Partial Epilepsy Of Childhood Research Articles

Benign focal epilepsies of childhood.

Until relatively recently, genetic influences in partial seizures were thought to be of minimal importance. However, with further identification of childhood benign partial seizures it is becoming clear that inheritance plays a major role in the pathogenesis of these seizures. Diagnostic criteria proposed for benign partial seizures include absence of neurologic or intellectual deficits, family history of epilepsy, onset of seizures after age 2 years, stereotyped brief seizures, frequent nocturnal occurrence, spontaneous remission in adolescence, and electroencephalograms (EEGs) demonstrating spikes with a distinctive morphology and localization superimposed on normal background activity. The two most commonly described benign partial epilepsies of childhood are benign Rolandic epilepsy (BRE) and benign occipital epilepsy (BOE). Both disorders begin in childhood, are associated with characteristic EEG patterns, have seizures that are easily controlled with medication, often are familial, and have an excellent prognosis. The other benign partial seizure disorders in children that have been described are not as well studied as BOE and BRE, and the role of inheritance pattern, if any, is less clear.

Long‐Term Follow‐Up of EEG Foci in Childhood Partial Epilepsy

Long‐Term Follow‐Up of EEG Foci in Childhood Partial Epilepsy

Speech and Oromotor Deficits of Epileptic Origin in Benign Partial Epilepsy of Childhood with Rolandic Spikes (BPERS)

The authors report three children who suffered temporary oromotor or speech disturbances as focal epileptic manifestations within the frame of benign partial epilepsy of childhood with rolandic spikes and review similar cases described in the literature. The deficit can occur as an initial symptom of the disorder without visible epileptic seizures and interferes in a variable way with simple voluntary oromotor functions or complex movements including speech production, depending on the exact location and spread of the discharging epileptic focus around the perisylvian region. The most severe deficit produces the anterior operculum syndrome. More subtle non-linguistic deficits such as intermittent drooling, oromotor apraxia or dysfluency, as well as linguistic ones involving phonologic production, can occur. The rapidity of onset, progression and recovery of the deficit is very variable as well as its duration and presumably reflects the degree of epileptic activity. In some cases, rapid improvement with antiepileptic medication occurs and coincidence between the paroxysmal EEG activity (which is usually bilateral) and the functional deficit is seen. The clinical and EEG profile of the seizures disorder and the dynamic of the deficit in these cases bear a strong resemblance to what is seen in the acquired epilepsy-aphasia syndrome (Landau and Kleffner). The variations in clinical symptoms appear more related to the main site, local extension and bilaterality of the epileptic foci rather than a basic difference in physiopathology.

A Study of Unilateral Brief Focal Atonia in Childhood Partial Epilepsy

We studied unilateral brief focal atonia (BFA) in seven patients with childhood partial epilepsy. BFA was observed as a transient dropping of one arm lasting from 100 to 150 ms when patients were asked to keep both arms outstretched in front of the body. Close examination using simultaneous video-polygraphic recordings showed dropping of the arm to correspond exactly with a single sharp and slow wave complex arising from the contralateral centrotemporoparietal region. The BFA occasionally would progress to atonic seizures or atonic absence seizures, when the localized epileptic discharge evolved into generalized discharges. In one patient we found a positive correlation between the intensity of BFA and the amplitude of the contralateral epileptic discharges. A higher amplitude corresponded to more pronounced BFA and a lower amplitude to less pronounced BFA. These results led us to conclude that the apparently interictal single sharp and slow wave complex in the rolandic region may inhibit contralateral motor control, thus producing BFA that corresponds with the spike amplitude.

Benign Partial Epilepsy of Childhood with Monomorphic Sharp Waves in Centrotemporal and Other Locations

We reviewed EEGs from children whose history and clinical course was compatible with benign partial epilepsy of childhood with centrotemporal spikes. In 21% of patients with a single EEG focus, the discharge was outside the centrotemporal area. In 37.5% of patients with more than one focus, one was in the centrotemporal area while the other was not. We suggest that the typical EEG features of this syndrome are the normal background, the stereotypic morphology of the sharp waves, and their activation by drowsiness and sleep, not their exclusive location in the centrotemporal regions. Insistence on a centrotemporal location for the EEG discharges in this syndrome may lead to a misclassification of the type of epilepsy in some children with implications for therapeutic decisions and prognostic statements.

