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Eyelid myoclonia with absences: phenomenology in children
L Kent1, A Blake, W Whitehouse
1University of Birmingham, Parkview Clinic, UK.
Insights
Eyelid myoclonia with absences (EMA) in children may involve compulsive self-induction, similar to Tourette's syndrome. This challenges the notion that self-induction is always deliberate, offering new insights into EMA phenomenology.
Area of Science:
- Pediatric Neurology
- Clinical Neurophysiology
- Epileptology
Background:
- Eyelid myoclonia with absences (EMA) is an epilepsy syndrome characterized by brief absences and eyelid fluttering.
- The phenomenon of self-induction, where patients trigger their seizures, is debated in EMA.
- Understanding the phenomenology and EEG correlates of EMA is crucial for accurate diagnosis and management.
Observation:
- Six pediatric patients diagnosed with EMA were evaluated, including clinical interviews and EEG reviews.
- Five patients exhibited compulsive or tic-like symptoms associated with absence attacks.
- Two patients also presented with separate facial tics unrelated to absences.
Findings:
- Observed compulsive behaviors in EMA patients suggest a potential for non-deliberate self-induction.
- The phenomenology of compulsive self-induction in EMA may share similarities with Tourette's syndrome (TS).
- Photosensitive patients may experience self-induced seizures through eye-blinking tics, similar to TS motor symptoms.
Implications:
- The concept of self-induction in EMA may need re-evaluation to include compulsive, non-deliberate actions.
- Findings suggest a potential link between EMA, TS, and photosensitivity, warranting further investigation.
- The positive response of some 'self-inducers' to pro-convulsive dopamine antagonists used in TS supports this connection.
Abstract:
The aim of this study is to describe the phenomenology of children with 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 paediatric 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 treatment responses were in keeping with the established literature. Five of the six patients demonstrated various compulsive or tic-like symptoms including premonitory sensations, compulsive, difficult to resist urges and a sense of relief associated with the absence attacks. Separate facial tics not associated with absences were also evident in at least two children. The argument that self-induction does not occur in EMA assumes its deliberate nature, which may not necessarily be the case 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 tics and obsessive compulsive symptoms. The relationship between this argument and EEG findings in EMA would presume that the initial eye ball roll/eye closure and eye blinking (tics seen in TS) are tic-like symptoms which cause the absences 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 response of some so-called 'self-inducers' to the proconvulsive dopamine antagonists (commonly prescribed for TS) lends further support to this argument.