Sturge-Weber Disease - Neurophysiological Evaluation of a Case with Secondary Epileptogenesis, Successfully Treated with Lobe-Ectomy

The epilepsy of Sturge-Weber syndrome: Clinical features and treatment in 23 patients

“…The commonest presentation is with partial motor seizures, contralateral to the side of the pial angioma and often of long duration. It has been noted that apparently healthy brain areas remote from the lesion could produce epi!eptic discharges (12) and removal of such a focus was associated with significant, although temporary, improvement in one case (16). The present series indicates more variability as six children had a relatively late onset of seizures (4.5 to 1 1 years).…”

“…In reported cases (10,12) as well as in personal cases (cases 16 and 17) an abrupt deterioration was clearly correlated temporally to worsening of seizures and of EEG abnormalities. The epileptic activity itself may be in part responsible.…”

“…The epileptic activity itself may be in part responsible. In reported cases (10,12) as well as in personal cases (cases 16 and 17) an abrupt deterioration was clearly correlated temporally to worsening of seizures and of EEG abnormalities. Bilateral diffusion of EEG paroxysms and the occurrence of secondary epileptogenesis in the contralateral hemisphere could account for widespread brain dysfunction (12).…”

“…The relationship between the angioma even when unilateral and epileptogenesis is not entirely clear. It has been noted that apparently healthy brain areas remote from the lesion could produce epi!eptic discharges (12) and removal of such a focus was associated with significant, although temporary, improvement in one case (16).…”

Secondary bilateral synchrony in unilateral pial angiomatosis: successful surgical treatment.

“…- 13 The differentiation between primary and secondary bilateral synchrony is difficult because patients with localised cortical foci can have predominantly generalised seizures. [14][15][16][17][18][19][20][21][22][23] The following arguments can help separate secondary from primary bilateral synchrony. (1) The appearance of the spike-wave complexes may be slower and less regular in secondary than in primary bilateral synchrony.9 However, localised frontal stimulation in epileptic patients can induce classical 3 Hz regular discharges.14 15 (2) Intracarotid injection of amylobarbitone and pentilenetetrazol24 -26 or the endovenous injection of thiopental20 have not gained wide acceptance.…”