Non-convulsive status epilepticus causing focal neurological deficits in CADASIL.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary small vessel disease caused by mutations of the Notch3 gene. Clinical manifestations include migraine with or without aura, psychiatric disorders, recurrent ischaemic strokes and cognitive decline. Brain MRI shows confluent hyperintense signal alterations involving characteristically the anterior part of the temporal lobes and widespread areas of the deep and periventricular white matter. Focal or generalised seizures represent a rare neurological manifestation in CADASIL with a frequency of 6-10% in two large series. Status epilepticus, however, has not been reported so far. Herein we describe a patient with CADASIL with an acute focal neurological deficit following a prolonged migraine attack. The symptoms were first interpreted as an ischaemic stroke but subsequently diagnosed to be due to a non-convulsive status epilepticus.

Non-convulsive status epilepticus causing focal neurological deficits in CADASIL.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary small vessel disease caused by mutations of the Notch3 gene. Clinical manifestations include migraine with or without aura, psychiatric disorders, recurrent ischaemic strokes and cognitive decline. Brain MRI shows confluent hyperintense signal alterations involving characteristically the anterior part of the temporal lobes and widespread areas of the deep and periventricular white matter. Focal or generalised seizures represent a rare neurological manifestation in CADASIL with a frequency of 6-10% in two large series.1(,)2 Status epilepticus, however, has not been reported so far. Herein we describe a patient with CADASIL with an acute focal neurological deficit following a prolonged migraine attack. The symptoms were first interpreted as an ischaemic stroke but subsequently diagnosed to be due to a non-convulsive status epilepticus.

Non-convulsive seizures and non-convulsive status epilepticus monitoring in the intensive care unit. A real need for the Gulf Cooperation Council countries.

Continuous EEG (cEEG) monitoring in the intensive care unit (ICU) is essential for detecting non-convulsive seizures/status epilepticus (NCSs, NCSE). Currently there exist a number of continuous EEG monitoring systems adapted for use in the ICU. However, these systems have been trained using EEG data collected from healthy, neurologically intact patients with epileptic seizures, a very different patient population from ICU patients. The review consists of 2 parts, clinical and technological aspects. In the first one, we summarize the electroencephalographic aspects of NCSs/NCSE and other EEG patterns encountered in the ICU. In the second part, we explain how to develop a novel cEEG monitoring system to be used in Hamad Medical Corporation ICUs, Doha, Qatar, that is able to detect pathological EEG patterns commonly occurring in the critically ill patient. Real-time monitoring of seizure discharges, and other pathological EEG patterns will allow correct diagnosis and adequate treatment in a timely fashion.

Fast oscillations trigger bursts of action potentials in neocortical neurons in vitro: a quasi-white-noise analysis study.

PURPOSE: Recent evidence supports the importance of action potential bursts in physiological neural coding, as well as in pathological epileptogenesis. To better understand the temporal dynamics of neuronal input currents that trigger burst firing, we characterized spectral patterns of stimulation current that generate bursts of action potentials from regularly spiking neocortical neurons in vitro. METHODS: Sharp microelectrodes were used for intracellular recording and stimulation of cortical neurons in rat brain slices. Quasi-white-noise (0-2 kHz) and "chirp" sine wave currents of decreasing wavelength were applied to represent a broad spectrum of stimulation frequencies. Action potential-related averaging of the stimulation current variations preceding bursting was used to characterize stimulation current patterns more likely to result in a burst rather than a single-spike response. RESULTS: Bursts of action potentials were most reliably generated by a preceding series of > or = 2 positive current transients at 164+/-37 Hz of the quasi-white-noise, and to sine wave currents with frequencies greater than 90 Hz. The intraburst action potential rate was linearly related to the frequency of the input sine wave current. CONCLUSIONS: This study demonstrates that regularly spiking cortical neurons in vitro burst in response to fast oscillations of input currents. In the presence of positive cortical feedback loops, encoding input frequency in the intraburst action potential rate may be safer than producing a high-frequency regular output spike train. This leads to the experimentally testable and therapeutically important hypothesis that burst firing could be an antiepileptogenic and/or anti-ictogenic mechanism.

Proposed criteria for referral and evaluation of children for epilepsy surgery: recommendations of the Subcommission for Pediatric Epilepsy Surgery.

The Commission on Neurosurgery of the International League Against Epilepsy (ILAE) formed the Pediatric Epilepsy Surgery Subcommission in 1998 and charged it with formulating guidelines and recommendations for epilepsy surgery in childhood. Also endorsed by the Commission on Paediatrics, the following document is the consensus agreement after a meeting of 32 individuals from 12 countries in 2003. The panel agreed that insufficient class 1 evidence exists to recommend practice guidelines at this time. Instead, the panel generated criteria concerning the unique features of pediatric epilepsy patients to justify dedicated resources for specialty pediatric surgical centers, suggested guidelines for physicians for when to refer children with refractory epilepsy, and recommendations on presurgical evaluation and postoperative assessments. The panel also outlined areas of agreement and disagreement on which future research and consensus meetings should focus attention to generate practice guidelines and criteria for pediatric epilepsy surgery centers.