Adult‐onset photosensitivity: clinical significance and epilepsy syndromes including idiopathic (possibly genetic) photosensitive occipital epilepsy

To evaluate the clinical associations of adult‐onset photosensitivity, we studied the clinical and EEG data of patients who were referred due to a possible first seizure and who had a photoparoxysmal response on their EEG. Patients with clinical evidence of photosensitivity before the age of 20 were excluded. Of a total of 30 patients, four had acute symptomatic seizures, two had vasovagal syncope, and 24 were diagnosed with epilepsy. Nine of the 24 patients had idiopathic (genetic) generalized epilepsies and predominantly generalized photoparoxysmal response, but also rare photically‐induced seizures, while 15 had exclusively, or almost exclusively, reflex photically‐induced occipital seizures with frequent secondary generalization and posterior photoparoxysmal response. Other important differences included a significantly older age at seizure onset and paucity of spontaneous interictal epileptic discharges in patients with photically‐induced occipital seizures; only a quarter of these had occasional occipital spikes, in contrast to the idiopathic (genetic) generalized epilepsy patients with typically generalized epileptic discharges. On the other hand, both groups shared a positive family history of epilepsy, common seizure threshold modulators (such as tiredness and sleep deprivation), normal neurological examination and MRI, a generally benign course, and good response to valproic acid. We demonstrated that photosensitivity can first occur in adult life and manifest, either as idiopathic (possibly genetic) photosensitive occipital epilepsy with secondary generalization or as an EEG, and less often, a clinical/EEG feature of idiopathic (genetic) generalized epilepsies. Identification of idiopathic photosensitive occipital epilepsy fills a diagnostic gap in adult first‐seizure epileptology and is clinically important because of its good response to antiepileptic drug treatment and fair prognosis.     

The National General Practice Study of Epilepsy. The syndromic classification of the International League Against Epilepsy applied to epilepsy in a general population.

In this prospective, population-based study of 594 cases of newly diagnosed epilepsy, proportions in categories as defined by the International League Against Epilepsy (ILAE) were as follows: (1) localization-related epilepsies: 1.1* idiopathic, 1.2%; 1.2* symptomatic, 16.2%; and 1.3 cryptogenic, 24.6%; (2) generalized epilepsies: 2.1* idiopathic (idiopathic generalized epilepsy) with 3-Hz spike and wave: absence epilepsy, 2.2%; juvenile myoclonic epilepsy, 1.5%; and nonspecific idiopathic generalized epilepsy, 5.6%; 2.3.1* symptomatic generalized epilepsies, 1.5%; 2.3.2* specific syndromes with generalized epilepsy, 0.3%; 3.2 seizures without unequivocal focal or generalized features, 32%; 4.1 situation-related syndromes, isolated seizures, 9.9%; seizures due to acute toxic or metabolic cause,* 4.5%. Only 33.6% were in diagnostic ILAE categories (asterisks) and many rare syndromes were not represented. The remainder (66.4%) were in various nonspecific categories. Only 24% of localization-related epilepsies could be clinically localized to a single ILAE-proposed site of origin and of these best localized cases, 14% had strongly discordant imaging or electroencephalograms. These major problems in applying the ILAE classification to epilepsy in the general population and its underemphasis of modern imaging techniques are discussed.     

Classification of epilepsies and epileptic syndromes using the 1989 International League against epilepsy classification: A hospital-based study of 1,250 patients in a developing country

The 1989 International Classification of Epilepsies and Epileptic Syndromes (ICE) of the International League Against Epilepsy was used to study the distribution of epilepsies and epileptic sundromes in 1,250 patients attending an Epilepsy Clinic in Sri Lanka. Of this largely adult population, 917 (73.4%) were classified as having localization-related epilepsy, 228 (18.2%) as having generalized epilepsy, and 104 (8.3%) as having epilepsy undetermined as to whether focal or generalized. Only one case was termed special syndromes because the definition of epilepsy excluded situation-related seizures. Of the localization-related epilepsies, the majority (82.9%) were cryptogenic and 14.5% were symptomatic. Of them, approximately one third were temporal lobe epilepsies; about half the cases could not be localized to a specific lobe of the brain. The generalized category consisted of 214 (93.9%) idiopathic and 14 (6.1%) symptomatic epilepsies. Juvenile myoclonic epilepsy (JME) was the most common idiopathic generalized epilepsy (115 cases, 50.4%). Epilepsies with specific modes of seizure precipitation accounted for 6.8% (85 cases) of the total series. All 85 cases were localization-related epilepsies; 76 had eating epilepsy (EE), and 9 had self-induced epilepsy (SIE). Although some overlap occurred between certain subcategories and specific localization of localization-related epilepsies was difficult, the 1989 ICE was relevant and applicable in a clinical setting with limited investigatory facilities.     

