M. Brinciotti

Outcome after discontinuation of antiepileptic drug therapy in children with epilepsy.

Summary: We studied recurrence risks and predictive factors of relapse after antiepileptic drug (AED) discontinuation in a prospective analysis of 425 children with epilepsy who had not had a seizure for at least 2 years (follow‐up after withdrawal 1.6–12 years, mean 8 years). Factors closely related by multivariate analysis to relapse were neurologic abnormalities, mental retardation, seizure type (infantile spasms, absence seizures), and appearance or persistence of EEG abnormalities during the course of the illness and before discontinuation. When multivariate analysis was performed to evaluate outcome of patients with a first relapse (isolated vs. multiple relapses), the variables closely related to a poor prognosis were etiologic factors, first relapse characterized by more than one seizure in a 24‐h period, seizure‐free period <4 years, unchanged seizure type at first relapse, more than one AED for seizure control, and abnormal EEG before the first relapse. In itself, resumption of therapy did not influence outcome. At the study cutoff point, 88% of patients with relapse were again seizure‐free. We conclude that AEDs can safely be discontinued if predictive factors are considered to individualize the risk of relapse for each patient.

Responsiveness of the Visual System in Childhood Migraine Studied by Means of VEPs

We have tried to ascertain whether the increased visual evoked potential (VEP) amplitude found in adult migraineurs is present also in children with migraine. We investigated 43 children, 26 male and 17 female, with a mean age of 11.4 years, 24 with common and 19 with classic migraine, and compared them with a control group of 20 children, 11 male and 9 female, with a mean age of 9.7 years. Flash and pattern reversal VEPs were recorded in both groups, and the study was carried out in the pain-free interval between attacks. The children with migraine showed a significant (p < 0.01) increase in VEP amplitude on flash stimulation but not on pattern reversal. There were no differences between classic and common migraine. The abnormal responsiveness of the visual system seems to be related to variations in light intensity rather than to spatial contrasts.

Electroclinical features in children and adolescents with epilepsy and/or migraine, and occipital epileptiform EEG abnormalities.

This study attempted to better define clinical and EEG features for differential diagnosis between epilepsy and migraine in children with occipital epileptiform EEG abnormalities. We studied 126 children (57 males, 69 females; age 4-18 years) suffering from epilepsy (63), migraine (43) or both (20). Patients were selected because of the presence of epileptiform abnormalities in the occipital regions on their EEG at rest. Differences among groups were statistically analyzed (Pearson chi square; ANOVA) for sex, age at onset of seizures and migrainous attacks, family history, ictal signs and symptoms, EEG at rest (unilateral vs bilateral distribution of epileptiform abnormalities), and EEG during Hyperventilation (HV) and Intermittent Photic Stimulation (IPS). Significant differences were found in family history, ictal signs and symptoms, EEG at rest and during activation tests. A family history of epilepsy, visual symptoms such as colored hallucinations and micro/macropsias, frequently associated with clinical signs in the visual system (eye deviation, nystagmus), unilateral EEG abnormalities, and abnormal response to IPS were closely related to diagnosis of epilepsy. On the other hand, a family history of migraine, visual symptoms such as amaurosis and scotomata, without evident clinical signs, bilateral EEG abnormalities, and no changes during IPS were significantly related to migraine. In conclusion, these clinical and EEG differences should be considered in the differential diagnosis between epilepsy and migraine in children with occipital epileptiform EEG abnormalities.

Pattern Sensitivity and Photosensitivity in Epileptic Children with Visually Induced Seizures

Summary: We evaluated photosensitivity and pattern sensitivity in 74 epileptic children (38 males, 36 females aged 4 4–19 years; mean age 11.9 years) with reflex seizures induced by environmental visual stimuli and analyzed clinical and EEG characteristics of patients according to type of sensitivity. Standard procedures of visual stimulation were used in all cases. Seven children were excluded because of poor cooperation (3) or no activation (4).Fifty‐one percent of the remaining 67 patients showed sensitivity to both light and pattern, whereas 33% showed photosensitivity and 16% showed pattern sensitivity. Generalized abnormalities were more frequently elicited by intermittent light stimulation than by pattern (73 vs. 36%, p < 0.001). Significant differences in clinical and EEG findings were noted among patients according to their sensitivity to light or/and pattern. In particular, pattern‐sensitivity patients without photosensitivity had a higher occurrence of localization‐related symptomatic epilepsies, neurologic abnormalities, and epileptiform EEG abnormalities, mainly focal, at rest.

