Tactile‐evoked Rolandic Discharges: A Benign Finding?

PURPOSE: This retrospective study was undertaken to determine if patients having tactile-evoked rolandic discharges were a more "benign" patient population than those with spontaneously occurring nontactile rolandic discharges and to determine whether the presence of tactile-evoked rolandic discharges was a marker for the future development of epilepsy, as previously reported. METHODS: During this 8-year study, 304 patients were seen with rolandic discharges. These patients all had tactile stimulation of their hands and/or feet. They formed two groups: patients with spontaneous rolandic discharges that could not be evoked by tactile stimulation (NT) and patients with spontaneous rolandic discharges that could be enhanced with tactile stimulation (TE). Over a 14-month period, every patient had tactile stimulation of both hands and both feet, resulting in a third group of patients having rolandic discharges seen only with tapping (TO). RESULTS: Tactile-enhanced discharges constituted 38.2% of all rolandic discharges. Patients with TE and TO discharges had a higher incidence of normal development and intelligence, normal neurologic examinations, and a lower incidence of seizures and focal or generalized background abnormalities on their EEGs. Only one patient with normal background and no coexisting epileptiform abnormalities had partial motor seizures with corresponding contralateral central discharges. Only two patients who had no seizures at the time of their first EEG subsequently went on to develop seizures. Neither fit the pattern of the seizure disorder described in the literature. CONCLUSIONS: It is hypothesized that tactile-evoked rolandic discharges are a benign, age-related phenomenon, which do not represent a marker for the future development of epilepsy and are not the interictal electrographic correlate to an already existing seizure disorder.     

Acute anterior bi-opercular syndrome of critical origin in epilepsy with rolandic spikes]

We report the fifth case of a palsy of the lips, the tongue and the pharynx corresponding to an acute pseudo-bulbar syndrome causing speech arrest, and hyper-sialorrhea. The clinical examination and the electroencephalograms showed a partial motor status with spikes discharges in the two central regions, in a 10-year old boy known to have epilepsy with rolandic spikes. The status epilepticus ceased with phenytoin therapy. Although epilepsy with rolandic spikes is a benign one without any cerebral lesion, a partial motor status epilepticus is possible and does not change prognosis.     

Increased frequency of rolandic spikes in ADHD children

PURPOSE: Some children with rolandic epilepsy have associated neuropsychiatric deficits resembling symptoms of attention deficit-hyperactivity disorder (ADHD), the most common neurobehavioral disorder of childhood. The clinical overlap between both syndromes has received relatively little attention. The study examines the frequency of rolandic spikes in nonepileptic children with ADHD and compares it with a historic control group of 3,726 normal school-aged children. ADHD patients with and without discharges are compared regarding age at admission, sex, global functioning, and distribution of ADHD subtypes. METHODS: The EEGs of 483 ADHD outpatients between 2 and 16 years meeting diagnostic criteria for ADHD according to Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) were evaluated prospectively. If rolandic spikes were present, separate sleep EEGs were performed to exclude a bioelectrical status epilepticus during slow-wave sleep. Results: Rolandic spikes were detected in the EEGs of 27 children (5.6%; 22 boys and five girls). Seizure rate during follow-up tended to be larger in children with rolandic spikes. No significant differences were found between ADHD patients with and without spikes regarding sex and global functioning. ADHD children with rolandic spikes came to our attention significantly earlier than did children without discharges and tended to exhibit more hyperactive-impulsive symptoms, evidenced in a larger proportion of the diagnosis of ADHD combined type than ADHD inattentive type. CONCLUSIONS: The frequency of rolandic spikes in children with ADHD is significantly higher than expected from epidemiologic studies. The question arises how ADHD symptoms are related to rolandic spikes in this ADHD subgroup. Possibly rolandic discharges or underlying, not fully understood mechanisms of epileptogenesis decrease the vulnerability threshold, advance the onset, or aggravate the course of ADHD.     

Magnetoencephalographic analysis of rolandic discharges in a patient with rolandic epilepsy associated with oromotor deficits

The purpose of this study was to clarify the neurophysiologic basis of oromotor deficits in a patient with atypical rolandic epilepsy. We investigated magnetoencephalographic analysis of rolandic discharges with right predominance before and during clonazepam therapy. Before clonazepam administration, current sources of rolandic discharges were broadly distributed in the secondary sensory cortex, superior temporal gyrus, and parietal association area in addition to hand and orofacial division of the primary somatosensory cortex. During clonazepam therapy, oromotor deficits were improved, along with a decrease in rolandic discharge, and current sources of residual right-sided rolandic discharges were shifted to the right superior parietal lobule. Taking the clinical course and magnetoencephalographic findings together, the distributed rolandic discharge focus might be closely related to oromotor deficits, and clonazepam was effective for the disorder.     

