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Review article
Epilepsy in inborn errors of metabolism
Nicole I Wolf, Thomas Bast, Robert Surtees
treatment by diet or supplementation. However, in most, no such treatment is available and conventional antiepileptic drugs must be used, often with no great success. However, because uncontrolled epilepsy will hamper development and may even lead to further cerebral damage, treatment is necessary. Seizure types are rarely specific for a particular metabolic disorder, nor are EEG findings. Other symptoms and findings must be taken into account in order to achieve a diagnosis and, in some cases, specific management.We review the main characteristics of epilepsy due to inborn errors of energy metabolism, to disturbed neuronal function due to accumulation of storage products, to toxic effects and to disturbed neurotransmitter systems. We also discuss vitamin-responsive epilepsies and a number of other metabolic disorders focusing on possible pathogenetic mechanisms and their implication for diagnosis and treatment.
Original article
Temporal lobe epilepsy: clinical semiology and age at onset
Vicente Villanueva, José Maria Serratosa
medial or neocortical temporal lobe epilepsy who underwent epilepsy surgery between 1997-2002, and achieved an Engel class I or II outcome. We classified the patients into two groups according to the age at the first seizure: at or before 17 years of age and 18 years of age or older. All patients underwent intensive video-EEG monitoring. We reviewed at least three seizures from each patient and analyzed the following clinical data: presence of aura, duration of aura, ictal and post-ictal period, clinical semiology of aura, ictal and post-ictal period. We also analyzed the following data from the clinical history prior to surgery: presence of isolated auras, frequency of secondary generalized seizures, and frequency of complex partial seizures. Non-parametric, chi-square tests and odds ratios were used for the statistical analysis. There were 41 patients in the “early onset” group and 9 patients in the “later onset” group. A relationship was found between early onset and mesial temporal lobe epilepsy and between later onset and neocortical temporal lobe epilepsy (p = 0.04). The later onset group presented a higher incidence of blinking during seizures (p = 0.03), a longer duration of the post-ictal period (p = 0.07) and a lower number of presurgical complex partial seizures (p = 0.03). The other parameters analyzed showed no significant differences between the two groups. We conclude that clinical and semiological differences exist between patients with temporal lobe epilepsy according to the age at onset. [Published with video sequences]
Epilepsy in middle-aged and elderly people: a three-year observation
Boguslaw Paradowski, Mieszko M Zagrajek
common type of seizure in the group studied was partial (66.2%), followed by seizures with secondary generalization (33.8%). Epilepsy was caused by cerebrovascular disease (50.8%) considerably more often in patients over 74 years of age, craniocerebral trauma in patients addicted to alcohol (13.1%), especially those under 65 years of age, primary or metastatic neoplastic disease (10.7%), and others. The authors wish to draw attention to the leukoaraiosis factor, which might be the proepileptogenic cause of epilepsy recognized in the group of patients over 74 years of age (56.5%) and is much more frequent in this group than in the group of patients under 65 years of age (1.6%). Moreover, some drugs, such as L-dopa and Baclofen, might have been related to the epileptic seizures. In 29 patients (22.3%) the definite cause of late-onset epilepsy was unknown. The authors suggest in such cases, both follow – up tomographic examination and careful clinical examinations. In the study group of patients with initially unknown seizure etiology, some diseases, such as cerebral tumor or colon and pancreatic neoplasm, were diagnosed during follow-up examination. These processes were revealed several months after the first epileptic seizure.
Ipsilateral head deviation related to orbito-frontal and fronto-polar seizures
S Rheims, Geneviève Demarquay, Marc Guénot, Marc Sindou, François Mauguière, Philippe Ryvlin
of ipsilateral head turning in frontal lobe seizures are available. We report a patient with frontal lobe seizures associated with reproducible, early, ipsilateral head deviation, where imaging and video-stereo-electroencephalography data, as well as surgical outcome, demonstrated the fronto-polar and orbito-frontal origin of the epileptic discharge. We conclude that early ipsilateral head deviation, in the context of frontal lobe epilepsy, raises the possibility of fronto-polar or orbito-frontal seizure onset.[Published with video sequences]
Psychometric validation of the French version of the side-effects and life satisfaction inventory (SEALS) in epileptic patients
Abdelkader El Hasnaoui, Arielle Crespel, Jean-Pierre Daurès, Michel Baldy-Moulinier, Marie-Christine Picot
(QOLIE-31) and a generic, health-related quality-of-life questionnaire, the Nottingham health profile (NHP). The psychometric properties of SEALS, assessed in 190 adult subjects with epilepsy, included: acceptability, test-retest reliability and validity, multitrait analysis including internal consistency and item-to-scale correlations, construct validity using factor analysis and discriminative validity using associations with disease characteristics and treatment effects, and, correlations with NHP and QOLIE-31 scores for convergent and divergent validity. Both acceptability and reproducibility were good and internal consistency was high (Cronbach’s α coefficient = 0.92). Factor analysis with varimax rotation identified five factors: the first, related to cognitive function accounted for 26.0% of the variance. Discriminative validity was good for most treatment characteristics (tolerability, seizure control, compliance) and clinical features (epilepsy type, seizure frequency and severity, depressive symptoms). Correlations with the NHP and QOLIE-31 scores were consistently strong. It was concluded that the psychometric properties of the French translation of SEALS were similar to the original English version. In addition, SEALS provides information on quality of life that is complementary to that obtained with QOLIE-31. In particular, with respect to the QOLIE-31, the SEALS provides information on cognitive and neuropsychological aspects of impairment of quality of life, whereas the QOLIE-31 has a broader scope, taking into account multiple aspects of quality of life in epilepsy.