Classification of epilepsy syndromes and role of genetic factors

In this report the types of epilepsy syndromes seen in children in a tertiary referral center in Beirut, Lebanon were studied and the importance of consanguinity and family history in the occurrence of these syndromes was investigated. Records of 230 pediatric patients evaluated during a 1-year period with the diagnosis of single seizure, febrile seizure, or epilepsy were reviewed. Each patient was classified according to the International League Against Epilepsy classification. The occurrence of consanguinity, of family history of febrile seizures or epilepsy, and of other variables was noted. Thirty-six percent of patients were diagnosed with localization-related epilepsy, 21.7% with generalized epilepsy, 11.7% with undetermined generalized or focal, and 24.3% with special syndromes. Twelve percent of patients were diagnosed with idiopathic, 15.1% with symptomatic, and 30.3% with cryptogenic epilepsies. Consanguinity was more common in patients with symptomatic and cryptogenic epilepsies than in patients with idiopathic epilepsies or with incidental seizures (P < 0.05). Family history of epilepsy was more common in patients with symptomatic, cryptogenic, and idiopathic epilepsies than in patients with incidental seizures (P < 0.05). Family history of febrile seizures but not consanguinity was more common in patients with febrile seizures (P < 0.05). We conclude that genetic factors are important not only in idiopathic epilepsies and febrile seizures but also in cryptogenic and symptomatic epilepsies.     

Incidence of epileptic syndromes in Rochester, Minnesota: 1980-1984

PURPOSE: To determine the incidence and the distribution of epileptic syndromes in a well-defined population. METHODS: By using the records-linkage system of the Rochester Epidemiology Project, we screened all the residents of Rochester, Minnesota, who received a diagnosis of seizures, convulsions, or epilepsy from 1980 through 1984. One hundred fifty-seven residents with incident epilepsy (recurrent unprovoked seizures) were classified by using the International League Against Epilepsy (ILAE) Classification of the Epilepsies and Epileptic Syndromes. Residents with special syndromes were excluded. With a pretested algorithm, patients were classified at three levels of specification: major syndromic groups (e.g., localization-related syndromes), syndromic subgroups (e.g., idiopathic epilepsy with age-related onset), and whenever possible, individual syndromes. RESULTS: All but one patient were classified into major syndromic groups and subgroups. The annual age-adjusted incidence per 100,000 population was 52.3 cases (34.9 for localization-related epilepsies; 7.7 for generalized epilepsies; 9.7 for undetermined epilepsies). Incidence was 0.2 for idiopathic, 17.2 for cryptogenic, 17.5 for symptomatic localization-related epilepsies, 3.7 for idiopathic, 1.7 for symptomatic or cryptogenic (age-related), and 2.3 for symptomatic (non age-related) generalized epilepsies. CONCLUSIONS: With the exception of idiopathic epilepsies, the incidence of the major syndromic categories in our study was higher than that provided by previous population-based studies.     

Refractory idiopathic absence status epilepticus: A probable paradoxical effect of phenytoin and carbamazepine

PURPOSE: To compare the frequency of seizures and status epilepticus and their response to first-line drugs in patients with idiopathic generalized epilepsies receiving carbamazepine or phenytoin to those receiving other drugs or no treatment. METHODS: We performed a retrospective chart review of all cases of idiopathic generalized epilepsies treated by the authors between 1985 and 1994. We compared seizure frequency and mean intravenous benzodiazepine dose required to control absence status epilepticus, intraindividually in subjects on carbamazepine or phenytoin before and after discontinuation of these compounds, and interindividually to subjects without treatment or receiving other drugs. RESULTS: Bouts of absence or tonic-clonic status epilepticus and seizures in subjects treated with phenytoin or carbamazepine at therapeutic concentrations were considerably more frequent and proved intractable to treatment with valproic acid or benzodiazepines, compared with a cohort of subjects also with idiopathic generalized epilepsies, but naive to, or receiving subtherapeutic or therapeutic doses of other agents. CONCLUSIONS: Our observations strongly suggest that therapeutic concentrations of phenytoin and carbamazepine exacerbate idiopathic generalized epilepsies. Subjects in whom absence is one of the seizure types seem at a particularly high risk for responding paradoxically. These findings underscore the value of accurate classification of seizures and particularly the syndromic approach to diagnosis and point to the potential for iatrogenic complications with indiscriminate use of antiseizure drugs.     

