Eyelid myoclonia with absences (Jeavons syndrome): a well-defined idiopathic generalized epilepsy syndrome or a spectrum of photosensitive conditions?
Eyelid myoclonia with absences (EMA), or Jeavons syndrome, is a generalized epileptic condition clinically characterized by eyelid myoclonia (EM) with or without absences, eye closure-induced electroencephalography (EEG) paroxysms, and photosensitivity; in addition, rare tonic-clonic seizures may also occur. Although first described in 1977 and widely reported by several authors within the last few years, EMA has not been yet recognized as a definite epileptic syndrome. However, when strict criteria are applied to the diagnosis, EMA appears to be a distinctive condition that could be considered a myoclonic epileptic syndrome, with myoclonia limited to the eyelids, rather than an epileptic syndrome with absences.
Striano
Salvatore
S
Epilepsy Center, Department of Neurological Sciences, Federico II University, Napoli, Italy. sstriano@libero.it
Capovilla
Giuseppe
G
Sofia
Vito
V
Romeo
Antonino
A
Rubboli
Guido
G
Striano
Pasquale
P
Trenité
Dorothée Kasteleijn-Nolst
DK
D003937:Diagnosis, Differential; D004569:Electroencephalography; D004832:Epilepsy, Absence / Q000150:complications*; D004829:Epilepsy, Generalized / Q000175:diagnosis*; D020195:Epilepsy, Reflex / Q000175:diagnosis*; D005143:Eyelids / Q000503:physiopathology*; D006801:Humans; D009207:Myoclonus / Q000150:complications* / Q000503:physiopathology*