Eyelid myoclonia with absences (EMA) and juvenile myoclonic epilepsy (JME) are two separate epileptic syndromes included in the new classification of epilepsies and epileptic syndromes by ILAE in 2001. Both are idiopathic generalized epilepsies with their clinical onset in the first two decades. EMA is characterized by eyelid myoclonia associated with absences and photosensitivity. Self-induced seizures are frequently seen in EMA. It can be associated with mildly mental retardation and resistance to treatment. JME includes three types of generalized seizures: typical absences, myoclonic jerks and generalized tonic-clonic seizures. The myoclonic jerks occur almost exclusively on awakening, involve preferently the upper extremities, may rarely affect the lower extremities or the entire body. More severe attacks may be accompanied by a fall. The myoclonic jerks occur rarely in EMA. They are usually mild and are freqently restricted to the upper extremities. Generalized tonic-clonic seizures, photosensitivity and generalized polyspike-wave discharges provoked by eye closure are features of both epileptic syndromes. In this study, we describe four female patients with eyelid myoclonia associated with absences, myoclonic jerks causing falling down and rare generalized tonic-clonic seizures. All patients had good school performance and total seizure control under sodium valproate treatment. Their EEGs show generalized polyspike-wave discharges with a frequency of 3.5-6Hz always appearing a few seconds after eye closure and photoparoxysmal response. These patients show the characterictics of both epileptic syndromes. It is clinically important to make a syndromic diagnosis for an optimum advise on treatment, lifestyle restrictions and prognosis. In this study, we have gathered evidence that EMA and JME are dynamic syndromes that tend to evolve into one another.
Photosensitivity is a typical feature of photosensitive epilepsy which is usually considered a form of idiopathic generalized epilepsy. Partial seizures featuring visual symptoms are rarely reported in photosensitive epilepsy. In this study, we describe 13 neurologically normal patients in whom daytime seizures were always induced by television and began with elementary visual hallucinations, followed frequently by vomiting, headache and then secondary generalization. Three patients additionally reported nocturnal seizures, which have not been described in previous studies. Two of these latter patients had generalized tonic-clonic seizures, the other always awoke from sleep and could describe typical visual hallucinations at the beginning of the seizure. EEG features included normal background activity and occipital spikes or spike-waves in all but two patients. Eight patients also showed generalized epileptiform activity during intermittent photic stimulation. Seizure frequency was low in all. Apart from two patients, who refused treatment, all patients received antiepileptic drugs. Only one patient continued to have rare seizures after treatment; in the others seizure control was achieved with monotherapy. We conclude that reflex occipital lobe epilepsy is an idiopathic form of the benign partial epilepsies, which may overlap with one another.
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