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West Syndrome (west + syndrome)
Selected AbstractsICTAL EEG Fast Activity in West Syndrome: From Onset to OutcomeEPILEPSIA, Issue 11 2007Ferruccio Panzica Summary Purpose: To characterize the fast EEG activities associated with infantile spasms in West syndrome, and their value in predicting the recurrence and localization of late seizures. Methods: We selected 23 infants who were followed for at least 2 years. Selected EEG recordings underwent autospectra, coherence, and phase analyses in order to assess the changes during follow-up. Results: Short discharges of fast-rhythms (331 ± 190 ms) with a lateralized onset were detected in 18 of the 23 infants (78.3%). There were no significant differences in the parameters characterizing ICTAL beta-activity (frequency, duration, inter-hemispheric coherence, or transfer time) between the infants with or without seizure recurrence. However, beta-discharges with a consistent location formed part of the ICTAL EEG in all 10 infants with seizure recurrence, but only in eight (61.5%) of those who remained seizure-free (SF) (p < 0.05). In all but one of the infants experiencing seizure recurrence, the ICTAL discharges associated with the late seizures apparently originated from the same hemisphere as that involved at the beginning of the spasm-associated beta-activity, although the precise location varied. Conclusions: Spectral, coherence and phase analyses detected spasm-associated runs of lateralized beta-rhythms in many of our infants with West syndrome. This ICTAL pattern significantly correlated with seizure recurrence. The consistent lateralization of the ICTAL EEG events associated with both the early spasms and late seizures suggests that EEG beta-activities should be considered as indicating local cortical dysfunction in infants who fail to respond to early treatment and often progress toward severe epilepsy. [source] Visual Function in Infants with West Syndrome: Correlation with EEG PatternsEPILEPSIA, Issue 7 2004Teresa Ranḍ 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. Methods: Twenty-five infants with West syndrome were enrolled and studied with (a) a full clinical assessment including a battery of tests specifically designed to assess visual function, (b) a video-polygraphic study, and (c) brain magnetic resonance imaging (MRI). Besides brain neuroimaging and EEG comparison with visual function, an intra-EEG analysis was performed to investigate the possible relation of EEG patterns to fluctuating visual behavior (fixation and following). Results: Twenty-two children had at least one abnormal result on one or more of the tests assessing visual function at T0. Visual impairment at the spasm onset was related to the sleep disorganization rather than to the hypsarrhythmic pattern in awake EEG. After 2 months, both EEG features become significantly linked to visual function. Visual function improved in several cases after 2 months, in parallel with the seizure regression. No relation was found between EEG patterns and fluctuating visual behavior. Conclusions: The study supplies new evidence of the involvement of visual function in West syndrome. The presence of abnormal visual findings in infants without lesions on brain MRI suggests that visual abnormalities are due not only to brain injury but also to epileptic disorder per se. New insight is also provided into the possible mechanisms underlying clinical and EEG abnormalities. [source] ICTAL EEG Fast Activity in West Syndrome: From Onset to OutcomeEPILEPSIA, Issue 11 2007Ferruccio Panzica Summary Purpose: To characterize the fast EEG activities associated with infantile spasms in West syndrome, and their value in predicting the recurrence and localization of late seizures. Methods: We selected 23 infants who were followed for at least 2 years. Selected EEG recordings underwent autospectra, coherence, and phase analyses in order to assess the changes during follow-up. Results: Short discharges of fast-rhythms (331 ± 190 ms) with a lateralized onset were detected in 18 of the 23 infants (78.3%). There were no significant differences in the parameters characterizing ICTAL beta-activity (frequency, duration, inter-hemispheric coherence, or transfer time) between the infants with or without seizure recurrence. However, beta-discharges with a consistent location formed part of the ICTAL EEG in all 10 infants with seizure recurrence, but only in eight (61.5%) of those who remained seizure-free (SF) (p < 0.05). In all but one of the infants experiencing seizure recurrence, the ICTAL discharges associated