Recurrent aphasia with subclinical bioelectric status epilepticus during sleep

Abstract

We present a six years follow up of a 6 1/2 year-old boy with recurrent aphasia, sporadic emotional regression and a convulsive disorder. The electroencephalogram reading during sleep showed continuous, generalized, hypersynchronous activity without clinical evidence of seizure ("subclinical bioelectric status epilepticus"). Clinical, laboratory and instrumental data revealed no evidence of a morphological lesion of the brain, either space-occupying, inflammatory, degenerative, or autoimmune. Remissions occured with and without anticonvulsant and steroid therapy. The hypothetical location, type, and etiology of the process are discussed. The syndrome appears to represent a functional disorganisation of speech-controlling areas of the brain.

Continuous spike-waves during slow waves sleep: a clinical and electroencephalografic study in fifteen children

“…Eight out of 18 patients reported by Tassinari et al had "encephalopathy" before the onset of the CSWSS, like 10 of our 15 patients 21 . Specific etiologies have not been completely defined but include mainly birth asphyxia, like 7 of our 15 patients, as well as meningitis, encephalopathy of unknown origin, congenital cytomegalovirus infection and cryptogenic category 1,8,12,16 .…”

“…In the first condition, there is an intense sleep activation of interictal abnormalities, however, it never reaches the 85% threshold required for the diagnosis of CSWSS 1,12 . In LKS there is activation of paroxysmal abnormalities during sleep onset and during each REM period, a distinguishing feature of CSWSS 12 . In LGS the SWC index is about 60% during the NREM sleep.…”

“…CSWSS occurs with a male to female ratio of 2:1. Onset appears to be age-related, occurring between the ages of 5 to 12 years 5,12,15,16 . In our patients we first recorded the CSWSS between the ages of 4 and 13 (mean 6.2).…”

“…However, cases have been described with discharges relatively focal in distribution, like 9 of our 15 cases, or characterized by nearly continuous diffuse bisynchronous sharp waves and not SWC, a controversy that the literature has called generalized SWC 1,[11][12][13][14][16][17][18] . These features support the assumption that the pathophysiological background of CSWSS may be associated to the reticular system, giving rise to paroxysmal bilateral synchronous SW, suggesting that CSWSS pattern is of a focal, rather than a generalized nature 1 .…”

“…This entity is characterized by spike-and-wave complexes (SWC) that occurs most continuously (>85%) during slow or non-rapid-eye movement (NREM) sleep. The close relationship between CSWSS and Landau-Kleffner syndrome (LKS), as well as with the benign childhood epilepsy with centrotemporal spikes (BECTS), is suggested by several reports [2][3][4][5][6][7][8][9][10][11][12] . It is considered a rare syndrome, has received considerable attention in Europe and has a few reports from North America [13][14][15][16][17][18][19][20][21][22] .…”