Early onset benign occipital susceptibility syndrome: video-EEG documentation of an illustrative case.
Early onset benign occipital susceptibility syndrome (EBOSS) is a recently delineated form of idiopathic childhood partial epilepsy, which is still not recognized by the International League Against Epilepsy as a distinct epileptic syndrome. It occurs with generally nocturnal, prolonged partial seizures, which can become a status epilepticus. The seizures are characterized by tonic eye deviation, vomiting, progressive impairment of consciousness, autonomic symptoms, frequent progression to hemiconvulsions or generalized tonic-clonic seizures. Age at onset is usually between 3 and 7 years, frequency is remarkably low (often a single seizure), and outcome is excellent. Interictal EEG shows occipital paroxysms with fixation-off sensitivity. We present the first video-EEG documentation of a typical case of EBOSS and discuss the clinical and EEG features of the case. The documentation is useful for a better definition of the syndrome and also permits a differential diagnosis with respect to other occipital forms of epilepsy and other sleep related paroxysmal disorders.
Vigevano
F
F
Neurology Division, Bambino Gesù Children's Hospital, Piazza S Onofrio 4, 00165, Rome, Italy. vigevano@opbg.net
Lispi
M L
ML
Ricci
S
S
D017668:Age of Onset; D002648:Child; D004569:Electroencephalography / Q000379:methods*; D004828:Epilepsies, Partial / Q000503:physiopathology*; D005260:Female; D006801:Humans; D009778:Occipital Lobe / Q000503:physiopathology*; D014741:Video Recording