Focal cortical myoclonus and rolandic cortical dysplasia: clarification by magnetic resonance imaging

Focal cortical myoclonus is rare. Obvious causes include tumor or atrophy involving the motor strip, but in some cases no cause is apparent. We present 4 patients who started to have focal myoclonus in childhood. All had focal motor seizures as well, and one had recurrent focal motor status epilepticus. All 4 had a mild progressive hemiparesis. Electrographic investigations showed focal epileptic discharges in the contralateral rolandic areas. Radiological studies were unrevealing, but magnetic resonance showed rolandic lesions in 3 patients. At surgery, abnormally wide gyri were found in the distribution demonstrated by magnetic resonance. The pathological substrate was focal cortical dysplasia. All patients have improved considerably following surgery. These findings suggest that focal myoclonus may be due to a rolandic neuronal migration disorder. Visualization of these lesions by magnetic resonance permits development of a surgical strategy leading to optimal treatment of these medically intractable epileptic disorders.     

Magnetoencephalographic analysis of roland discharges in benign childhood epilepsy

We report the detailed analysis of the generator and propagation of rolandic discharges in benign childhood epilepsy with centrotemporal spikes by means of 37-channel magnetoencephalography with neuromagnetic three-dimensional dipole localization. Equivalent current dipoles of prominent negative sharp waves of rolandic discharges appeared as tangential dipoles in the rolandic region, positive poles being situated anteriorly. These equivalent current dipoles showed a relatively limited localization and regular directions compared with other components. Equivalent current dipoles of preceding small positive waves, positive waves following negative sharp waves, and negative slow waves appeared in the vicinity of negative sharp waves. Equivalent current dipoles of rolandic discharges were located around the generator of somatosensory evoked magnetic fields stimulated at the lower lip. These findings suggest that rolandic discharges are generated through basically a mechanism similar to that for the middle-latency components of somatosensory evoked responses.     

Clinical Relevance of a Dipole Field in Rolandic Spikes

The clinical presentation of 366 children with rolandic spikes was examined to determine whether the presence of a temporal-frontal dipole field is associated with a lower incidence of clinical abnormality. Comparisons were made between the clinical presentation of 99 children with temporal-frontal dipole discharges versus 267 children with nondipole rolandic discharges. Criteria examined were birth history, developmental milestones, school history, total number of seizures, neurological examination, and computed tomography (CT) findings. For all clinical parameters, except birth history and CT finding, there was a lower incidence of clinical abnormality in the group with dipole discharges (p less than 0.001). The clinical profile seen with temporal-frontal dipole discharges was very different than with nondipole rolandic spikes. Children with dipole discharges less often presented with frequent seizures (10%), developmental delay (18%), school difficulties (34%), or abnormal neurological exam (22%). In contrast, children with nondipole rolandic discharges often presented with a history of frequent seizures (55%), developmental delay (55%), school difficulties (60%), and an abnormal neurological exam (63%). The incidence of clinical abnormalities in the nondipole group exceeded that found in our control population in all areas. Temporal-frontal dipole discharges are associated with a lower incidence of clinical abnormality than are nondipole rolandic spikes. These discharges may represent a benign functional focus.     

A comparison between averaged spikes and individual visually-analyzed spikes in rolandic epileptiform discharges

PURPOSE:This study compared some morphological features of individual rolandic epileptiform discharges, used to obtain an averaged estimate, with those of the resulting estimate. METHOD: Twenty-four averaged discharges from EEGs of 24 children showing rolandic spikes were compared with 480 individual discharges used in the averaging. The analysis was based on the occurrence of tangential dipole and "double spike" patterns. RESULTS: In 15 averaged discharges the tangential dipole pattern was found. Individual spikes used in the averaging process displayed the same pattern in 35-100% of them; in the remaining 9 averaged discharges, up to 20% of the individual spikes showed the same pattern, although this was not found in the averaged waveforms. "Double spike" pattern was found in 11 of the averaged waveforms and was recognized in 50-100% of its individual discharges, whereas up to 45% of individual spikes showed this pattern without expression in the averaged waveform. CONCLUSION: When visually analyzing an EEG with rolandic spikes, caution should be exercised in characterizing these patterns, since a few discharges showing them may not be expressed in the averaged waveform and the clinical correlations proposed for these patterns may not apply.     

