG. Twelve patients completed the study. The improvement was more than 50% for every type of seizure. SGS disappeared in three cases and improved by more than 50% in another three cases. EDA disappeared in six patients; and improved with more than 50% EDA reduction in five patients. CPS disappeared in two patients and improved by more than 50% in eight. EEG improved in nine cases, with SBS disappearing in six patients. Conclusions: We have demonstrated a good efficacy of LTG adjunctive therapy on EDA. Results include control of SGS and improvement of EEG tracing.

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Atypical evolution in childhood epilepsy with occipital paroxysms (Panayiotopoulos type)

Roberto Horacio Caraballo, Francisco Astorino, Ricardo Cersósimo, Ana M. Soprano, Natalio Fejerman

that showed atypical evolution. Neurological examination and brain imaging were normal in both. One child presented at age 2.5 years episodes of oculocephalic deviation, and ictal vomiting during nocturnal sleep. The EEG showed left occipital spikes during wakefulness and sleep. One year later, frequent inhibitory seizures appeared in the lower limbs causing, "pseudoataxic gait". At the same time she presented with behavioral disturbances and aphasia. EEG showed bilateral spike-waves while awake and continuous spike-waves during slow sleep (CSWSS). After switching AEDs to benzodiazepines, control of seizures along with improvement of behavior, and partial restoration of cognitive functions were achieved. The CSWSS disappeared and the last EEG at age 8 years only showed only isolated right occipital spikes. The other girl had a personal and familial history of febrile seizures. At 4 years of age she presented the first non-febrile seizures during sleep, with oculocephalic deviation and ictal vomiting, followed by a generalized tonic-clonic seizure. Partial control of seizures was obtained with antiepileptic drugs. At age 7, the child began to have weekly episodes of oculocephalic version, occasionally with secondary generalization. Repeated inhibitory seizures and absences also appeared. EEG showed frequent bilateral spikes occupying predominantly the posterior regions while awake, and CSWSS. At 7.5 years the same electro-clinical picture persisted. Ethosuximide was added to sodim valproate and clobazam. Fifteen days later, the seizures disappeared and the EEG showed less frequent bilateral occipital spikes. She is now 9 years old and she has been seizure-free for 18 months. Her present neuropsychological profile shows mild mental retardation. The two children with typical electroclinical features of "Panayiotopoulos Type" CEOP developed an atypical evolution which, to our knowledge, has not been described previously.

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Lettre de l'éditeur

Pathophysiological accounts of language impairments in Landau-Kleffner syndrome

Marie-Noëlle Metz-Lutz, Anne de Saint-Martin

aphasia is caused by a hyperexcitability of the speech cortices in response to phonological processing activity rather than by ablation or disconnection. This suggestion based on fMRI studies of language carried out on a patient with normal EEG for 12 years is questionable in certain respects.

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