A new type of epilepsy : benign partial epilepsy of childhood with occipital spike-waves
TL;DR: A benign epilepsy with occipital spike-waves (SEOSW) which could be as clearly defined (if not better) as the benign epilepsy of childhood with rolandic spikes (SERS) is identified and some theoretical remarks about etiopathology and physiopathogeny are made.
Abstract: In the field of benign partial epilepsies of childhood with a mainly if not exclusively functional focus, we 5 have recently identified a benign epilepsy with occipital spike-waves (SEOSW) which could be as clearly defined (if not better) as the benign epilepsy of childhood with rolandic spikes (SERS). That this epilepsy has until now escaped the attention of epileptologists is all the more curious when one considers that the existence of benign epilepsy with occipital focus had been forecasted 30 years ago by F. and E. Gibbs.\" however without the description of the corresponding clinical and EEG symptoms. Such a lack of recognition is undoubtedly related to the relative rarity of SEOSW (about half as frequent as SERS) and to the subjective nature of the characteristic visual symptoms, sometimes being neglected or poorly related by the younger child. The present study, based on 36 cases of such an epilepsy recently identified in our Department of Clinical Neurophysiology in Marseilles, concerns successively: 1) the status of the patients between seizures; 2) the features of the seizures; 3) the course of the disease and 4) some theoretical remarks about etiopathology and physiopathogeny. Rather than giving a glimpse of all 36 cases in a skeleton-like table, only four typical cases are annexed to illustrate the main semiological andlor evolutive features.
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TL;DR: Converging evidence from multiple and independent clinical, EEG and magnetoencephalographic studies has documented Panayiotopoulos syndrome (PS) as a model of childhood autonomic epilepsy, which is also common and benign.
Abstract: A big advance in epileptology has been the recognition of syndromes with distinct aetiology, clinical and EEG features, treatment and prognosis. A prime and common example of this is rolandic epilepsy that is well known by the general paediatricians for over 50 years, thus allowing a precise diagnosis that predicts an excellent prognosis. However, rolandic is not the only benign childhood epileptic syndrome. Converging evidence from multiple and independent clinical, EEG and magnetoencephalographic studies has documented Panayiotopoulos syndrome (PS) as a model of childhood autonomic epilepsy, which is also common and benign. Despite high prevalence, lengthy and dramatic features, PS as well as autonomic status epilepticus had eluded recognition because emetic and other ictal autonomic manifestations were dismissed as non-epileptic events of other diseases. Furthermore, PS because of frequent EEG occipital spikes has been erroneously considered as occipital epilepsy and thus confused with the idiopathic childhood occipital epilepsy of Gastaut (ICOE-G), which is another age-related but rarer and of unpredictable prognosis syndrome. Encephalitis is a common misdiagnosis for PS and migraine with visual aura for ICOE-G. Pathophysiologically, the symptomatogenic zone appears to correspond to the epileptogenic zone in rolandic epilepsy (sensory-motor symptomatology of the rolandic cortex) and the ICOE-G (occipital lobe symptomatology), while the autonomic clinical manifestations of PS are likely to be generated by variable and widely spread epileptogenic foci acting upon a temporarily hyperexcitable central autonomic network. Rolandic epilepsy, PS, ICOE-G and other possible clinical phenotypes of benign childhood focal seizures are likely to be linked together by a genetically determined, functional derangement of the systemic brain maturation that is age related (benign childhood seizure susceptibility syndrome). This is usually mild but exceptionally it may diverge to serious epileptic disorders such as epileptic encephalopathy with continuous spike and wave during sleep. Links with other benign and age-related seizures in early life such as febrile seizures, benign focal neonatal and infantile seizures is possible. Overlap with idiopathic generalized epilepsies is limited and of uncertain genetic significance. Taking all these into account, benign childhood focal seizures and related epileptic syndromes would need proper multi-disciplinary re-assessment in an evidence-based manner.
330 citations
Cites background from "A new type of epilepsy : benign par..."
...…epilepsy of Gastaut The ICOE-G is a relatively rare form of pure occipital epilepsy accounting for about 2–7% of benign childhood focal seizures (Gastaut, 1981, 1982a, b; Panayiotopoulos, 1981, 1999a, b; Beaumanoir, 1983; Gastaut and Zifkin, 1987; Gastaut et al., 1992; Ferrie et al., 1997;…...
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TL;DR: The results indicate that migraine and epilepsy are strongly associated, independent of seizure type, etiology, age at onset, or family history of epilepsy.