Rolandic discharges: Clinico-neurophysiological correlation

Objective

The aim of this study was to analyze neurophysiologic aspects of rolandic discharges.

Methods

We reviewed 45 electroencephalograms of patients divided into two groups: those with benign childhood epilepsy with centrotemporal spikes (BCECTS) and symptomatic partial epilepsy (SPE), following ILAE criteria (1989). The EEG data analyzed were: horizontal dipole discharges, double spike phenomenon, the extension of epileptiform discharges and background activity.

Results

There was a predominance of horizontal dipole between patients with BCECTS compared with patients with SPE; however, this difference was not statistically significant. There was also no statistically significant difference between the two groups when the double spike phenomenon and the extension of discharges beyond the rolandic area were considered. The slower background activity in the SPE group was the only variable with statistical significance.

Conclusions

This study revealed similarities between rolandic discharges of two different epilepsy groups. The only reliable parameter to differentiate the groups was the background activity.

Significance

Our findings suggest that most EEG rolandic features are not pathognomonic of BCECTS, as they are related to the area of the discharges and not to the epileptic syndrome itself.     

Nocturnal epileptiform EEG discharges, nocturnal epileptic seizures, and language impairments in children: review of the literature

This review addresses the effect on language function of nocturnal epileptiform EEG discharges and nocturnal epileptic seizures in children. In clinical practice, language impairment is frequently reported in association with nocturnal epileptiform activity. Vice versa, nocturnal epileptiform EEG abnormalities are a common finding in children with specific language impairment. We suggest a spectrum that is characterized by nocturnal epileptiform activity and language impairment ranging from specific language impairment to rolandic epilepsy, nocturnal frontal lobe epilepsy, electrical status epilepticus of sleep, and Landau-Kleffner syndrome. In this spectrum, children with specific language impairment have the best outcome, and children with electrical status epilepticus of sleep or Landau-Kleffner syndrome, the worst. The exact nature of this relationship and the factors causing this spectrum are unknown. We suggest that nocturnal epileptiform EEG discharges and nocturnal epileptic seizures during development will cause or contribute to diseased neuronal networks involving language. The diseased neuronal networks are less efficient compared with normal neuronal networks. This disorganization may cause language impairments.     

Coincidence of rolandic and absence features: rare,but not impossible

The purpose of the study was to evaluate the incidence of a possible combination of rolandic and absence signs in epileptic children, which remains a subject of controversy. The medical files and electroencephalographic (EEG) records of children with rolandic, childhood absence, and juvenile absence epilepsy were retrospectively analyzed. During the antiepilepsy treatment, 6 of 66 patients with rolandic epilepsy, most of them with initial carbamazepine therapy, had absences and generalized spike-wave discharges of a secondarily generalized type. Five cases of 34 children with childhood absence epilepsy and 3 of 11 patients with juvenile absence epilepsy were identified with an EEG focus of the rolandic type. We considered the likely relation of absence features in rolandic epilepsy to the treatment or to an atypical course. The presence of a rolandic focus in absence epilepsies, however, makes the coincidence of these entirely distinct phenomena, even if very rare, not excluded. Further studies are required to elucidate a probable genetic or functional link between partial and primarily generalized EEG discharges in the idiopathic childhood epilepsies.     

Speech and language deterioration in benign rolandic epilepsy

A 5-year-old boy presented with typical clinical and electrophysiologic features of benign rolandic epilepsy. His neurodevelopment, language, and behavior prior to the onset of epilepsy were appropriately normal. He demonstrated marked deterioration of language and cognitive function during the course to a mild and then a moderate disability range. Serial sleep electroencephalographic recordings initially showed continuous and bilateral rolandic discharges with evolution to localized left rolandic spikes. Language and cognitive improvements were subsequently seen. Educational support and evolution of the electroencephalogram to a localized focus could have been contributory. It is anticipated, however, that he will have significant long-term problems in complex language.     