with the late seizures apparently originated from the same hemisphere as that involved at the beginning of the spasm-associated beta-activity, although the precise location varied. Conclusions: Spectral, coherence and phase analyses detected spasm-associated runs of lateralized beta-rhythms in many of our infants with West syndrome. This ICTAL pattern significantly correlated with seizure recurrence. The consistent lateralization of the ICTAL EEG events associated with both the early spasms and late seizures suggests that EEG beta-activities should be considered as indicating local cortical dysfunction in infants who fail to respond to early treatment and often progress toward severe epilepsy. [source] Nonepileptic Disorders Imitating Generalized Idiopathic EpilepsiesEPILEPSIA, Issue 2005Natalio Fejerman Summary:, Differential diagnosis between epileptic and nonepileptic paroxysmal disorders is fundamental not only to allow correct management of patients but also to avoid the burden of unnecessary antiepileptic medication. The focus of this chapter is limited to imitators of idiopathic generalized epilepsies (IGE) which are expressed through myoclonic, tonic,clonic, tonic, atonic, and absence seizures. Apparent losses of consciousness and drop attacks also have to be considered. Benign myoclonus of early infancy is the main nonepileptic disorder in the differential diagnosis of infantile spasms, but is not dealt with here because West syndrome is not an IGE. Hyperekplexia, metabolic disorders, hypnagogic myoclonus, and disturbed responsiveness caused by the use of drugs are listed in Table 1. Other conditions that may imitate more focal epileptic seizures are omitted. Benign neonatal sleep myoclonus, apnea and apparent life-threatening events in infants, cyanotic and pallid breath-holding spells, syncope, staring spells, psychogenic seizures, hyperventilation syndrome, and narcolepsy have been selected based on frequency or difficulties in differential diagnosis with the intention to cover the most conspicuous imitators of IGE in different ages. Table 1. Nonepileptic disorders imitating idiopathic generalized epilepsies [source] Visual Function in Infants with West Syndrome: Correlation with EEG PatternsEPILEPSIA, Issue 7 2004Teresa Ranḍ 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. Methods: Twenty-five infants with West syndrome were enrolled and studied with (a) a full clinical assessment including a battery of tests specifically designed to assess visual function, (b) a video-polygraphic study, and (c) brain magnetic resonance imaging (MRI). Besides brain neuroimaging and EEG comparison with visual function, an intra-EEG analysis was performed to investigate the possible relation of EEG patterns to fluctuating visual behavior (fixation and following). Results: Twenty-two children had at least one abnormal result on one or more of the tests assessing visual function at T0. Visual impairment at the spasm onset was related to the sleep disorganization rather than to the hypsarrhythmic pattern in awake EEG. After 2 months, both EEG features become significantly linked to visual function. Visual function improved in several cases after 2 months, in parallel with the seizure regression. No relation was found between EEG patterns and fluctuating visual behavior. Conclusions: The study supplies new evidence of the involvement of visual function in West syndrome. The presence of abnormal visual findings in infants without lesions on brain MRI suggests that visual abnormalities are due not only to brain injury but also to epileptic disorder per se. New insight is also provided into the possible mechanisms underlying clinical and EEG abnormalities. [source] West syndrome and mitochondrial diabetes: relationship or coincidence?CLINICAL ENDOCRINOLOGY, Issue 1 2002B. Bouhanick Summary West syndrome occurs in infancy and in early childhood. It is characterized by intractable seizures occurring almost daily, severe psychomotor retardation, poor prognosis and EEG abnormalities, known as hypsarrhythmia. We report here the case of a 28-year-old patient, who was diagnosed with West syndrome when he was 8 months old and with diabetes mellitus when he was 25 years old. Sequencing analyses and restriction analyses were suggestive of mitochondrial diabetes. Four years after the diagnosis of diabetes, this patient's diabetes is still controlled by diet and biguanides. [source] ,Cognitive competence at the onset of West syndrome'DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 11 2006T Fosi No abstract is available for this article. [source] | ||||||||||