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At least 19 recordsLinked to original sources
PubMed1998

Language dysfunction in children with Rolandic epilepsy.

Rolandic epilepsy is regarded as the classic example of benign focal epilepsy. However, neuropsychological deficits have been noted in affected children. As Rolandic discharges are mainly distributed over the centrotemporal region, specific interference with language function might be suspected. Therefore, we conducted a standardized neuropsychological assessment in children with Rolandic epilepsy which covered all important aspects of language processing. We measured intelligence Quotient, verbal memory, auditory discrimination, vocabulary, grammar and literacy in 20 children with an active Rolandic focus. Information about performance at school was obtained from teachers by means of a questionnaire. Patients with Rolandic epilepsy failed five of the twelve standardized language tests significantly more often than the normative population and consequently showed impairment of the following functions: reading, spelling, auditory verbal learning, auditory discrimination with background noise and expressive grammar. Thirteen of the 20 children showed language dysfunction with difficulties in two or more of the twelve standardized language tests. In eight of these 13 children the Full Scale Intelligence Quotient was within average range, indicating a specific language deficit. Language dysfunction was closely associated with learning difficulties at school. This study documents a consistent pattern of language dysfunction in children with Rolandic epilepsy which suggests interictal dysfunction of perisylvian language areas.

Adolescent↗
PubMed1999

[Diagnosis and treatment of Rolandic epilepsy].

Rolandic epilepsy belongs to the most frequent forms of epilepsy in children. 43 patients aged 2-14 years were observed. Complex neurologic, genealogic, neurophysiologic and neuroradiologic examinations was performed. There was a microfocal symptomatology in the form of ptosis and syndrome of infantile cerebral paralysis. The changes found at neuroradiologic examination were not related to the location of an epileptic focus and didn't influence the prognosis of the disease. In 10 cases electroencephalographic patterns were observed in clinically healthy relatives. In all the patients a remission of the fits was achieved. Administration of low doses (20 mg/kg daily) of valproic acid (depakin) resulted in a remission of the fits.

Anticonvulsants↗
PubMed2000

Treatment in typical and atypical rolandic epilepsy.

Benign rolandic epilepsy is the most common epilepsy of childhood. Regarding seizure control typical and atypical rolandic epilepsies have a reasonable prognosis. In a recent prospective study STM was shown to control seizures in BECTS and STM is regarded as first line drug for this epilepsy in countries having access to this drug. Otherwise, CBZ is used most often and very effectively in BECTS. However, CBZ has the disadvantage to worsen clinical and EEG features up to the precipitation of CSWS in few patients. Epileptic seizures are often not the main problem in atypical rolandic epilepsies like CSWS or LKS and the amelioration of cognitive dysfunction by epileptic discharge is the prominent aim of an AED therapy. Steroids seem the have the best efficacy in these cases.

Adolescent↗
PubMed2005

The neuropsychological and language profile of children with benign rolandic epilepsy.

PURPOSE: Benign rolandic epilepsy (BRE) has an excellent prognosis for seizures, but recent research has raised concerns using cognition as an outcome measure. Methodologic problems related to recruitment bias and assessment processes are evident in previous studies. With well-defined criteria for inclusion and comprehensive assessment, the aim of this study was to define the cognitive profile of children with BRE and to assess the effect of interictal EEG activity. METHODS: Patients (n=42) were recruited from six EEG laboratories. The EEG features analyzed were spike frequency, trains, and laterality. Comprehensive neuropsychological and language assessments were conducted. Group means on cognitive measures were compared with normative means. Tests were correlated with EEG features. RESULTS: The study demonstrated that children with BRE have normal intelligence and language ability. However, a specific pattern of difficulties in memory and phonologic awareness was found. Furthermore, a large proportion of children had disproportionate scores in these areas compared with intellectual and language ability. EEG features were minimally associated with cognitive difficulties, and no correlation was found with memory indices and tests of phonologic awareness. CONCLUSIONS: Some children with BRE have specific difficulties in memory and phonologic processing skills, not explained by interictal activity. We recommend that pediatricians ask about academic performance specifically in areas of prereading, reading, spelling, and memory. If difficulties are suspected, assessment targeting phonologic awareness and memory are recommended, as they may not be reflected in overall intellectual and language ability. Difficulties in phonologic awareness affect literacy, and memory problems affect academic performance.