Benign rolandic epilepsy -- perhaps not so benign: use of magnetic source imaging as a predictor of outcome

The purpose of this study was to evaluate children with benign rolandic epilepsy, a childhood epilepsy characterized by centrotemporal/rolandic spike-wave discharges with infrequent partial seizures that may secondarily generalize. Recently, some investigators have questioned whether benign rolandic epilepsy is indeed "benign" or whether long-term cognitive outcome may be adversely affected. We initiated an ongoing study to identify children with benign rolandic epilepsy. The children were evaluated in the Texas Comprehensive Epilepsy Program using outpatient or continuous video-electroencephalographic monitoring, brain magnetic resonance imaging, magnetoencephalography, and neuropsychological testing. Neuropsychological testing revealed fine motor dysfunction, visuomotor integration deficits, dyscalculia, and/or expressive language deficits in all of the 9 patients evaluated, reaffirming that benign rolandic epilepsy is not necessarily a benign disorder. Our study shows a high concordance of motor and cognitive deficits in benign rolandic epilepsy, as others have previously suggested. Furthermore, magnetic source imaging shows a higher resolution of dipole localization compared with conventional electroencephalography, which may ultimately improve prediction of deficits. This reaffirms that magnetoencephalography is a valuable diagnostic tool in the evaluation of children with benign rolandic epilepsy.     

Magnetoencephalographic study of giant somatosensory evoked responses in patients with rolandic epilepsy

We report five patients with rolandic epilepsy associated with giant somatosensory responses to median nerve stimulation, in whom we analyzed the pathophysiologic relationship between rolandic discharges and the somatosensory responses using magnetoencephalography. Four of the five patients showed giant P30m, the current source of which was localized in the primary somatosensory cortex, while the first cortical response, N20m, was not enhanced, except in one patient. The current source of the giant middle-latency component, N70m, was localized posterior to that of N20m, possibly in the posterior parietal cortex, in all patients. The initial positive peak and large negative peak of rolandic discharges were identical to P30m and N70m with respect to the current source localization, wave form, topographic pattern, and time relationship in the electroencephalogram and magnetoencephalogram, and somatosensory evoked magnetic field and somatosensory evoked potential records, respectively. In addition, the secondary sensory cortex was considered to be the generator of the middle-latency component in one patient. In one patient, the current intensity of the N70m was normalized along with clinical improvement and the disappearance of rolandic discharges, whereas those of other somatosensory evoked magnetic field components remained unchanged. Our data suggest that the rolandic discharge generator mechanism in these patients could be closely related to the developmental alteration of excitability in the primary somatosensory cortex, posterior parietal cortex, and secondary somatosensory cortex, which decreased with age, and it could share a common neuronal pathway, at least in part, with the giant P30m-N70m (N90m) in the somatosensory evoked magnetic field through the sequential and parallel processing of somatosensory information.     

Nocturnal epileptiform EEG discharges,nocturnal epileptic seizures,and language impairments in children: Review of the literature

This review addresses the effect on language function of nocturnal epileptiform EEG discharges and nocturnal epileptic seizures in children. In clinical practice, language impairment is frequently reported in association with nocturnal epileptiform activity. Vice versa, nocturnal epileptiform EEG abnormalities are a common finding in children with specific language impairment. We suggest a spectrum that is characterized by nocturnal epileptiform activity and language impairment ranging from specific language impairment to rolandic epilepsy, nocturnal frontal lobe epilepsy, electrical status epilepticus of sleep, and Landau–Kleffner syndrome. In this spectrum, children with specific language impairment have the best outcome, and children with electrical status epilepticus of sleep or Landau–Kleffner syndrome, the worst. The exact nature of this relationship and the factors causing this spectrum are unknown. We suggest that nocturnal epileptiform EEG discharges and nocturnal epileptic seizures during development will cause or contribute to diseased neuronal networks involving language. The diseased neuronal networks are less efficient compared with normal neuronal networks. This disorganization may cause language impairments.     

Are there generalised spike waves and typical absences in benign rolandic epilepsy?

Generalised 3 Hz spike wave (SW) discharges with or without absences have been described in children with benign epilepsy with centrotemporal spikes (BECTS), leading to speculations about a continuum between childhood absence epilepsy (CAE) and BECTS. We thus decided to evaluate the prevalence of absence seizures (AS) and generalised 3 Hz SW in patients with BECTS. All patients with BECTS first referred since 1986 have been identified prospectively. Their medical and electroencephalograph (EEG) records were analysed retrospectively, in the search for AS and generalised SW discharges. Over a period of 11 years, we found typical rolandic spikes in 66 newly referred patients; 64 had seizures typical for the condition (18 female, 46 male), two were asymptomatic and were not further analysed. All had routine waking EEG recordings, and 49 children (76%) had at least one sleep EEG. AS or classical generalised 3 Hz SW were never recorded from history or EEG data, respectively. However, 17 patients had some diffuse SW discharges, lasting 1-5 s, which appeared as grossly symmetrical in only seven children, with a clearly asymmetrical aspect in the others. Among these seven patients, the discharges were only seen on awakening in one, both during waking and nREM sleep stage I or II in one and only during nREM sleep stage I or II in five. They were apparently subclinical in all. We thus found neither AS nor classical 3 Hz SW discharges among 64 consecutive patients with BECTS. Brief bursts of bilateral abnormalities occur in about 25% of the cases, mostly with sleepiness. Such findings do not substantiate the existence of a continuum between CAE and BECTS.     