Somatosensory evoked spikes and epileptic seizures: a study of 385 cases.

We examined 385 children whose EEG showed high voltage potentials evoked by taps applied to one or both feet or hands (SES). The relationship between characteristics of SES and the occurrence of epileptic seizures and the characterization of epileptic syndromes were studied. Ninety-one children (23.6%) had epilepsy, 42 (10.9%) had only febrile convulsions and 252 children had other complaints. Epilepsy occurred in a higher proportion of cases when: SES by foot tapping were multiphasic, with high amplitude or SES were obtained by hand stimulation and there was spontaneous epileptiform activity in the EEG. The following epileptic syndromes were diagnosed: benign childhood epilepsy with centrotemporal spikes in 21 cases, benign epilepsy of childhood with occipital paroxysms in 2, benign psychomotor epilepsy in 1, "partial idiopathic others" in 43, generalized idiopathic in 8, symptomatic epilepsies in 13 and undetermined in 3 cases. In most cases SES were observed in children without evidence of cerebral organic lesion, suggesting the existence of an age-related, functional mechanism. Some characteristics of SES and the occurrence of spontaneous epileptiform activity showed a positive association with epileptic seizures. SES occurred in different types of partial and generalized epilepsies of childhood but in nearly 50% of the cases with epilepsy, there was a benign condition involving mainly the parietal lobe with versive, unilateral and sleep-generalized seizures.     

Epilepsies and time to diagnosis. Descriptive results of the CAROLE survey

CAROLE is a prospective survey of children and adults who experienced epileptic unprovoked seizure(s) diagnosed for the first time between May 1 1995 and June 30 1996 by 243 French neurologists and neuropediatricians. Case records forms at entry allowed to compare patients who had a single seizure or several seizures prior to diagnosis. In patients with recurrent seizures, the time elapsed between the onset of attacks and the diagnosis (diagnostic delay) was looked for. Half of the 1942 included individuals already experienced more than one seizure when diagnosed. Due to natural history of epilepsies, 13 p.100 of the patients did not come to medical attention after a single seizure. Time to diagnosis ranged from 0 day to 52 years. While the overall median delay was 6 days, it ranged from 0 day to 7-8 months according to the type of seizure and the epilepsy syndrome. Two thirds of generalized convulsive seizures were immediately diagnosed versus one third of partial seizures. One half of infantile spasms were identified within 2 weeks versus 6 weeks in complex partial seizures, 4 months in absence seizures, 6 months in simple partial seizures, and 7 months in myoclonic seizures. Three quarters of idiopathic partial epilepsies were diagnosed within 4 weeks versus 3 months in symptomatic generalized epilepsies, 8 months in symptomatic partial epilepsies, 15 months in idiopathic generalized epilepsies, 30 months in cryptogenic partial epilepsies, and 33 months in undetermined epilepsies. So, the time elapsed between a first epileptic event and its diagnosis is epilepsy-dependent: seizure type and cause. Other reasons of diagnostic delay do exist: doctor and patient. They will be addressed in another study.     