Achievement↗
PubMed1997

Adolescent onset of idiopathic photosensitive occipital epilepsy after remission of benign rolandic epilepsy.

PURPOSE: We describe 2 girls, aged 19 years, who experienced a rolandic seizure at ages 4 and 5, respectively, together with the interictal EEG features of benign rolandic epilepsy (BRE). In adolescence both patients developed photosensitive occipital seizures accompanied by spontaneous and photic-induced occipital EEG paroxysms. METHODS: We have been following 33 patients with a history of BRE, between ages 12 and 28 years (mean 17 years). Twenty-one of these patients had experienced their last rolandic seizure before the age of 10 years and 9 of them had been without treatment since age 11 or earlier. In 2 of these 9 patients, other types of seizures recurred after remission of BRE. Clinical, EEG, and evoked potential findings on these 2 patients are presented. RESULTS: After having experienced BRE, both patients suffered partial seizures from age 12, with elementary visual hallucinations, visual blurring, slow head turning, cephalic pain, epigastric discomfort, unresponsiveness, and vomiting. Seizure onset was related to watching TV or exposure to bright light. EEG showed interictal occipital spikes, and a photoparoxysmal response limited to the occipital lobes. Visual evoked potentials were greatly increased in amplitude. One patient had two visual attacks only and remained seizure free after 4 years of follow-up, while the other had seizures controlled by an association of valproate and carbamazepine. CONCLUSIONS: Clinical and neurophysiological characteristics suggest that these two patients may have presented different age-related expressions within the spectrum of a benign seizure susceptibility syndrome rather than sharply distinct epilepsy syndromes.

Adolescent↗
PubMed2001

[Cognitive consequences of Rolandic Epilepsy].

Idiopathic focal epilepsies and particularly rolandic epilepsy are considered to have an excellent outcome with spontaneous recovery during adolescence. The characterization of this syndrome in 1959 by Beaussart and Nayrac was a great progress for childhood epileptology: the existence of a focal epilepsy with a good prognosis and no underlying brain lesion, so-called "benign" epilepsy, was recognized. Since the first descriptions, numerous neuropsychological studies were performed showing variable results. All the studies agree with the fact that children with rolandic epilepsy keep a normal global intellectual efficiency and a good long-term outcome. Nevertheless, some children may suffer transiently during the active phase of the epilepsy from oromotor dysfunction, neuropsychological deficits, or attention deficits with learning disorders. The analysis of cognitive and neurophysiological correlations evidenced a significant correlation between the epileptic focus localization and few specific dysfunctions. We evidenced mainly a significant effect of the persistence of a prolonged slow focus and a strong activation of night EEG spike and waves on cognitive decline and attention disorders. These abnormalities are mainly observed during severe or atypical evolutions of rolandic epilepsy. Preliminary longitudinal studies show that these cognitive deficits are transient. Thus, the presence of an active epileptic focus, without underlying brain lesion, could interfere with normal maturation of cognitive function.

Adolescent↗
PubMed2004

[Rolandic epilepsy: neurophysiological aspects].

Rolandic epilepsy (RE) is a common epileptic syndrome, which child neurologists often see. However, many problems concerning RE remains unsolved despite previous extensive studies. This paper discusses the current status of knowledge and our own observation especially on neurophysiological aspects of RE, such as the morphology of rolandic discharge (RD), effectiveness of clonazepam on RD, rhythmic slow activity, extreme somatosensory evoked potentials, and generator of RD. RD is characteristic of RE, and sometimes appears to be enigmatic to child neurologists. RE is a representative epileptic syndrome in childhood, and should be investigated further to address its underlying mechanism.

