Encephalopathy with hemi-status epilepticus during sleep or hemi-continuous spikes and waves during slow sleep syndrome: A study of 21 patients

Abstract

Our study suggests that the hemi-ESES/CSWSS syndrome has electroclinical features compatible with an epileptic encephalopathy. The most commonly used treatments were clobazam, ethosuximide, and sulthiame, alone or in combination. In refractory cases, high-dose corticosteroids were administered. Although the number of patients in this study is too low to draw definite conclusions, we consider that in children with hemi-ESES/CSWSS secondary to a unilateral lesion, surgery should be considered.

Continuous Spike-Wave in Slow Wave Sleep (CSWS) Mimicking Dementia in a 55-Year-Old Man with Intellectual Disability

“…Based on an EEG-finding and a good response to clobazam the CSWSS appears evident in our 55-year old patient; the somewhat atypical unilateral pattern of discharges found in our case has been described in the literature [5]. His specific epilepsy diagnosis is further supported by the facts that he had an acquired brain damage and that before the right diagnosis he had been on a narrow-spectrum antiepileptic medication both of which are known risk factors for CSWSS [6].…”

Electrical status epilepticus in sleep (ESES)/continuous spikes and waves during slow sleep (CSWS) syndrome in children: An electroclinical evaluation according to the EEG patterns

“…Treatment of the ESES syndrome is still controversial. In refractory cases, besides antiepileptic drugs, therapeutic alternatives such as corticosteroids, gamma globulins, the ketogenic diet, and surgery have been tried [11][12][13][14][15].…”

“…It still remains unknown if interictal discharges are the direct cause of developmental deterioration or the epiphenomenon mirroring the underlying brain pathology. Neuropsychiatric symptoms in the form of autistic-like or psychotic behaviors and more global regression are seen together with Epilepsy & Behavior 61 (2016) 107-111 epileptiform activities affecting the frontal region while there is a paroxysmal disorder that develops from the posterior temporal area and causes auditory agnosia and language deficits [10][11][12][13][14][15][16].…”

Encephalopathy with status epilepticus during sleep: Unusual EEG patterns

“…9 Several authors have described isolated cases with hemi-ESES based on the distribution of epileptiform activity during sleep [10][11][12][13][14][15] and a series of patients with hemi-ESES has recently been published. 16 Patterns of focal, bilateral asynchronic, and bilateral asymmetric ESES, slow-wave activity, and multifocal spikes or spikes and waves have also been considered, 3,7 but welldescribed cases with these EEG features during slow sleep have not been published yet.…”