Prevalence and characteristics of epilepsy in the Aeolian islands

PURPOSE: To estimate the prevalence and define the clinical characteristics of epileptic disorders in the 13,431 residents of the Sicilian Aeolian archipelago, on June 1, 1999. METHODS: All established or suspected cases were identified by the neurologists of our working group from available medical information sources. Possible epilepsy cases were then evaluated by the epileptologists by using a standardized questionnaire. The patients were further reviewed by the whole research team to confirm the clinical diagnosis. For a more detailed syndromic definition, some patients underwent EEG or neuroradiologic investigations or both. RESULTS: The crude point prevalence rate of active epilepsy was 3.13 (95% confidence interval, 2.2-4.2). The prevalence rate age-adjusted to the 2001 Italian population was 3.01. Females had a slightly higher prevalence rate than did males. The highest age-specific prevalence was found in patients aged 5 to 14 years (5.05) and in those aged 65 to 74 years (5.41). Partial seizures with or without secondary generalization were more common (61.7%) than were generalized seizures. Eighty-three percent of cases had symptomatic or cryptogenic localization-related epilepsies, and 8.5% had idiopathic (generalized or partial) epilepsies. Epilepsy was unclassified in 8.5% of cases. CONCLUSIONS: The prevalence of active epilepsy in the Aeolian islands is lower than that in other developed areas, including northern Italy, but is similar to that in Sicily. Partial seizures were the most common type, and localization-related symptomatic epilepsies were the largest syndromic category.     

Epilepsies of neonatal onset: seizure type and evolution

Most neonatal seizures are occasional seizures and not true epilepsy. This study investigates seizure types of true neonatal epilepsies and their evolution with development. Seventy-five children with epilepsies of onset within 1 month of life, who were examined between 1970 and 1995, and whose seizure types could be confirmed with ictal EEG recordings, were studied. The patients were followed up for a minimum of 3 years and the evolution of epileptic syndromes was investigated. Sixty-three (84%) of 75 patients had partial seizures, while nine had generalized seizures, and only three had both generalized and partial seizures. Twenty-three of 24 neonates with benign familial or non-familial neonatal convulsions presented with partial seizures; these syndromes should not necessarily be categorized into generalized epilepsy as they are in the present International Classification. Age-dependent changes were a common feature of symptomatic neonatal epilepsies. Eighteen (41%) of 44 patients with symptomatic epilepsies of neonatal onset developed West syndrome in infancy. Fifteen (83%) of these 18 patients presented with symptomatic localization-related epilepsy in the neonatal period. In seven of these 15 patients, West syndrome was followed by localization-related epilepsy. Symptomatic localization-related epilepsy with transient West syndrome in infancy is another type of age-dependent epileptic syndrome.     

Epilepsy in children in Navarre, Spain: epileptic seizure types and epileptic syndromes

Data for children 1 month to 15 years of age at the time of diagnosis of epilepsy were recorded from the children's hospital "Virgen del Camino" in Pamplona (Spain) from January to December 2005. International League Against Epilepsy criteria were used for diagnoses. A total of 365 children were recruited into the study. Mean age at diagnosis was 5.97 years, and time of follow-up was 4.6 years. Etiology was idiopathic in 166 (45.5%), cryptogenic in 106 (29.0%), and symptomatic in 93 (25.5%). Focal seizures were seen in 52.9% of the patients, generalized epilepsy in 43.5%, and 3.6% were not determined. In infants, West syndrome (34.1%) and focal symptomatic seizures (24.4%) were the most prevalent syndromes. In early childhood, the main syndromes were cryptogenic focal epilepsies (17.7%) and Doose syndrome (12.8%). In school-aged children, benign epilepsies (27.3%) and absences (24.5%) were prevalent. In adolescents, cryptogenic focal epilepsies (26.6%) and benign epilepsies (23.4%).     

Classification of epilepsies and epileptic syndromes of childhood according to the 1989 ILAE classification

We attempted to classify, according to the 1989 International Classification of Epilepsies and Epileptic Syndromes, 255 children under 6 years of age consecutively examined for 1 year in an outpatient clinic of child neurology of the university hospital. All the patients were classified as follows: 57 (22.4%) localization-related epilepsies (37 symptomatic and 20 cryptogenic cases), 55 (21.6%) generalized epilepsies (7 idiopathic, 46 symptomatic cases, and 2 cryptogenic), 33 (12.9%) undetermined epilepsies, and 110 (43.1%) special syndromes (103 with febrile convulsions and 7 others). The fact that a substantial proportion of the patients lacked specific clinical or EEG features suggesting individual epileptic syndromes made subclassification of symptomatic and cryptogenic localization-related epilepsies sometimes difficult. The boundary of cryptogenic cases was considered to be somewhat arbitrary because it was somewhat dependent on the extent of the diagnostic examinations. Further elucidation of new epileptic syndromes in symptomatic generalized epilepsies is necessary because 14 cases (30.4%) of 46 symptomatic generalized epilepsies were classified as “other symptomatic epilepsies”.     

