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Panayiotopoulos syndrome: a consensus view
- Colin Ferrie, Roberto Caraballo, Athanasios Covanis, Veysi Demirbilek, Aysin Dervent, Sara Kivity, Michael Koutroumanidis, Zarko Martinovic, Hirokazu Oguni, Alberto Verrotti, Federico Vigevano, Kazuyoshi Watanabe, Despina Yalcin, Harami Yoshinaga
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- Journal:
- Developmental Medicine and Child Neurology / Volume 48 / Issue 3 / March 2006
- Published online by Cambridge University Press:
- 17 February 2006, pp. 236-240
- Print publication:
- March 2006
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- Article
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The aim of this paper is to promote the correct classification of, and provide guidelines on, the diagnosis and management of Panayiotopoulos syndrome (PS). An international consortium of established researchers in the field collaborated to produce a consensus document. The resulting document defines PS, characterizes its electro-clinical features, considers its likely pathogenesis, and provides guidance on appropriate management. We conclude that PS is a common idiopathic, benign seizure disorder of childhood, which should be classified as an autonomic epilepsy, rather than an occipital epilepsy.
21 - Shuddering and benign myoclonus of early infancy
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- By Christa Pachatz, Section of Neurophysiology, Bambino Gesù Children's Hospital, Rome, Italy, Lucia Fusco, Section of Neurophysiology, Bambino Gesù Children's Hospital, Rome, Italy, Federico Vigevano, Section of Neurophysiology, Bambino Gesù Children's Hospital, Rome, Italy, Natalio Fejerman, Section of Neurophysiology, Bambino Gesù Children's Hospital, Rome, Italy, Roberto Caraballo, Section of Neurophysiology, Bambino Gesù Children's Hospital, Rome, Italy
- Edited by Renzo Guerrini, University of London, Jean Aicardi, Hôpital Robert-Debré, Paris, Frederick Andermann, Montreal Neurological Institute & Hospital, Mark Hallett, National Institutes of Health, Baltimore
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- Book:
- Epilepsy and Movement Disorders
- Published online:
- 03 May 2010
- Print publication:
- 13 December 2001, pp 343-352
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Summary
Introduction
Benign myoclonus of early infancy is a paroxysmal phenomenon in neurologically healthy infants with onset in the first year of life and a benign self-limited course. It was first described by Lombroso and Fejerman (1977) with the presentation of 16 cases based mainly on anamnestic features. The first polygraphic study on BM was reported by Dravet et al. (1986), who decided to call the phenomenon benign non-epileptic infantile spasms because of the close relationship with the clinical picture of epileptic spasms of West syndrome. We illustrated the clinical and neurophysiological study of 5 patients with BM (Pachatz et al., 1999) and showed that the so-called myoclonus is not a myoclonus in neurophysiological terms, and that it is also clearly distinguishable from epileptic spasms at electromyography and even from a clinical point of view. Since that report, we obtained additional clinical and neurophysiological data in another three children, confirming that BM in its most typical form is characterized by a brief shudder-type axial motor manifestation, which can superimpose a series of other paroxysmal motor phenomena.
Clinical and video-polygraphic findings from a recent series
Between the years 1994 and 1999 we studied eight children, three girls and five boys, at the Division of Neurology, Bambino Gesù Children's Hospital, Rome, Italy, using video-EEG recording in all eight patients and polygraphic recording in four of eight cases. Scalp electrodes were placed according to the International 10–20 System. Polygraphic recordings included surface electromyographic (EMG) recording from the two deltoid muscles, channels for respirogram and electrocardiogram.
In all patients we studied, family or personal history was negative for neurological pathologies.
7 - Nocturnal frontal lobe epilepsy
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- By Paolo Tinuper, Institute of Clinic al Neurology, University of Bologna, Italy, Elio Lugaresi, Institute of Clinic al Neurology, University of Bologna, Italy, Federico Vigevano, Section of Neurophysiology, Bambino Ges Children's Hospital, Rome, Italy, Samuel F. Berkovic, Department of Neurology, Austin and Repatriation Medical Centre, University of Melbourne, Vic toria, Australia
- Edited by Renzo Guerrini, University of London, Jean Aicardi, Hôpital Robert-Debré, Paris, Frederick Andermann, Montreal Neurological Institute & Hospital, Mark Hallett, National Institutes of Health, Baltimore
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- Book:
- Epilepsy and Movement Disorders
- Published online:
- 03 May 2010
- Print publication:
- 13 December 2001, pp 97-110
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- Chapter
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Summary
Introduction
Epilepsy is a chronic condition characterized by recurring seizures. In most epileptic syndromes spontaneous seizures are random, i.e they occur independently from the patient's state of arousal. However, the role of the sleep state, and particularly different stages of sleep, in facilitating ictal or interictal discharges, has been the object of several studies and recent reviews (Shouse et al., 1997a, b).
The synchronized EEG activity and preserved muscular tone characterizing the early stage of sleep facilitate the propagation of interictal discharges and seizure onset, whereas inhibited muscular tone and desynchronized EEG activity prevent seizure onset during Rem sleep. Moreover, thalamocortical drive evoking physiological sleep transients (K-complexes and spindles activity) and burst-pause firing in cortical neurons during NRem sleep may facilitate the spread of bisynchronous discharges in generalized epilepsies.
The most common epileptic situations related to sleep include some forms of idiopathic generalized epilepsies such as epilepsy with grand mal (GTC) on awakening and juvenile myoclonic epilepsy (JME) in which myoclonic jerks or generalized convulsion appear typically after awakening and are provoked by sleep deprivation. Sleep is a strong seizure trigger in benign epilepsy of childhood with centrotemporal spikes (BECT) a form of idiopathic (with age-related onset) localization-related epilepsies, in which partial motor seizures occur in sleep in 70–80% of cases and interictal spikes appear only during sleep in about 30% of patients.
Other very rare conditions in which sleep plays a fundamental role in the pathogenetic epileptic process are the Landau–Kleffer syndrome (LKS) and the situation named continuous spikes and waves during sleep (CSWS).