Autosomal dominant rolandic epilepsy and speech dyspraxia: A new syndrome with anticipation

We describe a family of 9 affected individuals in three generations with nocturnal oro-facio-brachial partial seizures, secondarily generalized partial seizures, and centro-temporal epileptiform discharges, associated with oral and speech dyspraxia and cognitive impairment. The speech disorder was prominent, but differed from that of Landau-Kleffner syndrome and of epilepsy with continuous spike and wave during slow-wave sleep. The electroclinical features of this new syndrome of autosomal dominant rolandic epilepsy resemble those of benign rolandic epilepsy, a common inherited epilepsy of childhood. This family shows clinical anticipation of the seizure disorder, the oral and speech dyspraxia, and cognitive dysfunction, suggesting that the genetic mechanism could be expansion of an unstable triplet repeat. Molecular studies on this syndrome, where the inheritance pattern is clear, could also be relevant to identifying a gene for benign rolandic epilepsy where anticipation does not occur and the mode of inheritance is uncertain.     

Can absence status epilepticus be of frontal lobe origin?

Five women with an unclassifiable nonconvulsive status epilepticus (NCSE) characterized by young age at onset, prolonged confusions, focal motor seizures, and both generalized spike-and-wave discharges and focal epileptic discharges on the EEG were studied with video-EEG monitoring. Electrographically, the NCSE originated from the left frontal lobe in 4 patients, and the left hemisphere with multifocal seizure discharges in 1 patient. Focal motor seizures seemed to originate from the left hemisphere in all 5 patients, particularly from its anterior part in 3 of them. Results show that the NCSE is complex partial status epilepticus of frontal lobe origin electroclinically mimicking absence status epilepticus once it reaches a full-blown phase.     

Evidence of shared genetic risk factors for migraine and rolandic epilepsy

Purpose:   Evidence for a specific association between migraine and rolandic epilepsy (RE) has been conflicting. Children with migraine frequently have electroencephalographic (EEG) abnormalities, including rolandic discharges, and approximately 50% of siblings of patients with RE exhibit rolandic discharges. We assessed migraine risk in RE probands and their siblings.
Methods:   We used cohort and reconstructed cohort designs to respectively assess the relative risk of migraine in 72 children with RE and their 88 siblings using International Classification of Headache Disorders (ICHD-2) criteria. Incidences were compared in 150 age and geographically matched nonepilepsy probands and their 188 siblings. We used a Cox proportional hazards model, using age as the time base, adjusting hazard ratios (HRs) for sex in the proband analysis, and for sex and proband migraine status in the sibling analysis.
Results:   Prevalence of migraine in RE probands was 15% versus 7% in nonepilepsy probands, and in siblings of RE probands prevalence was 14% versus 4% in nonepilepsy siblings. The sex-adjusted HR of migraine for an RE proband was 2.46 [95% confidence interval (CI) 1.06–5.70]. The adjusted HR of having ≥1 sibling with migraine in an RE family was 3.35 (95% CI 1.20–9.33), whereas the HR of any one sibling of a RE proband was 2.86 (95% CI 1.10–7.43).
Discussion:   Migraine is strongly comorbid in RE and independently clusters in their siblings. These results suggest shared susceptibility to migraine and RE that is not directly mediated by epileptic seizures. Susceptibility gene variants for RE may be tested as risk factors for migraine.     

Prolonged Intermittent Drooling and Oromotor Dyspraxia in Benign Childhood Epilepsy with Centrotemporal Spikes

Prolonged isolated sialorrhea of epileptic origin was described by Penfield and Jasper (1954) in a patient with a lesional epilepsy. A child with prolonged but intermittent drooling, lingual dyspraxia, and other clinical and electroencephalographic (EEG) features compatible with benign childhood epilepsy with centrotemporal spikes (BCECS) is described. The fluctuant course of the symptomatology and correlation with the intensity of the paroxysmal discharges on EEG are consistent with an epileptic dysfunction located in the lower rolandic fissure. No lesion was demonstrated by magnetic resonance imaging (MRI). Our case bears analogies with the recently reported status epilepticus of BCECS and the "acquired aphasia-epilepsy syndrome."