Abstracts of the 38th Congress of the Japan Epilepsy Society, Shizuoka, Japan, September 30-October 1, 2004

Diagnosis and Treatment of Idiopathic Focal Epilepsies (Benign Partial Epilepsies) in Infancy and Childhood. ¹ Tamiko Negoro ( ¹ Department of Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan ). Introduction: According to the revised classification of epilepsies and epileptic syndromes proposed by the Commission on Classification and Terminology of the International League Against Epilepsy (1989), benign childhood epilepsy with centrotemporal spikes (BCECT), childhood epilepsy with occipital paroxysms (Gastaut‐type late‐onset CEOP), and primary reading epilepsy were included in the idiopathic localization‐related epilepsies (ILRE). Since then, new epileptic syndromes such as Panayiotopoulos‐type early‐onset benign childhood occipital epilepsy (also known as benign childhood epilepsy with occipital paroxysms or BCEOP) and benign partial epilepsies in infancy (BPEI) have been additionally included as ILRE. The diagnostic criteria for benign partial epilepsies include (1) normal neurological examination; (2) normal intelligence; (3) normal neuroimaging; (4) a family history of benign‐type seizures; (5) brief stereotyped seizures; (6) frequent nocturnal occurrence; (7) easy control with antiepileptic drugs (AEDs), except ethosuximide; and (8) remission before adolescence. The EEG features include (1) normal background activity; (2) spikes with a characteristic morphology and location; (3) sleep activation; and (4) occasional generalized paroxysms. In our 114 cases of childhood onset localization‐related epilepsies (LRE) diagnosed between 1997 and 2000, 48 cases (42%) were diagnosed as ILRE and 66 were cryptogenic or symptomatic LRE. Among the 48 ILRE cases, 28 (58%) were classified as BCECT, six (13%) as BPEI, five (10%) as BCEOP, two (4%) as atypical benign partial epilepsy, and seven (15%) as unclassified. Carbamazepine (CBZ), valproate (VPA) and clonazepam (CZP) were equally effective in controlling the seizures in our ILRE patients. This review describes briefly the diagnosis and treatment of BCECT, BCEOP, and BPEI. Benign Childhood Epilepsy with Centrotemporal Spikes: BCECT is the most common ILRE (also known as idiopathic focal epilepsies) in infancy and childhood. Sylvian seizures (simple partial seizures or focal motor or sensory seizures beginning unilaterally at the lower part of the face) and EEG findings (central‐midtemporal high‐amplitude, repetitive sharp waves) are quite stereotyped, and diagnosis of typical cases is relatively easy. Frontal lobe seizures with bilateral perioral twitches or unilateral eyelid twitches may sometimes be misdiagnosed as Sylvian seizures. Since status epilepticus is very rare and many patients only have occasional seizures, many physicians choose not to treat the disorder. If treatment is necessary, the seizures are usually easily controlled with carbamazepine, valproic acid, or clonazepam. Continuation of AEDs is not necessary after the EEG has normalized around puberty. The prognosis is excellent. However, a small percentage of patients may show atypical evolutions such as atypical benign partial epilepsy, epilepsy with continuous spike‐and‐waves in slow wave sleep, or epilepsy with centrotemporal spikes and oromotor deficit. Seizure and EEG exacerbations by AEDs, especially carbamazepine, have to be considered. Early recognition and proper treatment are necessary for these conditions. Benign Childhood Epilepsy with Occipital Paroxysms: BCEOP is also known as Panayiotopoulos‐type early‐onset benign childhood occipital epilepsy. The mean age at first seizure is around 5 years. Seizures comprise an unusual constellation of autonomic, mainly emetic, syndromes with unilateral deviation of the eyes and impairment of consciousness. Visual symptoms, which are the main symptoms in Gastaut‐type, late‐onset CEOP, are exceptions in BCEOP. According to our 41 cases examined between 1984 and 1993, two‐thirds of the patients show hemi‐ or generalized convulsions and one‐fourth experience prolonged seizures for longer than 30 minutes. EEG findings (bilateral occipital paroxysms) consist of some extraoccipital abnormalities especially in the frontal area (48% of our cases) or generalized paroxysmal discharges (32% of ours cases), together with various migrations of spike foci. EEG findings seem to normalize around the age of 10 years. Many patients only have occasional seizures and these seizures are usually easy to control with carbamazepine or valproic acid. The prognosis is excellent. Prevention of status epilepticus is the major problem in this disorder. Recently, new concepts including early‐onset benign occipital seizure susceptibility syndrome (EBOSS) and Panayiotopoulos syndrome (idiopathic susceptibility to early‐onset benign childhood seizures with mainly autonomic symptoms), have been introduced for BCEOP. Benign Partial Epilepsies in Infancy: BPEI comprise two forms. One is partial epilepsy with complex partial seizures (CPS) and the other is partial epilepsy with secondarily generalized seizures (SGS). Onset is mostly during the first year of life. Seizures often occur in clusters and are characterized by motion arrest, decreased responsiveness, staring or blank eyes often with automatisms, and mild convulsive movements in the CPS form; and motion arrest or opening of eyes with staring or blank eyes followed by generalized tonic–clonic seizures in the SGS form. Interictal EEGs mostly show no abnormal findings. According to the ictal EEGs, the most frequent site of seizure origin is in the frontal or temporal area in the CPS form; and central, parietal, or occipital area in the SGS form. Treatment with carbamazepine, phenobarbital, zonisamide or valproic acid immediately stops the cluster of seizures. The prognosis is excellent. Recently, sleep‐related and low‐voltage Rolandic and vertex spikes have been reported as an EEG marker of benignity in this disorder. Most of benign infantile convulsions may belong to partial epilepsy with SGS, although confirmation with ictal EEG recording is necessary for accurate diagnosis.     

