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Editorial
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J. Aicardi
one of the pioneers of epilepsy surgery in Europe and his contribution to presurgical investigations was especially prominent. His emphasis on clinical analysis of seizure symptoms remains an essential part of his teaching and an inspiration for Epileptic Disorders
Synthèse
Visual phenomena and headache in occipital epilepsy: a review, a systematic study and differentiation from migraine
C.P. Panayiotopoulos
analysis showed that visual seizures usually lasted for seconds to 1-3 minutes. Three patients also had longer visual seizures of 20-150 minutes. Elementary visual hallucinations mainly consisted of coloured and small circular patterns flashing or multiplying in a temporal hemifield. Flashing lights or non-circular patterns were rare. Three patients experienced achromatic flickering lights. None of the patients had the over 4 minute, linear, zigzag, and achromatic or black and white patterns characteristic of migraine visual aura. Blurring of vision could precede visual hallucinations. Visual seizures were usually frequent, often occurring in multiple clusters daily or weekly. They usually occurred alone but they often advanced to other occipital and extra-occipital ictal symptoms. In 7 patients they progressed to temporal lobe seizure manifestations, and in 6 to motor partial seizures or ipsilateral hemiconvulsions. All but 2 had secondary generalised tonic clonic convulsions. Ictal blindness ab initio occurred in 2 and ictal, mainly orbital headache in another 2 patients. One patient had ictal vomiting as an occasional symptom. Postictal headache, often severe and indistinguishable from migraine, occurred in two thirds of the patients, even after brief visual seizures without convulsions. Despite relevant structural lesions in brain imaging, 10 patients had a normal mental and neurological state. In 8 patients, EEG was also normal or non-specific. Misdiagnosis of visual seizures as visual aura of migraine was common and 3 patients were misdiagnosed as suffering from migraine. The differential diagnosis between migraine and the occipital epilepsies is reviewed. It is concluded that elementary visual hallucinations, blindness or both, alone or followed by headache and vomiting of symptomatic occipital epilepsy are identical to those of idiopathic occipital epilepsy. Progress to temporal lobe structures is different and consistent with symptomatic occipital lobe epilepsy. The clinical diagnosis of visual seizures is easy if individual elements of duration, colour, shape, size, location, movement, speed of development and progress are identified. They are markedly different from visual aura of migraine, although they often trigger migrainous headache, probably by activating trigeminovascular or brain stem mechanisms.
Conférence mondiale, Martinique 27 Novembre-4 Décembre 1999
Evolution of epilepsy surgery in childhood: the neurologist’s point of view
J. Aicardi
the frequency of severe, extensive brain damage, and technical problems. Surgery for epilepsy in infants and children raises problems quite different from those in adults. This applies especially to severe infantile epilepsies for which the aim of treatment may be more to facilitate neurodevelopment than to control the seizures. In addition, intractability cannot be defined in the same terms at different ages, the time scale being usually much shorter in young children and the operations required often extensive. The results of surgery in childhood are comparable to those in adults. However, indications for surgery and assessment of its results in catastrophic epilepsies of infants requires different rules to those that apply to adults and older children.
When is imaging enough?
J. Engel Jr
history, careful description of the ictal events, and the neurological examination will always be necessary. Interictal EEG and neuropsychological testing also add important information. In many situations, however, video-EEG monitoring of habitual seizures now plays a confirmatory, rather than a primary, role in the presurgical evaluation, and can occasionally contribute confounding data that delay or prevent surgical intervention. In the present climate of cost-containment, it is reasonable to ask if this expensive diagnostic tool is always needed when it is clear by other means that seizures are not psychogenic, particularly in certain pediatric surgically remediably syndromes such as those catastrophic epilepsys of infants and young children amenable to hemispherectomy, and drop attack that can be treated with corpus callosotomy, as well as mesial temporal lobe epilepsy and seizures due to discrete resectable lesions that often become medically intractable in adolescence. Retrospective studies should be carried out to determine when long-term, in-patient, video-EEG monitoring in these conditions add information that appropriately changes the therapeutic approach.
Research perspectives in cortical dysplasia and associated epilepsies
C. Munari, L. Tassi, G. Lo Russo, S. Francione, R. Mai, F. Cardinale, R. Spreafico
with advances in diagnostic tools, therapeutic approaches and surgical techniques. In the present paper, we briefly review the most important advances in these fields from the point of view of the clinician concerned with cortical malformation-related epilepsies. We propose that a highly effective way forward, expected not only to widen knowledge of the basic mechanisms of seizure generation, but also to improved the management of patients, would be to promote interdisciplinary research programmes on resected human cortex that involve neurosurgeons, neurologists and laboratory neuroscientists.
