1,721,136 research outputs found
COGNITION AND PAROXISMAL EEG ACTIVITIES: FROM A SINGLE SPIKE TO ELECTRICAL STATUS EPILEPTICUS DURING SLEEP
No full textNegative myoclonus. An overview of its clinical features, pathophysiological mechanisms, and management.
No full textNegative myoclonus (NM) is an unspecific motor disorder that can characterize a variety of neurological conditions. From the clinical point of view, NM appears as a shock-like involuntary jerky movement caused by a sudden, brief interruption of muscle activity. Asterixis is a type of NM that occurs typically in toxic-metabolic encephalopathies. NM of epileptic nature, or epileptic negative myoclonus (ENM), is defined as an interruption of tonic muscle activity, which is time-locked to an epileptic EEG abnormality, without evidence of an antecedent positive myoclonia in the agonist-antagonist muscles. ENM can be observed in idiopathic, cryptogenic, and symptomatic epileptic disorders. Pathophysiological hypotheses on the origin of NM involve subcortical as well as cortical mechanisms. Recent neuroimaging and neurophysiologic investigations, including intracerebral recordings and electrical stimulation procedures in epileptic patients, suggest the participation of premotor, primary motor, primary sensory, and supplementary motor areas in the genesis of NM. Polygraphic monitoring is essential for the diagnosis of NM, allowing the demonstration of brief interruptions of a tonic EMG activity, not preceded by a positive myoclonus in the agonist and antagonist muscles of the affected limb. Simultaneous EEG-EMG monitoring demonstrating the association of NM with an epileptic potential is consistent with the diagnosis of ENM. Evolution and prognosis of NM is mainly related to aetiology. In childhood idiopathic partial epilepsy, ENM can respond to some drugs (in particular, ethosuximide), whereas other medications (such as carbamazepine or phenytoin) have been reported to induce or worsen i
Night-time unravelling of the brain web: impaired synaptic downscaling in ESES--the Penelope syndrome.
No full textESES (encephalopathy with status epilepticus during sleep) is an epileptic encephalopathy with heterogeneous clinical manifestations (cognitive, motor, and behavioral disturbances in different associations, and various seizure types) related to a peculiar electroencephalography (EEG) pattern characterized by paroxysmal activity significantly activated during slow sleep—that is, a condition of continuous spikes and waves, or status epilepticus, during sleep. The pathophysiologic mechanisms underlying this condition are still incompletely understood; since 2005 we suggested that the abnormal epileptic EEG activity occurring during sleep might cause the typical clinical symptoms by interfering with sleep-related physiologic functions, and possibly neuroplasticity processes mediating higher cortical functions such as learning and memory consolidation (the ‘Penelope syndrome’ hypothesis - Tassinari et al., 2005, 2009; Tassinari and Rubboli, 2006). In this Editorial we discussed the results of the first study demonstrating that physiological downscaling during NREM sleep is impaired in ESES (Bölsterli et al. 2011), confirming our previous hypothesis, and opening a new way for the understanding of the relevance of paroxysmal activities during sleep, not only for ESES but for a large population of children with significant activation of focal paroxysmal epileptiform activity during sleep
Motor Manifestations in Epileptic Photosensitivity: Clinical Features and Pathophysiological Insights
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Eye-closure-triggered paroxysmal activity and cognitive impairment: a case report
No full textPURPOSE: To study the neuropsychological status of an epileptic patient presenting with epileptic activity triggered by eye closure in a 14-year follow-up period.
METHODS: The patient was studied at 12 and 26 years of age; during this period he underwent periodical clinical evaluations and EEG investigations; brain magnetic resonance imaging (MRI) was performed at 12 years of age. A neuropsychological assessment was carried out both at 12 years of age (T0) and at 26 years of age. At T0 and T1, neuropsychological tests (digits and words span, graphoestesia, reactions time to auditory stimuli, sentences repetition, words repetition, digital gnosis, backward counting [i.e.,100-0]) were performed during video-EEG monitoring either with eyes closed or with eyes open, to evaluate possible transitory effects related to ongoing epileptic activity. Moreover, at T0 the patient underwent Wechsler Intelligence Scale for Children-Revised, and at T1 to Wechsler Adult Intelligence Scale-Revised.
RESULTS: EEG recordings showed continuous epileptic activity triggered by eye closure, disappearing only with eyes opening, both at T0 and T1 (in this latter case, anteriorly predominant). The results of neuropsychological assessment during eyes closed as compared to performances with eyes open did not show significant differences, at T0 as well as at T1. Wechsler Intelligence scales showed a deterioration of performances at T1 with respect to T0; in addition, at T1, attention and short-term memory abnormalities, impairment in facial recognition and block design, and defective results in Continuous Performance Test and Wisconsin Card Sorting Test were observed.
CONCLUSIONS: Lack of differences between the results of neuropsychological tests performed with eyes closed as compared to the eyes open condition suggests that in our patient epileptic activity did not cause transitory cognitive abnormalities. Deterioration of Wechsler Intelligence Scales in the follow-up period might be interpreted as the result of a disruption of cognitive processes possibly related to the persistence of a continuous epileptic activity during eye closure over the years. We speculate whether a dysfunction in posterior cortical areas involved in visual processing might be related to the impairment in face recognition and block design tests as well to eye closure sensitivity
In search of the Rosetta Stone for ESES.
No full textWe commented on the lack of a common language among North-American pediatric epileptologists regarding encephalopathy with electrical status epilepticus during sleep or continuous spike-and-wave during sleep (ESES/CSWS) and related conditions.
In our view, there is no reason to consider the term “ESES” as indicative of EEG pattern, or for “CSWS” as the syndrome.
On the contrary, it will be valuable to use words and/or acronyms that denote the underlying concept, thus we proposed to use E-SES to account for the Epileptic Encephalopathy associated with a Status Epilepticus durind Sleep
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