Language- and praxis-induced jerks in patients with juvenile myoclonic epilepsy
Patrícia da Silva Sousa, Katia Lin, Eliana Garzon, Américo Ceiki Sakamoto, Elza Márcia T Yacubian
language-induced jerks was documented in video-polygraphic EEG recordings.[Published with video sequences]
Special article
Making sense of non-epileptic seizures
Nuri Gene-Cos, Howard A Ring
Methods. Medline and PsycLit review of clinical and laboratory studies. Results. The concept of NES is clarified, common clinical presentations are reviewed and the differential diagnoses considered. A general overview of possible mechanisms and pathological findings is presented. Finally, epidemiological, prognostic, and treatment issues are described. Conclusions. Clinicians increased awareness of NES, together with new diagnostic techniques will improve diagnosis and outcome of this condition. Further research is needed into the pathophysiology, etiology and maintenance mechanisms of non-epileptic seizures.
Video case report
Motor impairment on awakening in a patient with an EEG pattern of “unilateral, continuous spikes and waves during slow sleep”
Pierangelo Veggiotti, Simonetta Cardinali, Elisa Granocchio, Paolo Avantaggiato, G Papalia, Antonio Cagnana, Giovanni Lanzi
an epileptic encephalopathy who, from the age of seven years and four months, has twice presented a movement disorder affecting the right arm, manifesting on awakening and disappearing by early afternoon. Sleep EEG during these periods showed continuous, high-amplitude, diphasic spikes and slow waves over the left hemisphere. Association of clobazam, valproic acid and, on the second occasion, ethosuccimide led to disappearance of the above-described EEG picture and associated motor symptoms. Neurophysiological investigations excluded other possible aetiologies. In view of this, and of the close relationship between the EEG picture and clinical course, we interpret the patient’s impairment as “motor neglect” secondary to the continuous electrical activity recorded during sleep over the left hemisphere and involving the associative areas. This electrical activity in sleep, may be regarded as a “functional lesion” whose clinical consequences can be correlated with the site of the abnormalities.[Published with video sequences]
Gelastic seizures due to right temporal cortical dysplasia
Nese Dericioglu, Oguz Cataltepe, Gaye Guler Tezel, Serap Saygi
rare. Depending on the location of the lesion, gelastic seizures may or may not be accompanied by a subjective feeling of mirth. The pathophysiological mechanisms of this type of seizure are still undefined, and little is known about which pathways promote laughter and its emotional content, mirth. We present a young man with drug-resistant, gelastic seizures due to focal cortical dysplasia of the right inferior temporal gyrus. The lesion was evident on cranial MRI. Interictal EEG displayed a right temporal focus, whereas ictal EEG was not informative. Ictal loss of consciousness precluded reporting of any possible emotional experience. The patient underwent surgical resection of the lesion and has been seizure-free with anti-epileptic medication for two years. Although various anatomical regions may elicit laughter, in view of the current literature it seems that the anterior cingulate region is involved in the motor aspects of laughter, while the basal temporal cortex is involved in the processing of mirth. The fact that the present case exhibited gelastic seizures stresses once more the importance of the baso-lateral temporal cortex in the genesis of this type of seizures.[Published with video sequences]
Post-ictal rage and aggression: a video-EEG study
Alexei E Yankovsky, Martin Veilleux, François Dubeau, Frederick Andermann
rage and violence. This mentally retarded man had a lifelong history of seizures. He developed increasing episodic rage and aggression. Caregivers were afraid of him, although there was no record of directed violence. In one of these episodes he fractured his tibia and fibula. Interictal discharges arose from both temporal areas independently. He had focal seizures with secondary generalization. Immediately after cessation of the ictal discharge he became greatly agitated, with undirected aggression, loud screaming, kicking and fighting against the restraints. A video sequence illustrates the behavior. Imaging studies showed bilateral, periventricular, nodular heterotopia in the lateral aspect of both temporal horns and the trigones. Increasingly frequent, severe bursts of rage and aggression were proven to be post-ictal. Documented attacks occurred while he was restrained and this may have been a factor in their severity. Such attacks however, have been described while he was not restrained and these increased in severity and frequency over time. Developmental abnormalities with periventricular, nodular heterotopia in the region of the trigones and inferomesial temporal areas are considered to be causally related to his retardation and epilepsy. [Published with video sequences]
Correspondence
A semiological classification of status epilepticus
H.O. Lüders, S. Rona, F. Rosenow, S. Arnold, M. Carreño, B. Diehl, A. Ebner, B. Fritsch, H. Hamer, H. Holthausen, S. Knake, S. Noachtar, S. Pieper, I. Tuxhorn
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