Experience with the International League Against Epilepsy Classifications of Epileptic Seizures (1981) and Epilepsies and Epileptic Syndrome (1989) in Epileptic Children in a Developing Country

Four hundred eighty-three epileptic children attending the Pediatric Epilepsy Clinic at Bai Jerbai Wadia Hospital for Children, Bombay, India were classified according to the International League Against Epilepsy (ILAE) classification of epileptic seizures (1981) and epilepsies and epileptic syndromes (1989). The predominant seizures were partial (53.6), generalized (40.3%), and unclassifiable (6%). In epilepsies and epileptic syndromes, 55.3% were partial, 27% were generalized, 13.5% were undetermined, and 4.1% were special syndromes. Although our results were similar in many respects to those of other reported series, some differences were observed in the incidence of partial and generalized seizures, and partial and generalized epileptic syndromes and their subgroups, such as idiopathic, symptomatic, and cryptogenic partial syndromes, idiopathic generalized syndromes, and symptomatic specific syndromes. These differences are probably due to different age limits, methods of case ascertainment and inclusion criteria, different genetic and environmental factors, variable interpretation of clinical and EEG features, and lack of facilities for investigation in developing countries. Despite various limitations, we were able to classify most cases; the ILAE classification can be used in developing countries so that comparison can be made with other studies.     

Classification of epilepsies and epileptic syndromes in a child neurology unit

We attempted to classify, according to the 1989 International Classification of Epilepsies and Epileptic Syndromes, 1076 patients examined during a 4-year period (1 January 1988 to 31 December 1991) in the Child Neurology Unit. We aimed to determine the proportion of the clearly defined epileptic syndromes or the non-specific categories of the International Classification of Epilepsies and Epileptic Syndromes, to estimate the relative incidence of different categories and to review the criteria for defining epileptic syndromes. The proportion in categories as defined by the International Classification of Epilepsies and Epileptic Syndromes in our patients was as follows. Localization-related epilepsies and syndromes: idiopathic 3.15%, symptomatic 17%, cryptogenic 9.20%. Generalized epilepsies and syndromes: idiopathic 20.35%, cryptogenic or symptomatic 14.68%, symptomatic 11.5%. Epilepsies and syndromes undetermined whether focal or generalized: with both generalized and focal seizures 7.8%, without unequivocal focal or generalized features 1.68%. Special syndromes: situation-related seizures: febrile convulsions 12.83%, isolated seizures or isolated status epilepticus 1.02%, seizures due to an acute toxic or metabolic event 1.20%. The presence of non-specific categories in the International Classification of Epilepsies and Epileptic Syndromes enables the categorization of all patients but it gives the false impression of diagnostic precision to what are essentially uncertain cases.     

