EYELID MYOCLONIA WITH ABSENCES - PHENOMENOLOGY IN CHILDREN

Citation
L. Kent et al., EYELID MYOCLONIA WITH ABSENCES - PHENOMENOLOGY IN CHILDREN, Seizure (London), 7(3), 1998, pp. 193-199
Citations number
11
Categorie Soggetti
Neurosciences,"Clinical Neurology
Journal title
ISSN journal
10591311
Volume
7
Issue
3
Year of publication
1998
Pages
193 - 199
Database
ISI
SICI code
1059-1311(1998)7:3<193:EMWA-P>2.0.ZU;2-I
Abstract
The aim of this study is to describe the phenomenology of children wit h eyelid myoclonia with absences (EMA) in relation to EEG findings and diagnosis, with particular reference to the issue of self-induction. Case notes of all children with a diagnosis of EMA within a regional p aediatric neurology clinic were identified. Each child was interviewed by a paediatric neurologist with or without a child psychiatrist and their EEG reviewed by a clinical neurophysiologist. Six patients (five female, one male) were identified. Clinical and EEG features and trea tment responses were in keeping with the established literature. Five of the six patients demonstrated various compulsive or tie-like sympto ms including premonitory sensations, compulsive, difficult to resist u rges and a sense of relief associated with the absence attacks. Separa te facial ties not associated with absences were also evident in at le ast two children. The argument that self-induction does not occur in E MA assumes its deliberate nature, which may not necessarily be the cas e for a proportion of these children. Compulsive 'self-induction' may be similar to the phenomenology described in Tourette's syndrome (TS) where individuals experience motor and vocal ties and obsessive compul sive symptoms. The relationship between this argument and EEG findings in EMA would presume that the initial eye ball roll/eye closure and e ye blinking (tics seen in TS) are tie-like symptoms which cause the ab sences and discharges of EMA in those who are photosensitive. In this model the self-induction of discharges in an individual may or may not be deliberate, or even go unnoticed by the patient. The beneficial re sponse of some so-called 'self-inducers' to the proconvulsive dopamine antagonists (commonly prescribed for TS) lends further support to thi s argument.