Abstract: We investigated comorbidity of migraine and epilepsy by using information from structured telephone interviews with 1,948 adult probands with epilepsy and 1,411 of their parents and siblings. Epilepsy was defined as a lifetime history of two or more unprovoked seizures, and migraine as severe headaches with two or more of the following symptoms: unilateral pain, throbbing pain, visual aura, or nausea. Cumulative incidence of migraine to age 40 was 24% in probands with epilepsy, 23% in relatives with epilepsy, and 12% in relatives without epilepsy. Using Cox proportional hazards analysis to control for years at risk and gender, the rate ratio for migraine was 2.4 (95% CI, 2.02 to 2.89) among probands and 2.4 (1.58 to 3.79) among relatives with epilepsy in comparison with relatives without epilepsy. Migraine risk was highest in probands with epilepsy due to head trauma, but it was significantly higher in every subgroup of probands than in unaffected relatives when probands were stratified by seizure type, age at onset, etiology of epilepsy, and history of epilepsy in first-degree relatives. Age-specific incidence of migraine among probands was increased to a greater extent after onset of epilepsy than before, but it was also significantly increased more than 5 years before onset and 1 to 5 years before onset. These results indicate that migraine and epilepsy are strongly associated, independent of seizure type, etiology, age at onset, or family history of epilepsy.
306 citations
TL;DR: To characterize the natural history of seizures, epilepsy, and EEG abnormalities in males with fragile X syndrome, patients were investigated for seizures and EEG findings.
Abstract: Summary: Purpose and Methods: One hundred and ninety-two fragile X male patients were investigated for seizures and EEG findings, 168 in a retrospective and 24 in another prospective study, to characterize the natural history of seizures, epilepsy, and EEG abnormalities in males with this syndrome.
Results: Seizures were documented in 35 (18.2%) of 192 patients; they never started before the age of 2 years or after the age of 9 years. Seizures were frequently of the complex partial type and less frequently of the partial motor and generalized type. Seizures involving frontal and temporal lobes were commonly seen and were usually well controlled by anticonvulsants. In the majority of young fragile X patients studied, an age-related paroxysmal EEG pattern was found, which showed neurophysiologic characteristics very similar to those of the centrotemporal spikes.
Conclusions: These findings confirm that fragile X syndrome can be considered a genetic model of epilepsy.
243 citations
TL;DR: The severe nature and intractability of LGS emphasizes the need for the development of specific AEDs which would completely modify the quality of life for these patients, including specific generalized epilepsies arising from the frontal lobe.
Abstract: One of the most challenging areas in nosology is in the field of severe generalized epilepsy of early childhood. This is certainly true in the case of Lennox-Gastaut syndrome (LGS), an age-related epileptogenic encephalopathy which comprises several types of generalized seizures including tonic seizures, atypical absence seizures and frequent status epilepticus. EEG shows generalized slow spike waves, and as the disease progresses, cognitive functions deteriorate. LGS is listed in the 1989 classification of the International League Against Epilepsy alongside epilepsy with myoclonic astatic seizures and West's syndrome. A number of variants or atypical forms have been proposed. As a result, differential diagnosis presents a major challenge and includes specific generalized epilepsies, i.e., metabolic or inflammatory; secondarily generalized epilepsies, i.e., those arising from the frontal lobe; and severe forms of idiopathic generalized epilepsy, i.e., Doose syndrome. Antiepileptic drug (AED) treatment of LGS has been disappointing. Results obtained from anterior callosotomy have been promising, but only a small number of patients have been evaluated. Although the syndrome is rare, the severe nature and intractability of LGS emphasizes the need for the development of specific AEDs which would completely modify the quality of life for these patients.
227 citations
Cites background from "A new type of epilepsy : benign par..."
...Gastaut (1982) reported occasional cases of SSW predominance in the posterior leads, but this has been questioned (Niedermeyer, 1988)....
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TL;DR: A study is currently being undertaken at the National Hospital for Neurology and Neurosurgery and The Chalfont Centre for Epilepsy with the aim of developing and evaluating diagnostic criteria for parietal and occipital epilepsy.
