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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.
18 - Epileptic and non-epileptic periodic motor phenomena in children with encephalopathy
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- By Giuseppe Gobbi, Infancy and Childhood Neuropsychiatry Service, Maggiore C.A. Pizzard Hospital, Bologna, Italy, Antonella Pini, Infancy and Childhood Neuropsychiatry Service, Maggiore C.A. Pizzard Hospital, Bologna, Italy, Lucia Fusco, 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 307-318
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- Chapter
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Summary
Introduction
Paroxysmal epileptic motor phenomena occurring in the first year of life in symptomatic encephalopathies (hypoxic–ischemic, metabolic or malformative) are frequently constituted by spasms. Apart from the West syndrome, epileptic spasms are characteristic of some specific infantile epileptic syndromes such as Ohtahara syndrome (Ohtahara, 1978), early myoclonic epileptic encephalopathy (Aicardi & Goutires, 1978), tuberous sclerosis complex and Aicardi disease. They may also occur in neurofibromatosis and in other symptomatic encephalopathies of different origin (Commission on Pediatric Epilepsy of the ILAE, 1992; Roger & Dulac, 1994). In these syndromes epileptic spasms tend to recur in clusters. Recently it has been established that epileptic spasms may be present beyond infancy (Gobbi et al., 1987; Commission on Paediatric Epilepsy of the ILAE, 1992; Roger & Dulac, 1994; Talwar et al., 1995). Among these ‘non-age-related’ epileptic spasms, a particular type named periodic spasms has been described and their electroclinical characteristics have been detailed (Gobbi et al., 1987; Bednarek et al., 1998). The term ‘periodic spasms’ has been chosen to emphasize one of the most striking characteristics of these spasms: their repetition into the cluster in an almost periodic sequence at rather regular intervals. The most important aspect of periodic spasms is that the whole cluster of spasms has to be considered as a single, complicated partial seizure, with a particular type of secondary generalization, and not simply as the result of a long-lasting series of seizures (the spasms), which repeat in a periodic sequence (Gobbi et al., 1987). Finally, periodic spasms may be very polymorphous and their clinical expression may be very subtle or suggestive of a movement disorder. As a consequence they may be missed or misdiagnosed.