Teresa Randò

Epileptic disorders with onset in the first year of life: neurological and cognitive outcome

We examined prospectively a series of 150 children with epilepsy beginning in the first year of life. We classified the types of epilepsy into five categories: West syndrome, other epileptic encephalopathies, generalized, partial and undetermined epilepsies. Of 150 patients, 15 died; 135 were followed for at least 4 years. In order to define possible factors influencing prognosis we evaluated neurological and cognitive outcome and made percentage comparisons between groups, for aetiology, age of onset, family history of epilepsy, and psychomotor development before onset. Epileptic encephalopathies as well as the symptomatic forms of West syndrome showed a very poor neurological and cognitive outcome. As previously recognized, only cryptogenic forms of West syndrome had a benign prognosis. For the generalized epilepsies, analysis of different factors, namely late age at onset, cryptogenicity and absence of primary cognitive impairment, indicated a good prognosis. In contrast, partial epilepsies usually had a poor outcome, irrespective of the aetiology, whether cryptogenic or symptomatic.

Development of visual attention in West syndrome.

Summary:  Purpose: To study prospectively the evolution of visual attention in children with West syndrome to evaluate its development before the onset of spasms, its possible deterioration as a consequence of epileptic disorders, and its outcome at the age of 2 years, and the possible relation between the impairment of visual attention and cognitive development.

Visual function in infants with West syndrome: correlation with EEG patterns.

Summary:  Purpose: Several studies have reported behavioral and electrophysiological evidence of visual impairment during the active stage of West syndrome. The underlying mechanisms are, however, poorly understood, and little has been reported about the correlation between visual impairment, EEG patterns, and brain lesions. The aim of the study was to assess visual function at the onset of spasm and 2 months thereafter and relate visual findings to brain lesions and EEG features.

Epilepsy in Shunted Posthemorrhagic Infantile Hydrocephalus Owing to Pre- or Perinatal Intra- or Periventricular Hemorrhage

Epilepsy is relatively common in infants with hydrocephalus. Its mechanism is controversial; in fact, studies on etiologically heterogeneous series are not able to clarify the mechanism generating epilepsy or to suggest effective prevention and treatment strategies. Our study is aimed at assessing the onset and evolution of epilepsy, as well as concurrent cognitive development of a homogeneous series of shunted posthemorrhagic hydrocephalus owing to pre- or perinatal intra- or periventricular hemorrhage. Forty patients were enrolled in the study. Twenty-six were patients with grade II—III intraventricular hemorrhage, 16 of whom had associated ischemic lesions. In the remaining 14 patients, a grade IV intraventricular hemorrhage was found. Epilepsy was observed in 27 patients. Aside from 10 cases with nonsyndromic forms of epilepsy, it was possible to define at least three different age-dependent epileptic syndromes: symptomatic neonatal location-related epilepsy with transient Wests syndrome in infancy in 5 patients; Wests syndrome in 8 patients; and continuous spike-waves during sleep in 4 patients. Epilepsy was significantly correlated with ischemic lesions only. Early thalamic injuries frequently evolved toward continuous spike-waves during sleep, indicating that patients with thalamic injury must be monitored to detect continuous spike-waves during sleep early. Cerebellar atrophy, in addition to epilepsy and other brain injuries, accounted for disorders of cognitive development. (J Child Neurol 2005;20:219—225).

Cognitive competence at the onset of West syndrome: correlation with EEG patterns and visual function

The aim of this study was to evaluate cognitive development at the onset of West syndrome (WS) with regard to electroencephalogram (EEG) patterns and visual function. Twenty-five patients (14 males, 11 females) at the onset of spasms (T0) in WS and 2 months later (T1) underwent a full clinical evaluation, including neuroimaging, cognitive assessment, video-EEG, and visual function. Mean age of the patients at spasm onset was 5.9 months (SD 2.5; range 2 to 13mo). Cognitive development, assessed with Griffiths Mental Development Scales (GMDS), was generally impaired at T0 and was significantly related to visual function (p<0.001) at both T0 and T1. In general, there was a specific major impairment in the eye-hand coordination scale of the GMDS which tended to disappear after 2 months in less severe cases. At the onset of spasms, sleep EEG organization seemed to be better related to cognitive abilities than awake hypsarrhythmia. These results support a close link between visual function and cognitive competence in WS and provide additional information to improve the understanding of possible mechanisms underlying cognitive impairment.

Brain ischemic lesions of the newborn

Abstract Ischemia is the most frequent pathogenetic mechanism of brain lesions in infancy. The authors give a brief report on the recent advances achieved in knowledge of the underlying neuropathology, clinical manifestations, strategies of management and outcome of ischemic brain lesions in the newborn. A better knowledge of pathophysiological mechanisms is necessary so that prevention can be made more effective and outcomes improved. As far the vulnerability to ischemic insults in term and preterm infants is concerned, the different types of brain damage cause various neurological consequences at different gestational ages. Thus, the authors deal separately with white matter lesions, which are typical of infants born prematurely, and cortical brain ischemic lesions, which are found in full-term newborns.

Prognostic value of EEG performed at term age in preterm infants

RationaleElectroencephalography (EEG) was performed at term age on 32 infants born prematurely (25–32 weeks). EEG was assessed looking for overall background activity and transients.MethodsA quantitative analysis was performed, selecting 5-min epochs of “tracé alternant” free of artefacts during quiet sleep. EEG findings were compared with cranial ultrasound (US) findings at term age and with neurodevelopmental outcome at 2 years (Student’s t-test).ResultsThe overall EEG background activity was not always related to the outcome or to the severity of cranial US. Infants with normal US and normal outcome had longer synchrony percentage of bursts, longer maximum duration of bursts and shorter mean of abnormal transients per interbursts than children with major lesions and abnormal outcome. Infants with minor lesions, who all had normal outcome, also had better results than those with major lesions and abnormal outcome, but the range of the EEG findings was more variable.ConclusionOur results suggest that the EEG performed at term age does not provide additional prognostic information compared to cranial US.