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An unusual case of benign reflex myoclonic epilepsy of infancy
1Department of Paediatric Neurology, The Children's Hospital, Dublin, Eire.
Insights
A rare case of reflex myoclonic epilepsy in infancy, triggered by head tapping, was successfully treated with sodium valproate. This distinct epilepsy syndrome shows a good prognosis in infants.
Area of Science:
- Pediatrics
- Neurology
- Epileptology
Background:
- Reflex myoclonic epilepsy in infancy is a rare, age-dependent epileptic syndrome.
- Idiopathic generalized epilepsy syndromes can present with varied clinical manifestations.
- Understanding specific triggers is crucial for diagnosis and management.
Observation:
- A previously healthy one-year-old boy experienced myoclonus exclusively upon tactile head stimulation.
- No developmental regression was noted.
- Electroencephalogram (EEG) showed generalized polyspike wave activity.
Findings:
- Sodium valproate effectively ceased myoclonic episodes within one week.
- The infant remained seizure-free at 18 months with normalized EEG.
- This case highlights non-progressive reflex myoclonic epilepsy of infancy triggered by specific tactile stimuli.
Implications:
- Reflex myoclonic epilepsy of infancy is a distinct subtype with a favorable prognosis.
- Early diagnosis and targeted treatment can lead to excellent outcomes.
- This case expands the understanding of reflex epilepsy triggers in infants.
Abstract:
A previously healthy one-year-old boy, the youngest child of unrelated parents, presented with a four-week history of episodes of myoclonus triggered only by tactile stimulation to his head. There had been no loss of developmental skills. The electroencephalogram (EEG) revealed generalised polyspike wave activity both with and without clinical correlate. The infant was started on sodium valproate, which resulted in cessation of the myoclonic episodes one week after starting therapy. At subsequent follow-up (at 18 months) the infant was seizure free and a repeat EEG was normal. This case of non-progressive reflex myoclonic epilepsy of infancy triggered only by head tapping (and not by acoustic stimuli) is an extremely rare phenomenon. Reflex myoclonic epilepsy of infancy represents a distinct subtype of myoclonic epilepsy in infancy. It should be considered as an age-dependent idiopathic generalised epileptic syndrome with an apparently good prognosis.
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