Monika Eisermann

Infantile spasms in Down syndrome--effects of delayed anticonvulsive treatment.

To investigate the impact of treatment lag in infantile spasms (IS) on treatment response, occurrence of later epilepsy, and long-term cognition and behavior in patients with one single etiological entity, we examined 18 patients with Down syndrome (DS) and earlier IS retrospectively (follow-up period of 32-180 months with a mean of 85.1 months), and determined their history and present condition, in terms of previously mentioned items. There was a statistically significant correlation between treatment lag and lag to cessation of spasms (R=0.55, P=0.02), developmental quotient (DQ) (R=-0.75, P=0.003), and score of autistic features (AF) (R=0.57, P=0.04). Moreover we found that the later the response to treatment of IS, the lower was the DQ (R=-0.86, P=0.001) and the higher was the score of autistic features (R=0.5, P=0.06). A long duration of spasms also determined a low DQ (R=-0.93, P<0.0001) and a high score of autistic features (R=0.66, P<0.01). All patients with persistent epilepsy (n=5) had had a treatment lag of over 2 months. Conversely, for all children treated within 2 months (n=8) spasms ceased within 3 months of treatment and none of them had later epilepsy. This group of patients with a treatment lag of less than 2 months had earlier treatment response (P=0.002), higher DQ (P=0.004) and lower score of autistic features (P=0.006). The data stress the importance of a short treatment lag in view of mental development and prevention of later epilepsy and autistic features, and raise the question of antiepileptogenic effect in this specific condition.

Early electro-clinical features may contribute to diagnosis of the anti-NMDA receptor encephalitis in children

OBJECTIVE To describe initial and follow-up electroencephalographic (EEG) characteristics in anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. METHODS Consecutive polygraphic video-EEG recordings were analyzed in nine pediatric patients with anti-NMDAR encephalitis at the initial stage of the disease and during the intermediate period until motor recovery. EEG characteristics in waking and sleep stages as well as EEG correlates of abnormal movements are described. RESULTS In six patients, [corrected] the waking EEG showed preserved background activity and either focal or unilateral hemispheric slowing. These children had more favorable outcome than the three children with diffuse slowing. Unilateral [corrected] abnormal movements contra-lateral to hemispheric or focal slowing were also indicative of milder severity when compared to generalized abnormal movements and diffuse slowing. During non-rapid eye movement (NREM) sleep, a decrease in the expected slow waves and unilateral or diffuse theta-alpha band rhythms were observed in six children, not correlated with the outcome, representing a suggestive EEG pattern of anti-NMDAR encephalitis. [corrected]. CONCLUSIONS In pediatric patients presenting behavioral disorders and abnormal movements, early EEG patterns may be suggestive of anti-NMDAR encephalitis. Moreover early electro-clinical presentation contributes to outcome prediction. SIGNIFICANCE This case series demonstrates that early EEG patterns may be suggestive of anti-NMDAR encephalitis in pediatric patients with behavioral disorders and abnormal movements.

Cryptogenic late-onset epileptic spasms: an overlooked syndrome of early childhood?

Summary:  Purpose: Few reports detailing late‐onset epileptic spasms have been published. To determine whether this condition merely represents a late variant of classic West syndrome or exhibits specific features distinct from the latter and related to a later stage of brain maturation, we analyzed the whole population with this specific seizure type, excluding symptomatic cases to avoid the effect of brain lesion.

Normal EEG in childhood: from neonates to adolescents.

The important EEG changes that occur throughout childhood are a major challenge for the neurophysiologist. These reflect brain maturation, which is especially fast during the first year of life. This article describes normal EEG features and variants, characteristic patterns of development, as well as some patterns that are unusual for age, from the neonatal period to adolescence. We also describe how to adapt techniques and prepare patients in order to get interpretable records of appropriate duration, in neonates, infants, and young children.

Brain magnetic resonance imaging pattern and outcome in children with haemolytic‐uraemic syndrome and neurological impairment treated with eculizumab

The aim of this study was to describe the magnetic resonance imaging (MRI) findings and the neurological and neuropsychological outcomes in paediatric, diarrhoea‐associated haemolytic–uraemic syndrome (D+HUS) with central nervous system impairment treated with eculizumab, a monoclonal antibody.