Familial Occurrence of Benign Myoclonus of Early Infancy

Summary: Three cases of benign myoclonus of early infancy (BMEI) were observed in the same family. Previously, only sporadic cases have been reported. Electroencephalogram (EEG) recordings were consistently normal, and the affected girls had normal neurological development. Therapy was not administered and the episodes spontaneously disappeared within the first months of life. While etiological mechanisms of BMEI are still unknown, a hereditary mechanism is now hypothesized on the basis of these cases.

Pattern-sensitive epilepsy : genetic aspects in two families

Summary: We report two families in which five members (three in one and two in the other) suffer from patternsensitive epilepsy. In all affected members seizures were induced by environmental visual patterned stimuli. Diagnosis was confirmed by neurophysiological studies, including EEG with pattern and intermittent light stimulation and visual evoked potentials. The role of genetic aspects and the relationship between this form of reflex seizures and photosensitive epilepsy are emphasized.

Self‐Induced Pattern‐Sensitive Epilepsy in Childhood

Abstract. We studied five children (1 boy and 4 girls) with self‐induced pattern‐sensitive epilepsy. All patients had refractory epilepsy with multiform, though mainly myoclonic, seizures and medium grade to severe mental retardation. Spontaneously self‐induced seizures were documented in all cases by EEG. All the patients underwent full neurophysiological assessment (baseline EEG recording, with activation: eyelid closed, hyperventilation, ILS, EEG during the randomized presentation of 3 types of spatial structured stimuli, VEP‐F and VEP‐PR). The pattern that triggered the EEG anomalies was highly specific and selective for each patient. Clinical seizures were reproduced by the same patterns as the ones used by the patients to bring on the seizures at will. Authors stress the importance of identifying such peculiar type of epilepsy and of attempting adequate treatment.

Decharges EEG localisees continues pendant le sommeil chez l'enfant

Continuous generalized spike-and-wave complexes occurring during slow-sleep were first observed in some children by Patry et al. (1971). A general review of the reported cases was made by Tassinari et al. (1984). We have observed a new clinical-EEGraphic picture. Spikes and spike-and-wave complexes localized to one hemisphere were recorded during at least 85% non-REM sleep both in 3 quadriplegic and in 3 hemiplegic mentally retarded children, aged from 2 years and 10 months to 11 years (mean age: 5 years and 2 months). Cyclic organization of sleep was almost normal. Wake EEG showed an epileptic focus. Brain CT showed midline defect and brain atrophy in quadriplegic patients, and a wide temporal-parietal porencephalic cyst in hemiplegic patients. This EEGraphic pattern was associated with a more pronounced mental impairment: neuropsychological improvement was observed in 2 cases, when continuous sleep discharges disappeared. As aforesaid, this picture seems to be related either to hemispheric or to midline brain defects. In our cases, sleep phase organization could be examined, while continuous generalized discharges occurring during sleep do not allow such a study.

Visual evoked potentials in infants of diabetic mothers: Relations to clinical and metabolic status during pregnancy and delivery

OBJECTIVE To evaluate Visual Evoked Potentials (VEPs) and psychomotor development of infants of diabetic mothers (IDMs) in relation to clinical and metabolic data during pregnancy and delivery. METHODS VEPs and psychomotor development (Brunet-Lézine) were analysed in 40 two-month-old IDMs (21 males, 19 females), 24 from mothers with type-1 diabetes, 13 gestational diabetes, and 3 type-2 diabetes. Normative VEP data were obtained from 63 age matched controls. RESULTS VEP latencies were significantly longer in IDMs than in controls (O1 wave IV=197.9+/-35.5 vs 155.3+/-30.3; P<0.001; O2 wave IV=200.2+/-33.8 vs 155.6+/-29.0; P<0.001). The mean developmental quotient was normal. In IDMs with type-1 diabetes delayed VEPs were related to increased weight during pregnancy (r 0.516; P 0.009), 1st trimester fasting blood glucose (r 0.458; P 0.037), insulin requirement during the 2nd (r 0.441; P 0.035) and 3rd trimester (r 0.422; P 0.039); in IDMs with gestational diabetes, VEP latency showed negative relation to Apgar scores (r -0.748; P 0.008). CONCLUSIONS IDMs have delayed VEPs, which may possibly be related to poor metabolic control in pre-gestational diabetes, and to delivery complications in gestational diabetes. SIGNIFICANCE IDMs show subtle neurophysiologic changes detectable by VEPs.

Reading epilepsy with absences, television-induced seizures, and pattern sensitivity

A 14-year-old right-handed girl suffering from absence seizures from age 6 began to have reflex seizures elicited by reading and watching television when she was 13. Neurophysiological studies showed pattern sensitivity and photo-sensitivity. VEPs, obtained with flash and pattern reversal stimulation, were normal. This atypical form of reading epilepsy suggests an interaction of pattern vision and cognitive functions as precipitating stimuli in reflex seizures.