Anticonvulsants↗
PubMed2006

Longitudinal assessment of neuropsychologic and language function in children with benign rolandic epilepsy.

Previous studies of benign rolandic epilepsy have reported improvement in cognitive functioning over time. Their focus was the impact of paroxysmal electroencephalographic (EEG) activity on neuropsychologic function. Comprehensive longitudinal language assessment has not previously been undertaken. In a cross-sectional study, we demonstrated that some children with benign rolandic epilepsy have difficulties in verbal and visual memory and phonologic awareness. The current study evaluated a subgroup longitudinally to determine if difficulties improved. Twenty-eight patients underwent comprehensive longitudinal neuropsychologic and language assessments. The clinical features evaluated included seizure frequency, absolute age, medications, and a follow-up EEG. Differences in performance were analyzed using t-tests. Improvement in cognitive functioning, particularly in the areas of verbal memory, receptive language ability, and phonemic manipulation, was demonstrated. Visual memory and aspects of phonologic awareness showed no change. The improvements were not related to the clinical variables. It is important to recognize cognitive difficulties in children with benign rolandic epilepsy. Some difficulties can resolve; however, continued monitoring, particularly in areas of visual memory and phonologic awareness, is required.

Adolescent↗
PubMed2000

Autosomal dominant rolandic epilepsy with speech dyspraxia.

Autosomal Dominant Rolandic Epilepsy with Speech Dyspraxia (ADRESD) is a rare disorder which highlights the relationship between Benign Rolandic Epilepsy (BRE) and speech and language disorders. Subtle speech and language disorders have recently been well characterised in BRE. ADRESD is associated with long term, more severe speech and language difficulties. The time course of rolandic epilepsy in ADRESD is typical of that of BRE. ADRESD is inherited in an autosomal dominant manner with anticipation. It is postulated that the anticipation may be due to an, as yet unidentified, triplet repeat expansion in a gene for rolandic epilepsy. BRE follows complex inheritance but it is possible that ADRESD may hold some valuable clues to the pathogenesis of BRE.

Age of Onset↗
PubMed2004

Is benign rolandic epilepsy genetically determined?

Benign rolandic epilepsy (BRE) is considered to be a genetically determined idiopathic partial epilepsy. We studied twins with BRE and compared the concordance with a twin sample of idiopathic generalized epilepsy (IGE). All eight BRE pairs (six monozygous [MZ], two dizygous [DZ]) were discordant. MZ pairwise concordance was 0 (95% confidence interval [CI], 0-0.4) for BRE compared with 0.7 (95% CI, 0.5-0.9) for 26 IGE MZ pairs. Our data suggest that conventional genetic influences in BRE are considerably less than for IGE, and other mechanisms need to be explored.

Adolescent↗
PubMed1999

Benign rolandic epilepsy: neuropsychological findings.

Benign rolandic epilepsy (BRE) is a partial idiopathic epilepsy of childhood presenting with a nocturnal seizure and with a typical EEG showing centrotemporal spike and multifocal or generalized sharp slow waves. Although normal neurological and intellectual development are expected in BRE, it is not infrequent to detect subtle defects in neuropsychological functions and neuromotor development. This study included 20 cases of BRE diagnosed according to the criteria of ILAE. The patients underwent several tests of neuropsychological functions as well as detailed neurological examination and the results were compared statistically to normal controls. In the patient group, a family history of language delay or learning disability (P < 0.005), presence of consanguinity (P < 0. 05), dyspraxia in the lower extremities (to imitation) (P < 0.05), difficulties in go-no-go test (P < 0.001), as well as some problems related to language such as dysprosody (P = 0.05), minor motor deficits in the left (P < 0.05) and right upper extremity (P < 0.05) were significantly more frequent compared to the control group. One should be rather guarded against the prognosis in BRE with respect to the higher cortical functions and neurodevelopmental problems.

