Overlap cases of eyelid myoclonia with absences and juvenile myoclonic epilepsy

Abstract

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.

Eyelid myoclonia seizures in adults: An alternate look at the syndrome paradox

“…As demonstrated in our study in the second and third groups, EMs can be regarded as a type of seizure in many different epileptic syndromes and even in some symptomatic epilepsy [9][10][11]21,46,47].…”

“…Hence, EMA has been included with other types of idiopathic generalized epilepsies (IGEs) [10,11,[19][20][21][22].…”

Eyelid fluttering, typical EEG pattern, and impaired intellectual function: A homogeneous epileptic condition among the patients presenting with eyelid myoclonia

“…Photosensitivity is almost invariably present, although its clinical and EEG expressivity usually decreases with age and can be modified by antiepileptic drugs (AEDs) (Appleton et al., 1993; Giannakodimos & Panayiotopoulos, 1996; Panayiotopoulos et al., 1996; Incorpora et al., 2002; Striano et al., 2002; Sevgi et al., 2007). In addition, during their life, patients with EMA may experience generalized tonic–clonic seizures, either spontaneous or photo‐induced (Giannakodimos & Panayiotopoulos, 1996; Kent et al., 1998; Baykan‐Kurt et al., 1999; Wakamoto et al., 1999; Kimura, 2000; Striano et al., 2002; Burneo et al., 2004; Covanis, 2005; Destina Yalcin et al., 2006; Sevgi Demirci & Saygi, 2006). Neurologic and magnetic resonance imaging (MRI) examinations are unremarkable in most cases, although patients with borderline intellectual functioning or frank mental retardation have been described (De Marco, 1989; Striano et al., 2002; Covanis, 2005; Ogura et al., 2005; Sevgi Demirci & Saygi, 2006; Termine et al., 2006; Joshi & Patrik, 2007).…”

A pilot trial of levetiracetam in eyelid myoclonia with absences (Jeavons syndrome)

“…Eyelid myoclonia with absences (EMA) or Jeavons syndrome is a generalized form of epilepsy first described by Jeavons in 1977 (Jeavons, 1977). It is clinically characterized by the triad of eyelid myoclonia with or without absences, eye closure‐induced seizures with EEG paroxysms, and photosensitivity (Appleton et al, 1993; Giannakodimos & Panayiotopoulos, 1996; Panayiotopoulos et al, 1996; Incorpora et al, 2002; Striano et al, 2002; Destina Yalcin et al, 2006; Sevgi et al, 2007). Typical onset of EMA is in childhood, with a peak around 8 years of age (range: 2–14) and a preponderance among girls.…”

“…C 2008 International League Against Epilepsy absences, eye closure-induced seizures with EEG paroxysms, and photosensitivity (Appleton et al, 1993;Panayiotopoulos et al, 1996;Incorpora et al, 2002;Striano et al, 2002;Destina Yalcin et al, 2006;Sevgi et al, 2007). Typical onset of EMA is in childhood, with a peak around 8 years of age (range: 2-14) and a preponderance among girls.…”