Epilepsia rolândica relato de 53 casos

The clinical and EEG features of 53 out-patients with benign partial epilepsy of childhood with rolandic spikes were studied. The age mean (years) of seizures onset was 5.5 +/- 3.2. Simple partial seizures with speech arrest were more frequent than other seizure types. In seven cases (13.2%) cognitive disabilities were present. In the left-side foci a expected correlation between the interictal EEG focus and clinical lateralization of seizure was observed; in the right-side foci, the right-side interictal focus was correlated with ipsilateral seizures.

Neuropsychological features of benign partial epilepsy in children

Rolandic paroxysmal epilepsy (RPE) is a useful model for investigating the complex links between epilepsy and cognitive dysfunction. 44 children with RPE who met the following (among other) criteria: negative CT scan, freedom from drug treatment, and IQ greater than or equal to 80, were assigned to three subgroups by side of EEG focus: left, right and bilateral. A neuropsychological battery elicited small differences in cognitive performance between the whole group and the controls and among the subgroups, only partially correlated with EEG side. A follow-up assessment showed that the short falls had disappeared along with the seizures and EEG anomalies, thus confirming the benign nature of RPE. Our findings suggest too that the mere presence of paroxysmal cortical activity is enough to trigger cognitive dysfunction.

Partial epilepsy in childhood: Clinical EEG and CT study of 250 cases

Partial epilepsy in childhood: Clinical EEG and CT study of 250 cases

Prognosis of partial epilepsy.

The prognosis of partial epilepsy in childhood (excluding cases of benign partial epilepsy) was studied; the average follow up period was 7.4 years. Improvement rate of seizure status was 82.3%. We studied favourable prognostic factors and found that those most often associated with seizure improvement were familial convulsions and idiopathic forms, no generalised seizures before partial onset, low frequency of seizures after 12 months of treatment, short duration of epilepsy, and no background activity abnormalities on electroencephalography. We also observed such factors as mental retardation, neurological abnormalities, and behaviour and cognitive disorders. Factors that determined the prognosis for social adjustment were similar to those for seizure improvement. We discuss the favourable prognosis of partial seizures in childhood and the predictive factors.

Etude de l'EEG de sieste dans les epilepsies partielles idiopathiques et symptomatiques de l'enfant

A nap sleep EEG with at least one full sleep cycle has been recorded in 3 groups of children free from diffuse encephalopathy and presenting with partial epilepsy with onset after the age of 3 years: 15 cases with typical benign epilepsy with centrorolandic spikes (BERS), 15 cases with partial symptomatic epilepsy without clinical or radiological evidence of a cerebral lesion, and 12 cases with partial epilepsy symptomatic of a proven cerebral lesion. The time course of paroxysmal abnormalities was quantified according to individual sleep stages; the number of foci was also quantified in the waking vs sleeping state. Paroxysmal abnormalities are significantly enhanced by sleep in all 3 groups: throughout sleep stages in BERS, in slow sleep stages only in the other groups. The enhancement is more striking in BERS, where a clear decrease of abnormalities is also found upon awakening. There is no major difference between the 2 symptomatic groups, with a greater enhancement of paroxysms in the proven lesional group. Contrary to the other groups, only the group including those patients with a documented cerebral lesion showed a significant increase in the number of foci during sleep: numerous foci were found in these patients that were distinct from the primary lesional focus. Under the conditions of our study, the nap EEG seems to provide an accurate evaluation of sleep-induced changes of paroxysmal activity in partial epilepsies of childhood.

The partial epilepsies of childhood.

The partial epilepsies of childhood.

Phenacemide therapy of complex partial epilepsy in children: determination of plasma drug concentrations.

We used monotherapy with phenacemide to treat complex partial seizures in 13 children who were refractory to conventional antiepileptic drug therapy. Twelve patients responded with a reduction in seizure frequency, and 5 have been totally seizure free since the start of therapy. Phenacemide therapy was well tolerated with a minimum of untoward side effects and no evidence of irreversible drug toxicity. We developed a rapid and sensitive assay for the determination of plasma phenacemide concentrations by high performance liquid chromatography to monitor drug levels during therapy. Seizure control was achieved at plasma drug levels that ranged from 16 to 75 micrograms/ml. The median effective dose in our series was 52 micrograms/ml. The recurrence of seizures in three patients was, in each case, associated with trough plasma phenacemide levels below 50 micrograms/dl.

Combined myoclonic-astatic and "benign" focal epilepsy of childhood ("atypical benign partial epilepsy of childhood"). A separate syndrome?

