Chandrika Azad and Vishal Guglani

Jeavons syndrome (eyelid myoclonia with absence seizure) is a rare type of idiopathic generalized epilepsy [1]. We report a young girl who presented with this disorder after introduction of carbamazepine.

A nine year old girl, presented with two episodes of unprovoked seizures during sleep characterized by deviation of eyes and head to right and tonic clonic movement of all four limbs during sleep for two weeks before presentation. Antenatal and birth histories were uneventful. Maternal uncle had history of generalized seizures. Patient was a developmentally normal child. Physical examination was noncontributory. MRI brain showed no abnormality. Her first EEG showed generalized discharges. She was already on carbamazepine (CBZ) started by some private physician. CBZ was continued. Over next two months her academic performance deteriorated and she felt giddy on looking at television or sun. After three months, she presented with continuous eye blinking for three days. EEG showed absence status. She was diagnosed as having eyelid myoclonia with absence seizure. Status was controlled by intravenous benzodiazepines and sodium valproate. CBZ was stopped. Seizures stopped within 48 hours. She was discharged on sodium valproate and clonazepam. Only two brief episodes of eyelid myoclonia occurred in first month of starting valparin and clonazepam. The child is asymptomatic for last 15 months.

Jeavons syndrome is a generalized epileptic condition clinically characterized by eyelid myoclonia with or without absences, eye closure-induced EEG paroxysms, and photosensitivity; in addition, rare tonic–clonic seizures may also occur [1]. It is commoner in females between 2- 14 years.

CBZ though a very useful drug can unmask or aggravate various types of seizures. Idiopathic generalized epilepsies e.g. absences, tonic, atonic, tonic-clonic, myoclonic etc. are known to be aggravated by CBZ [2,3]. It can even mask or reduce the beneficial effect of valparin or phenobarbitone [3]. With the use of CBZ in patients with GTCS, absences and myoclonic jerks can appear de novo, and generalized paroxysmal discharges can appear in various focal epileptic syndromes [4]. Menon, et al. [5] have described a similar case in an adult patient, although eye blinking in that case was not continuous.

1. Striano S, Capovilla G, Sofia Z. Eyelid myoclonia with absences (Jeavons syndrome): A well-defined idiopathic generalized epilepsy syndrome or a spectrum of photosensitive conditions? Epilepsia. 2009;50:15-9.

2. Gelisse P, Genton P, Kuate C. Worsening of seizures by oxcarbamezepine in juvenile idiopathic generalized epilepsies. Epilepsia. 2004;45:1282-6.

3. Thomas P, Valton L, Genton P. Absence and myoclonic status epilepticus precipitated by antiepileptic drugs in idiopathic generalized epilepsy. Brain. 2006;129:1281-92.

4. Yang T, Liu Y, Liu L,Yan B, Zhang Q, Zhou D. Absence status epilepticus in monozygotic twins with Jeavons syndrome. Epileptic Disord. 2008;10:227-30.