Adolescent↗
PubMed2002

Typical and atypical rolandic epilepsy in childhood: a follow-up study.

Atypical features of rolandic epilepsy are not uncommon, although the long-term prognosis of this condition is not known. Eighty-five children (50 male and 35 female) attending the Department of Pediatrics of the University of Chieti, the Department of Pediatrics of Brindisi Hospital, and the Department of Neurology of San Valentino Hospital were selected for the study; these patients were subdivided into two groups according to their clinical presentation. Group A consisted of children who suffered from typical rolandic epilepsy and Group B consisted of children with atypical features of rolandic epilepsy. All patients of both groups were re-evaluated after at least 8 years from the first evaluation, and the frequency of seizures and the response to treatment were similar in the two groups of children. In spite of this fact, in patients who suffered from atypical rolandic epilepsy, we found a significantly higher percentage of learning and behavioral disabilities than in children affected by the classical form of rolandic epilepsy (45.5% vs 7.8%; P < 0.0001). In conclusion, atypical rolandic epilepsy seems to be associated with a high percentage of learning and behavioral disorders.

Anticonvulsants↗
PubMed2006

Coexistence of childhood absence and rolandic epilepsy.

The coexistence of absence and rolandic epilepsy is extremely rare. This coexistence has been attributed to either the treatment or an atypical course of the disease. However, for some authors, this issue remains controversial. We report the case of a 9-year-old girl presenting with both absence seizures and focal seizures consistent with rolandic epilepsy.

Anticonvulsants↗
PubMed1999

Cognitive and behavioral effects of carbamazepine in children: data from benign rolandic epilepsy.

The effects of antiepileptic drugs on cognition are difficult to delineate, yet of critical importance for children with epilepsy. We investigated the cognitive and behavioral effects of carbamazepine in children with benign rolandic epilepsy. Ten subjects with benign rolandic epilepsy were evaluated with and without carbamazepine treatment. Fourteen unmedicated subjects with migraine headache evaluated twice served as a control group. Subjects were 6 to 12 years of age, fluent in English, and not mentally retarded. We found that children with benign rolandic epilepsy were quicker on a visual-search task and recalled stories better when not treated than when treated with carbamazepine. After correction for multiple comparisons only the memory finding remained significant. Higher carbamazepine serum level was associated with slower performance on the same visual-search task. This latter finding did not meet multiple comparison criteria. Numerous significant practice effects were found within the control group. Comparisons with reliable change indices identified two subjects with benign rolandic epilepsy with particularly poor scores while receiving carbamazepine. These findings suggest some effects on memory from carbamazepine; however, they do not support meaningful dosage-related effects, within the recommended range. Significant practice effects confirmed the need to control for such effects when evaluating treatments. Finally, identification of two subjects who performed more poorly while on carbamazepine suggests that some children might experience particular difficulties while receiving this medication and highlights the need to investigate individual subject responses to treatment.

Analysis of Variance↗
PubMed2003

Children with Rolandic spikes and ictal vomiting: Rolandic epilepsy or Panayiotopoulos syndrome?

Centrotemporal spikes are the EEG marker of Rolandic epilepsy, while ictus emeticus is one of the main seizure manifestations of Panayiotopoulos syndrome. Ictus emeticus has not been reported in Rolandic epilepsy. Out of a population of 1340 children with focal afebrile seizures we studied 24 children who had emetic manifestations in at least one seizure and centrotemporal spikes in at least one EEG. They were of normal neurological status and had a follow-up of at least two years after the last seizure. All children had sleep EEG following sleep deprivation. Two groups of patients were identified. Group A (12 patients) with EEG centrotemporal spikes only and group B (12 patients) with centrotemporal spikes and spikes in other locations. In 21 patients, ictal emetic manifestations culminated in vomiting and in three only nausea or retching occurred. The commonest presentation was ictus emeticus at onset followed by deviation of the eyes or staring, loss of contact and floppiness. In 79%, seizures occurred during sleep. Autonomic status epilepticus occurred in 37.5%. The mean age at onset was 5.3 years. Overall analysis of the clinical and EEG data points out that the vast majority of these patients primarily suffer from Panayiotopoulos syndrome. Twenty patients (83%) had ictal semiology typical of Panayiotopoulos syndrome, but five also had concurrent Rolandic symptoms and four later developed pure Rolandic seizures. The other four patients (17%) had typical Rolandic seizures with concurrent ictus emeticus. These findings suggest a link between Rolandic epilepsy and Panayiotopoulos syndrome, the two most important phenotypes of the benign childhood seizure susceptibility syndrome.