Catastrophic epilepsy in infants and children: identification of surgical candidates
E. Wyllie
stagnation or regression. Increasing numbers of infants with catastrophic epilepsy are being considered for surgical treatment, if they have the key elements of surgical candidacy including medically intractable epilepsy, a localized epileptogenic zone, and a low risk of new, unacceptable, postoperative neurologic deficits. Most of these infants have had low grade tumor, cortical development malformations, or other epileptogenic lesions visualized on preoperative MRI. The assessment of potential risks and benefits for surgery in these patients involves complex, age-related issues including the possible impact of uncontrolled seizures or surgery, on cognitive development. This review will address some of these issues in the context of two clinical cases.
Articles originaux
Can protrusion of the tongue stop seizures in Rolandic Epilepsy?
P. Veggiotti, F. Beccaria, A. Gatti, G. Papalia, C. Resi, G. Lanzi
polygraphic EEG recording of a seizure which terminated upon voluntary protrusion of the tongue. We mention the close link between the post-central (somatosensory) cortex and pre-central (motor) cortex, and how the primary sensory area has direct access to the motor cortex. We also examine how a tactile stimulus may provoke the inhibition of an epileptic discharge.
Gelastic seizures: video-EEG and scintigraphic analysis of a case with a frontal focus; review of the literature and pathophysiological hypotheses
A. Biraben, E. Sartori, D. Taussig, A.M. Bernard, J.M. Scarabin
cryptogenic epilepsy. Few cases of gelastic seizures originating in the frontal lobe have been reported in the literature, most of them involving a diencephalic hamartoma or a temporal focus although, no clinical pattern has been found to be specific for each of these three anatomical regions. The ictal laughter is of variable nature, unmotivated or associated with feelings of mirth, forced or natural, except in the case of a frontal focus where the laughter seems consistently described as forced and unmotivated. However, mirth and laughter are two dissociable clinical elements; their genesis probably involves distinct mechanisms. Anatomical considerations lead to several hypotheses concerning laughter generation: it could be a simple reactional behavior in response to a modified cognitive process, an automatic behavior or a forced action. In a few cases with a temporal focus, laughter seems directly related to a disorganization of the associative temporal cortex and may be considered as a reactional behavior. In cases with frontal focus, anterior cingulate and orbital structures would be particularly implicated in laugher genesis, although with possible different pathophysiological routes: in the first case by disconnection within the premotor mesial system or by an imbalance between premotor mesial and premotor lateral systems, and in the second case by activation of a previously conditioned orbital region.
Exceptionally long absence status: multifactorial etiology, drug interactions and complications
M. D. D’Agostino, F. Andermann, F. Dubeau, M. Fedi, A. Bastos
who developed petit mal status lasting 6 weeks. Valproate levels remained low because of interactions with phenytoin. When phenytoin was discontinued, valproate levels increased, and he progressively improved. Chronic ischemic changes in the white matter may have been an additional factor in the causation and, most likely, in the duration of his status. Exceptionally long status and stupor increase the risk of medical complications. Valproate remains the medication of choice for the treatment of petit mal status. Despite the extraordinary course in this patient, complete recovery took place, confirming the benign nature of even such a prolonged episode.
Rotatory seizures in a patient with tuberous sclerosis
G. P. Ramelli, F. Donati, M. Kollar, L. Remonda, F. Vassella
pathology. Our patient had a right temporal epileptic focus, confirmed by magnetic resonance imaging (MRI) to be a subcortical lesion in the right temporal region. This case is probably the first reported case of tuberous sclerosis associated with rotatory seizures with an ictal EEG.
Non-convulsive status in the ring chromosome 20 syndrome: a video illustration of 3 cases
J. Petit, A. Roubertie, Y. Inoue, P. Genton
emotional events.
This recognizable clinical entity associates a slight mental deficiency, behavioural disorder and epilepsy, often resistant to treatment and with a particular clinical presentation. We demonstrate with video EEG recording, atypical absence status triggered by emotional behaviour with ictal EEG characterized by bifrontal slow waves, often rhythmic, or sharp waves.
Our report highlights the characteristic features of non-convulsive status in this electro-clinical syndrome.
