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content:epileptic_encephalopathy_with_continuous_spike-waves_during_sleep [2020/02/24 09:30] – [EEG] icna | content:epileptic_encephalopathy_with_continuous_spike-waves_during_sleep [2024/03/23 07:14] – biju.hameed@gmail.com | ||
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====== Epileptic encephalopathy with continuous spike-waves during sleep ====== | ====== Epileptic encephalopathy with continuous spike-waves during sleep ====== | ||
- | <alert type=" | + | <WRAP center round info 60%> |
+ | The term Developmental and/or Epileptic Encephalopathy with spike-wave activation in sleep (D/EE-SWAS)has been proposed which, | ||
+ | </WRAP> | ||
+ | |||
+ | |||
Epileptic encephalopathy with continuous spike-waves during sleep (ECSWS)\\ | Epileptic encephalopathy with continuous spike-waves during sleep (ECSWS)\\ | ||
Epileptic encephalopathy with continuous spike-waves during slow-wave sleep (CSWS) | Epileptic encephalopathy with continuous spike-waves during slow-wave sleep (CSWS) | ||
- | * a spectrum of epileptic conditions best defined by the association of cognitive or behavioral impairment acquired during childhood and not related to another factor other than the presence of abundant interictal epileptiform discharges (IED) during sleep[(: | + | * a spectrum of epileptic conditions best defined by the association of cognitive or behavioral impairment acquired during childhood and not related to another factor other than the presence of abundant interictal epileptiform discharges (IED) during sleep[(: |
* characterised by infrequent nocturnal focal seizures, atypical absences related to secondary bilateral synchrony, negative myoclonia, and atonic and rare generalized tonic-clonic seizures. | * characterised by infrequent nocturnal focal seizures, atypical absences related to secondary bilateral synchrony, negative myoclonia, and atonic and rare generalized tonic-clonic seizures. | ||
* [[Landau-Kleffner syndrome]] (LKS) is a particular presentation where acquired aphasia is the core symptom | * [[Landau-Kleffner syndrome]] (LKS) is a particular presentation where acquired aphasia is the core symptom | ||
- | * LKS and CSWS are said to “describe the clinical epileptic syndromes seen with ESES. There is an overlap between the two syndromes in that patients with CSWS have more global regression and more problematic epilepsy than those with LKS, who had only acquired auditory agnosia and fewer seizures[(: | + | * LKS and CSWS are said to “describe the clinical epileptic syndromes seen with ESES. There is an overlap between the two syndromes in that patients with CSWS have more global regression and more problematic epilepsy than those with LKS, who had only acquired auditory agnosia and fewer seizures[(: |
- | * The EEG patterns CSWS and electrical status epilepticus during sleep (ESES) are likely equivalent terms. CSWS is used by some authors, and ESES by others[(: | + | * The EEG patterns CSWS and electrical status epilepticus during sleep (ESES) are likely equivalent terms. CSWS is used by some authors, and ESES by others[(: |
* The term " | * The term " | ||
Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. | Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. | ||
Epilepsia 1989; | Epilepsia 1989; | ||
- | * transition from [[childhood epilepsy with centro-temporal spikes]] (CECTS) into LKS and CSWS has been shown to occur particularly when there are atypical signs, high discharge rates, prolonged duration of ESES, | + | * transition from [[childhood epilepsy with centro-temporal spikes]] (CECTS) into LKS and CSWS has been shown to occur particularly when there are atypical signs, high discharge rates, prolonged duration of ESES, cognitive dysfunction, |
- | cognitive dysfunction, | + | |
+ | **Electrical status epilepticus during sleep** | ||
+ | |||
+ | Patry et al. in 1971[(: | ||
+ | characteristic of petit mal status but without any clinical accompaniment nor changes in normal sleep patterns. Lasting throughout the whole night, it subsides upon awakening. They proposed to call the phenomenon an " | ||
+ | |||
+ | ESES can be divided into symptomatic and idiopathic forms[(: | ||
+ | |||
+ | The idiopathic form was characterized by early normal development, | ||
+ | absences, negative myoclonus, exclusively diffuse epileptiform activity, cortical subatrophy on MRI, and some cognitive disturbances. | ||
+ | |||
+ | ===== ESES: Clinical features ===== | ||
+ | * acquired aphasia[(: | ||
+ | * cognitive function impairment | ||
+ | * neuropsychological impairment (behaviour problems) | ||
+ | * motor impairment (ataxia, dyspraxia, dystonia, or some unilateral deficit) | ||
+ | * all types of generalized seizures may occur | ||
+ | * improvement in seizures usually is associated with improvement in cognition followiing AED institution | ||
+ | |||
+ | |||
===== EEG ===== | ===== EEG ===== | ||
* continuous, diffuse, bilateral spike-waves during slow-wave sleep | * continuous, diffuse, bilateral spike-waves during slow-wave sleep | ||
- | * in CSWS EEG foci is predominantly in the frontotemporal or frontocentral areas, whereas in LKS the EEG foci is in the posterior temporal areas[(: | + | * in CSWS EEG foci is predominantly in the frontotemporal or frontocentral areas, whereas in LKS the EEG foci is in the posterior temporal areas[(: |
+ | * in ESES nearly continuous, irregular generalized spike–wave complexes, seen in at least 85% of the sleep record and with minimal abnormalities during wakefulness[(: | ||
+ | * the bilateral complexes in ESES represent [[content: | ||
===== Treatment ===== | ===== Treatment ===== | ||
- | * prednisolone[(: | + | * prednisolone[(: |