Adolescent↗
PubMedDate not supplied

[Rolandic epilepsy. An analysis of the clinical and electrophysiological characteristics, treatment and prognosis in 87 patients].

INTRODUCTION: Rolandic epilepsy is the most common epileptic syndrome in infancy. It usually has a favourable prognosis and seizures disappear during the teenage years. AIMS: The aim of this study was to describe a sample of children diagnosed with rolandic epilepsy in clinical, electrophysiological and imaging terms. We also intend to compare the results obtained with those described in the literature. PATIENTS AND METHODS: We conducted a retrospective, descriptive study of the children diagnosed with rolandic epilepsy who had been submitted to a follow-up at the Neuropaediatrics Unit at the HGSA since 1989. Children who were less than 2 years old when they suffered their first seizures or who had abnormal neurological/imaging examinations were excluded. Two groups were defined (typical and atypical), several variables were characterised and these were then analysed statistically. RESULTS: A total of 87 children (51 males) were included in this study, their mean age being 13.6 years. The population was divided into two groups: A (typical cases; n = 69) and B (atypical cases; n = 18). The mean age at the onset of seizures was 6.2 and 6 years for groups A and B, respectively. The predominant type of seizures was simple partial for group A and complex partial for group B; they were mainly sporadic and nocturnal in both groups. Medication was administered to 51 (73.9%) of the children in group A, with a good response in 78.4% of them; 13 members of group B (72.2%) received medication, with a positive response in 76.9% of them. CONCLUSIONS: Rolandic epilepsy has a broad clinical spectrum and usually courses with normal psychomotor development, but may coexist with learning disabilities. In this study, no significant differences were found between the typical and atypical forms of presentation, as far as their progress and response to treatment are concerned.

Adolescent↗
PubMed2006

Analyzing the etiology of benign rolandic epilepsy: a multicenter twin collaboration.

PURPOSE: Benign rolandic epilepsy (BRE) is considered a genetically determined idiopathic partial epilepsy. We analyzed a large sample of twins from four international twin registers to probe the genetics of BRE. We also aim to synthesize the apparently conflicting family and twin data into a model of BRE etiology. METHODS: Large population-based twin registries of epilepsies from Odense (Denmark), Richmond, Virginia (United States), and Oslo (Norway) were reviewed for BRE cases and added to our Australian twin data. Diagnosis of classic BRE was based on electroclinical criteria with normal neurologic development. Cases with a compatible electroclinical picture but abnormal neurologic development were termed non-classic BRE. RESULTS: Eighteen twin pairs were identified (10 monozygous; eight dizygous) of whom at least one twin was diagnosed with classic BRE among a total sample of 1,952 twin pairs validated for seizures, and all were discordant for BRE. The estimated monozygous pairwise concordance for BRE in this sample was 0.0 [95% confidence interval (CI), 0.0-0.3). Four twin pairs (one monozygous, three dizygous) had non-classic BRE, and all co-twins had seizures. CONCLUSIONS: The twin data showing an absence of any concordant twin pairs with classic BRE suggest that noninherited factors are of major importance in BRE. Modelling the data shows that the familial occurrence of centrotemporal spikes makes only a minor contribution to the familial aggregation of BRE. Genetic factors are probably more important in non-classic BRE. The etiology and mode(s) of inheritance of BRE are much more complicated than initially conceptualized.

Adult↗