Photosensitivity in epileptic syndromes of childhood and adolescence.

PURPOSE: Photosensitivity, a reaction of the brain to external photic stimulation, can be graded from 1 to 4, and is most frequently seen in the first decades of life. This study investigated photosensitivity in children with epilepsy. METHODS: A retrospective study performed in the neuropaediatric department of the largest paediatric hospital in Kiel, treating patients at all medical care levels. The clinical data and EEG records of 566 patients with the most common epileptic syndromes were analyzed, in particular regarding photosensitivity. Their EEGs included application of intermittent light stimulation using standard techniques at twice the minimum. RESULTS: The proportion of photosensitive patients was significantly higher in the paediatric cohort than in adult patients, as published in the literature: 46% of patients with generalized epilepsies showed photosensitivity as compared to 20% with focal epilepsies. Photosensitivity was more common in idiopathic generalized epilepsy (IGE), (epilepsy with grand mal on awakening, 74%; juvenile absence epilepsy, 56%; juvenile myoclonic epilepsy, 50%; childhood absence epilepsy, 44%) than in focal types (idiopathic partial - Rolandic epilepsy, 23%; symptomatic/cryptogenic type of epilepsy, 16%), while in patients who experienced occasional seizures (neonatal/febrile seizures), this ranged between 40% and 23%, respectively. The generalized photoparoxysmal response, (PPR), grades 3 and 4 were found significantly more often in patients with IGE (92%) than in patients with focal epilepsies. Finally, the female preponderance was confirmed (37% to 27% of all epilepsies). CONCLUSIONS: Photosensitivity can be detected both in patients with IGE, with idiopathic and symptomatic/cryptogenic types of focal epilepsies, and with epileptic (occasional) seizures. PPR grades 3 and 4 are the most common in IGE.     

Sleep seizures versus wake seizures: a comparative hospital study on clinical, electroencephalographic and radiological profile

Background: Epileptic seizures, predominantly or exclusively during sleep had been the focus of attention for many electroencephalographers. Though few epileptic syndromes are associated with sleep seizures (SS) its frequencies in Indian patients is still unknown. Aim: To find out the patterns of epilepsies in patients having SS and compare them with patients having wake seizures (WS). Setting and Design : Open label hospital based study. Materials and Methods: One hundred and forty-four (13%) patients having predominantly SS were compared with 976 (87%) patients of WS by various clinical, electrophysiological and radiological factors. Statistical Analysis: Chi square test and student T test, using software SPSS (version 10, 1999) was applied to compare various parameters. Relative risk was calculated by 2 x 2 contingency table. Results: The seizure semiology was better defined in patients with WS and GTCS was more common in SS ( P = 0.001). Wake-electroencephalogram (EEG) was abnormal in significantly ( P = 0.001) higher number of patients with WS. Symptomatic etiologies were found in more than half patients. Left lobe involvement was more common in patients having SS ( P = 0.000). After symptomatic, idiopathic generalized and frontal lobe epilepsy were most frequent with SS. Undetermined epilepsy was found in 37 (25.7%) patients with SS. Conclusion: Epilepsies associated with SS were less frequent and had symptomatic cause in most cases. Left hemispherical and frontal lobe lesion were more commonly associated with SS. Frontal lobe and idiopathic generalized epilepsy was most frequent in patients of SS. Sleep EEG should always be done in patients with sleep seizures.     

Sulthiame add-on therapy in children with focal epilepsies associated with encephalopathy related to electrical status epilepticus during slow sleep (ESES)

