Continuous spike-waves and dementia in childhood epilepsy with occipital paroxysms

Tenembaum, Sílvia; Deonna, Thierry; Fejerman, Natalio; Medina, Carlos Alberto Hernández; Ingvar-Maeder, Malin; Gubser-Mercati, Daniéle

doi:10.1016/s0896-6974(97)90006-2

Cited by 26 publications

(23 citation statements)

References 33 publications

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“…This patient had severe cognitive deterioration associated with continuous spike‐waves during slow‐wave sleep with a good response to intravenous gamma globulin, and an excellent evolution on cognitive abilities. This patient was previously reported by our group (Fejerman et al, 2001; Tenembaum et al, 1997). There was no relationship between the antiepileptic drug and both the nonbenign and atypical evolution of epilepsy.…”

Section: Resultssupporting

confidence: 81%

Childhood occipital epilepsy of Gastaut: A study of 33 patients

2007

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SUMMARYPurpose: To characterize the electroclinical features and evolution of childhood occipital epilepsy of Gastaut (COE-G). Methods: Children with electroclinical criteria of COE-G were retrospectively identified and followed-up clinically, and with sleep and awake EEGs between 1990 and 2007. Results: We identified 33 patients with COE-G. In the same length of time, 201 children with Panayiotopoulos syndrome and 410 children with benign childhood epilepsy with centrotemporal spikes were registered. COE-G had a peak age at onset of 8.5 years. Visual manifestations were the most common ictal event. Ictal deviation of the eyes was frequent. Approximately half of the patients had migraine-like symptoms. In all patients the seizures occurred while awake, and 11 also had seizures during sleep. The majority of the patients had occipital spike-wave discharges when the eyes were closed that disappeared or attenuated when the eyes were opened. Prognosis was excellent in 80% of the cases. Conclusion: This study confirms the existence of COE-G, a rare but well-defined syndrome within the group of idiopathic focal epilepsies in childhood.

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Section: Resultssupporting

confidence: 81%

Childhood occipital epilepsy of Gastaut: A study of 33 patients

2007

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“…Caraballo et al . suggested that the atypical evolution of COE‐G may be due to a thalamocortical mechanism and that, when the basal ganglia are activated, absence seizures may be triggered . Wakamoto et al ., in 2011 , found a high rate of patients with GTCS associated with COE‐G; they hypothesized that a delay in AED treatment led to secondarily generalized seizures or that all types of motor seizures reported could have been inappropriately described as GTCS.…”

Section: Discussionmentioning

confidence: 99%

Clinical dissection of childhood occipital epilepsy of Gastaut and prognostic implication

Verrotti

Laino

Rinaldi

et al. 2015

Euro J of Neurology

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“…Anecdotal cases treated successfully with intravenous immunoglobulins have been reported, but there are not sufficient data to consider it as a possible treatment (Tenembaum et al., 1997; Mikati et al., 2002; Arts et al., 2009).…”

Section: Discussionmentioning

confidence: 99%

Sulthiame add‐on therapy in children with focal epilepsies associated with encephalopathy related to electrical status epilepticus during slow sleep (ESES)

et al. 2012

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Summary Purpose: In children with symptomatic or idiopathic focal epilepsies, their disease may evolve into an epileptic encephalopathy related to continuous spike and wave during slow sleep (CSWS) or electrical status epilepticus during slow sleep (ESES). ESES syndrome implies serious risks of neuropsychologic impairment, and its treatment has frequently been disappointing. The aim of this study is to present our experience using sulthiame as add‐on treatment in 53 patients with ESES syndrome that was refractory to other antiepileptic drugs (AEDs). Methods: Neurologic examinations, cerebral magnetic resonance imaging (MRI), and repeated prolonged sleep electroencephalography (EEG) studies were performed in all cases. Data about school achievements and or neuropsychological evaluations were obtained repeatedly during the follow‐up of 1.5–16 years. Sulthiame was added in doses ranging between 5 and 30 mg/kg/day. Key Findings: Since add‐on of sulthiame, 10 of 28 patients in the symptomatic group became seizure free: 4 patients with normal EEG studies and 6 with residual spikes. Nine of 28 patients showed a significant reduction in number of seizures and presented spikes but no ESES on EEG. The other nine cases showed neither clinical nor EEG improvement. A striking result was that 3 of 11 children with unilateral polymicrogyria and ESES syndrome became seizure free, and in another six a significant improvement in frequency of seizures and in EEG abnormalities seemed to be related to the add‐on of sulthiame. Twenty‐one of the 25 patients in the idiopathic group became seizure free and without ESES in <3 months after add on of sulthiame. In two of the patients the changes were seen in a few days. Significance: We understand that sulthiame may be effective as add‐on treatment in children with ESES syndrome.

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Continuous spike-waves and dementia in childhood epilepsy with occipital paroxysms

Cited by 26 publications

References 33 publications

Childhood occipital epilepsy of Gastaut: A study of 33 patients

Childhood occipital epilepsy of Gastaut: A study of 33 patients

Clinical dissection of childhood occipital epilepsy of Gastaut and prognostic implication

Sulthiame add‐on therapy in children with focal epilepsies associated with encephalopathy related to electrical status epilepticus during slow sleep (ESES)

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