Standardized computer-based organized reporting of EEG: SCORE – Second version

Standardized terminology for computer-based assessment and reporting of EEG has been previously developed in Europe. The International Federation of Clinical Neurophysiology established a taskforce in 2013 to develop this further, and to reach international consensus. This work resulted in the second, revised version of SCORE (Standardized Computer-based Organized Reporting of EEG), which is presented in this paper. The revised terminology was implemented in a software package (SCORE EEG), which was tested in clinical practice on 12,160 EEG recordings. Standardized terms implemented in SCORE are used to report the features of clinical relevance, extracted while assessing the EEGs. Selection of the terms is context sensitive: initial choices determine the subsequently presented sets of additional choices. This process automatically generates a report and feeds these features into a database. In the end, the diagnostic significance is scored, using a standardized list of terms. SCORE has specific modules for scoring seizures (including seizure semiology and ictal EEG patterns), neonatal recordings (including features specific for this age group), and for Critical Care EEG Terminology. SCORE is a useful clinical tool, with potential impact on clinical care, quality assurance, data-sharing, research and education.

Épilepsie et lésions focales chez l’enfant. Traitement chirurgical

Epilepsy surgery has gained a large role in the treatment of intractable seizures in the last few decades because of the development of operative techniques and better identification of the cerebral anomalies using electrophysiological recordings and neuroimaging. A series of 419 children, aged from five months to 15 years, with epilepsy (medically refractory in 85.5% of them) associated with focal cortical lesions, who underwent surgery between 1986 and 2006 was analyzed to identify the factors that correlated with outcome. Mean follow-up was 5.2 years. According to Engels classification; 75.8% of the children were seizure-free. When the lesion was well defined, correlations between clinical data, radiological features and electrophysiological features, suggesting a zone of seizure onset around (or even in) the lesion, was the best guarantee of achieving good seizure control by lesionectomy. Nevertheless, seizure outcome was also determined by other factors: the duration of the epilepsy and the surgery. Persistence of seizures was found to be significantly associated with the preoperative duration of epilepsy, the completeness of the lesional resection and de novo brain damage induced by the surgical procedure itself. Early surgery must be considered in children because of the benefits of seizure control on the developing brain and the risk of secondary epileptogenesis.

Can fever treat epileptic encephalopathies

PURPOSE To describe resistant epileptic encephalopathies that significantly improved after an acute febrile episode (FE). METHODS We reviewed the clinical history of patients with daily pharmacoresistant seizures referred to the Saint-Vincent de Paul Hospital in the last 5 years. Four patients experienced seizure arrest in relation with a febrile episode. RESULTS The four patients suffered from epileptic encephalopathy. Three were symptomatic, one cryptogenic. They presented spasms and atypical absences, beginning after the age of 1 year. All seizures stopped at the onset of fever, and significant EEG improvement was observed. The seizure-free period ranged from 2 to 24 months. DISCUSSION AND CONCLUSION The close link between the occurrence of FE and the disappearance of seizures and EEG improvement, contrasting with the previous pharmacoresistance of this epileptic encephalopathy, supports a non fortuitous association. Several mechanisms could explain this phenomenon, including viral etiology, hyperthermia, inflammatory-immune reaction and ACTH release. Better understanding this phenomenon could open new therapeutic perspectives.

Epilepsy in young Tsc1+/− mice exhibits age‐dependent expression that mimics that of human tuberous sclerosis complex

To describe the epileptic phenotype of Tsc1+/− mice pups in comparison with age‐related seizures in human tuberous sclerosis complex (TSC).

EEG in children, in the laboratory or at the patient's bedside.

In pediatrics, EEG recordings are performed on patients from the neonatal period up to young adults. This means adapting techniques to many different conditions, concerning not only the patients age, the need for asepsis and the patients behavior, but also the environment (e.g. in the laboratory, at the patients bedside, or in the neonatal intensive care unit [NICU]). Technical requirements depend on age, indication and the type of examination; in infancy, there should be a minimum of 12 EEG electrodes, ECG and respiration recording. In epileptology, surface EMG is also necessary to characterize the type of seizures and refine the diagnosis of epilepsy syndrome, on which physicians will base their treatment choice. The role of the EEG technician is essential because the quality of the recording, its analysis and conclusion will depend on the quality of the technical set-up and the interaction with the child. Sleep is a systematic part of the study up to the age of 5 years for several reasons: sleep EEG yields information on brain maturation; the EEG tracing during wakefulness can contain too many artefacts; and some grapho-elements, key to the diagnosis, only appear during sleep. The time of the examination must be chosen according to the childs usual nap times, possibly after sleep deprivation. Grapho-elements and spatio-temporal organization of the EEG vary with age, and normal variants and unusual aspects are quite wide for any given age; this is why a physician experienced in pediatric EEG should perform the interpretation. This chapter concerns EEG performed in infants, children and adolescents, its technical aspects according to age and indications (general pediatrics, emergency, epilepsy).