The authors have followed six children with atypical epilepsies but a favorable evolution, consisting in minor motor seizures of the myoclonic-astatic type (with diffuse slow spike-waves on the electroencephalogram) together with clinical and EEG features seen in benign focal epilepsy of childhood (BFEC), an association recently reported by Aicardi and Chevrie (1982). The maintenance of a normal neurological function despite severe epilepsy, the absence of tonic seizures and the marked activation of the spike discharges during sleep were described by these authors as important characteristics suggesting a good prognosis. The purpose of this study was to see if these children indeed represent a particular subgroup of idiopathic epilepsy and to draw attention to a special clinical and EEG combination indicating a possibly favorable ultimate outcome, in children usually diagnosed as suffering from Lennox-Gastaut syndrome. The six cases closely resembled the group described by Aicardi and Chevrie (1982), although the clinical and EEG features of BFEC were not as striking as in their cases. Also transient mental deterioration occurred during the active seizure periods. The therapeutic benefit of the various drugs tried was difficult to assess, but the behavior was often perturbed by medication. Although it is not possible to decide at the present time if these cases represent a particular epileptic syndrome, the special combination of clinical and EEG features seems characteristic enough to justify prospective studies of similar cases in the future.

Partial epilepsy in neurologically normal children: clinical syndromes and prognosis.

A clinical and electroencephalographic study of 107 neurologically normal children with partial seizures was undertaken to verify the existence and determine the frequency of epileptic syndromes reported in selected populations. Sixty-three children had simple partial seizures, 39 had complex partial seizures, and 5 children were unclassifiable. The syndrome of benign partial epilepsy of children with rolandic spikes (BPEC, 38 cases) was clearly identified and its uniformly benign final prognosis was confirmed even if some of these children had at times severe or poorly controlled seizures. Among the children with simple partial seizures outside the BPEC (25 cases) and complex partial seizures (39 cases), no homogeneous clinical or electroclinical subgroup could be found. Two children with benign partial epilepsy and myoclonic-astatic seizures ("atypical benign partial epilepsy of childhood") and one child with "benign epilepsy with occipital spike-waves" were identified. 74% of children with epilepsy with complex partial seizures (ECP) had a 1-year seizure-free interval, and many children with epilepsy with simple partial seizures outside the BPEC group (ESP) had no more than two seizures. A benign course is thus not limited to the BPEC but is difficult to predict. Prospective studies are necessary to confirm the existence of well-defined benign syndromes among the idiopathic partial epilepsies of childhood, which appear quite rare outside the BPEC.

Prognosis of Partial Epilepsy in Childhood

Prognosis of Partial Epilepsy in Childhood

Partial epilepsy in childhood: clinical and EEG study of 261 cases.

A prospective clinical and EEG study is presented based on 261 children affected by partial seizures with or without focal EEG abnormalities, or by generalized seizures with focal EEG disturbances. From the clinical point of view, the partial seizures were most frequently elementary and motor, quite often associated with generalized seizures (64.36%), appearing in the majority of the cases during the first year of life, with rare frequency (less than 1 seizure/6 months). Perinatal disturbances appear to be the most important etiological factor. Partial epilepsy is often included within a previous neurological syndrome (secondary epilepsy). The clinical outcome is most frequently favorable (71.72%) with therapeutic treatment, especially with respect to the cases of primary epilepsy, characterized by rare seizures, which appear after the first year of life. From the EEG point of view, we noted specific monofocal abnormalities (72.79%), most frequently with temporal and rolandic localisation; during the follow-up the EEG abnormalities presented modifications in 58.89% of the cases, with normalisation in 22.98%.

Sylvian seizures and midtemporal spike foci in children.

"S ylvian SEIZURES" is a term proposed for one of the partial epilepsies of childhood, with a relatively elementary symptomatology, yet one that may be confused within the protean group of temporal lobe epilepsy. Their age incidence, the peripheral manifestations, the clinical and electrographic correlates, the mode of propagation, prognosis are all sufficiently homogenous to justify a special subgrouping. The most characteristic peripheral features can be summarized as follows. First, there is almost always some somatosensory involvement, most often of the tongue, but occasionally of inner cheeks, lips or gums, or even of a single tooth, while typical visceral aurae are rare. The sensory involvement is usually contralateral to the electroencephalographic focus but may be ipsilateral. More rarely a vertiginous component may be present. Second, speech arrest, not due to dysphasia but to motor interference, or anarthria. Third, preservation of consciousness, in most cases. Fourth, excessive pooling of saliva. Fifth,