Abstract: Parietal and occipital seizures have been investigated relatively little. Recent interest in seizures of frontal lobe origin has led to a definition of several well-identifiable clinical patterns of frontal lobe seizures. A similar delineation of the clinical and EEG pattern of parietal and occipital epilepsy, using modern investigations, appears appropriate, not least because the few reported series of surgical treatment in parietal and occipital epilepsy have indicated that operation may be relevant in selected cases, not only for removal of space-occupying lesions. Such a study is currently being undertaken at the National Hospital for Neurology and Neurosurgery and The Chalfont Centre for Epilepsy with the aim of developing and evaluating diagnostic criteria for these seizures. From the existing literature we can conclude that precise incidence and prevalence are largely unknown. A recent community-based study of 252 subjects with partial epileptic seizures in an epileptic population of 594, showed that parietal seizures and seizures of posterior origin each comprised 6.3% and central or sensory-motor seizures comprised 32.5% of focal seizures in the 160 cases in which seizures could be subclassified (Manford et al., 1992). This incidence seems low for occipital seizures as compared with the 1953 study by Gibbs and Gibbs, who observed occipital epileptiform activity in 8% of subjects with focal epilepsy. The most prominent clinical manifestations of parietal epilepsy are elementary sensory phenomena at the beginning of seizures and elementary visual hallucinations in occipital epilepsy. These symptoms are not associated solely with posterior hemisphere epilepsy, however, and more studies are obviously needed to define how close this relation is. Scalp EEG is frequently negative or maybe misleading; furthermore, spread of epileptic discharges from the parietal and occipital lobes to frontal and temporal regions may obscure seizure origin. Because of these controversial symptoms, diagnostic criteria may be difficult to define. The wide difference in clinical and EEG manifestations between reported series of parietal and occipital epilepsy also reflects a considerable problem with patient sampling. Classification of epilepsy according to the anatomic division of the brain may be arbitrary, and it may be appropriate to define epileptic syndromes such as sensorimotor seizures or occipitotemporal seizures that cross such artificial divides.
191 citations
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01 Jan 1941
TL;DR: The author's major concern is with spina bifida cystica, beginning with a unique description of the spinal lesion and associated dissection of the nerve roots and peripheral nerves in 12 cadavers.
Abstract: is needed, both to increase the number of survivors and, more important, to improve the quality of the survivors, is shared by the reviewer. The introductory chapters discuss terminology and the management of spina bifida occulta. The author's major concern is with spina bifida cystica, beginning with a unique description of the spinal lesion and associated dissection of the nerve roots and peripheral nerves in 12 cadavers. The roots and peripheral
1,124 citations
TL;DR: An effort is made to describe objectively convulsions activated by Metrazol in patients admitted to the epilepsy unit for the study of intractable seizures.
Abstract: ALTHOUGH bearing the hallmark of the Montreal school, this study derives from the branch of electroencephalography and clinical neurohysiology of the National Institute of Neurofogical Diseases and Blindness. In the main, an effort is made to describe objectively convulsions activated by Metrazol in patients admitted to the epilepsy unit for the study of intractable seizures. Data upon 119 such cases is included, 52 of which were classified as having a unilateral or a bilateral temporal origin. Electroencephalogra hic tracings done
275 citations
TL;DR: In the present paper, an in vitro incubation study was undertaken with cat brain hemispheres in toto, one covered by the meninx and the other devoid of this enveloping membrane, and it will be shown that the incorporation of amino acids in the naked hemisphere is higher than in the other.
Abstract: IN THE STUDY of the barrier phenomena in the brain, the role of the cerebral lining epithelia has not been fully evaluated. In the case of the ventricular cavities, several authors have demonstrated that different substances diffuse without restriction through the ependymal lining into the adjacent nervous parenchyma.lJ Others suggest a differential ependymal permeability according to anatomic regional variations.\".\" In our laboratory, we have found that penetration of radioactive amino acids into the brain apparently is accomplished by an exchange process, without significant increase in their net tissue levels.5Jj In the subarachnoid space, most studies utilize the technique of a single intracisternal injection for administration of materials to the brain.7.8 In this way, it is difficult to correlate concentration in the tissue with the real concentration in the cerebrospinal fluid (CSF) because it is not a stagnant fluid and because of the tortuosity and irregular shape of the space where it flows. Consequently, the role of the pia mater-the lining membrane covering the hemispheres-in the penetration of substances into the brain is not easy to assess. In the present paper, an in vitro incubation study was undertaken with cat brain hemispheres in toto, one covered by the meninx and the other devoid of this enveloping membrane. It will be shown that the incorporation of amino acids in the naked hemisphere is higher than in the other. Because, to our knowledge, this is the first time that uptake of materials by the brain tissue in vitro through only one absorption surface has been studied, we compare results with those from incubating brain slices under our experimental arrangement.
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