Purpose: In children with symptomatic or idiopathic focal epilepsies, their disease may evolve into an epileptic encephalopathy related to continuous spike and wave during slow sleep (CSWS) or electrical status epilepticus during slow sleep (ESES). ESES syndrome implies serious risks of neuropsychologic impairment, and its treatment has frequently been disappointing. The aim of this study is to present our experience using sulthiame as add‐on treatment in 53 patients with ESES syndrome that was refractory to other antiepileptic drugs (AEDs). Methods: Neurologic examinations, cerebral magnetic resonance imaging (MRI), and repeated prolonged sleep electroencephalography (EEG) studies were performed in all cases. Data about school achievements and or neuropsychological evaluations were obtained repeatedly during the follow‐up of 1.5–16 years. Sulthiame was added in doses ranging between 5 and 30 mg/kg/day. Key Findings: Since add‐on of sulthiame, 10 of 28 patients in the symptomatic group became seizure free: 4 patients with normal EEG studies and 6 with residual spikes. Nine of 28 patients showed a significant reduction in number of seizures and presented spikes but no ESES on EEG. The other nine cases showed neither clinical nor EEG improvement. A striking result was that 3 of 11 children with unilateral polymicrogyria and ESES syndrome became seizure free, and in another six a significant improvement in frequency of seizures and in EEG abnormalities seemed to be related to the add‐on of sulthiame. Twenty‐one of the 25 patients in the idiopathic group became seizure free and without ESES in <3 months after add on of sulthiame. In two of the patients the changes were seen in a few days. Significance: We understand that sulthiame may be effective as add‐on treatment in children with ESES syndrome.     

Clinical Characteristics of Grand mal (Generalized Tonic-Clonic Convulsion): A Contribution to the Ictal Symptomatology

Abstract: The purpose of this study is to evaluate the possibilities of whether the long-ranged clinical course of generalized tonic-clonic convulsions (GTC) and the ictal symptomatology were modified in accordance with the patho-etiological backgrounds of epilepsies as reflected by the International Classification (1970). The study consisted of two parts: 1) A transsectional survey of 1,896 patients dealing with the onset age and seizure frequency of GTCs, the time of occurrence in the sleeping-waking cycle, the GTC monosymptomatic course in comparison with that of combined seizures and the seizure prognosis as a result of drug response. It was found that the mode of occurrence and the clinical course unequivocally differed in accordance with the type of epilepsies: primary (idiopathic) generalized, secondary (symptomatic) generalized and partial. 2) An analysis of the ictal symptomatology of GTC by the use of a simultaneous recording was conducted on 42 GTCs of 42 patients who were carefully selected based on the criteria the authors defined. The clinical features of the convulsive phenomena and concurrent seizure discharges enabled us to subclassify them into three groups; typical, atypical and secondarily generalized. GTCs in patients with partial epilepsies were, of course, all found in the secondarily generalized group while GTCs in patients with primary generalized epilepsies were typical. It was emphasized that atypical GTCs were found in patients either with the Lennox-Gastaut syndrome or with other secondary generalized epilepsies, whereas there was no atypical GTC in patients either with primary generalized or partial epilepsies. The ictal EEG expressions were found crucial in differentiating these three groups in addition to the symmetry and asymmetry of GTCs. It has been discussed that such distinctly different ictal expressions of GTCs may reflect the pathoetiological backgrounds of epilepsies: primary (centrencephalic), secondary (diffuse or multifocal) and partial (localization-related lesional).     

Observations on the misdiagnosis of generalized epilepsy as partial epilepsy: causes and consequences.

More therapeutic options (surgical and pharmacologic) are available for partial than for generalized epilepsies. This report describes and analyzes a possible bias to diagnose focal epilepsies. Data were prospectively collected on patients who underwent noninvasive prolonged EEG-video monitoring over a 2-year period at an epilepsy program. Cases where the diagnosis of 'partial seizures' (after monitoring) was questionable were identified and the data reviewed. Sixteen cases were identified. (a) Six had an idiopathic generalized epilepsy. All had generalized tonic-clonic (GTC) seizures, two had myoclonic seizures, and three had typical absences. All patients had generalized spikes and spike-wave complexes. All had normal IQs and normal brain imaging. One patient underwent invasive EEG. (b) Ten patients had a symptomatic or cryptogenic generalized epilepsy. IQs ranged from 49 to 74 (mean: 63). All patients had diffuse EEG slowing, and generalized ictal EEG patterns. Interictal EEG showed generalized spike-wave complexes in nine, and multifocal spikes in five. Seizures included GTC in all, generalized tonic in four, and atypical absences in two. Two of the 10 patients underwent invasive EEG. The misdiagnosis of generalized epilepsy as partial epilepsy occurs for both idiopathic and cryptogenic or symptomatic generalized epilepsies, more often in the latter case. Risk factors may include: asymmetry in EEG or seizure semeiology, the eagerness to enroll in drug studies or surgical programs, and the lack of team thinking involving several epileptologists. This problem is almost certainly under-reported and may occasionally result in unwarranted invasive procedures.