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content:epileptic_encephalopathy_with_continuous_spike-waves_during_sleep [2020/02/24 13:22] – [ESES: Clinical features] bijuhameed | content:epileptic_encephalopathy_with_continuous_spike-waves_during_sleep [2024/03/23 19:48] (current) – biju.hameed@gmail.com | ||
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====== Epileptic encephalopathy with continuous spike-waves during sleep ====== | ====== Epileptic encephalopathy with continuous spike-waves during sleep ====== | ||
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- | Epileptic encephalopathy with continuous spike-waves during sleep (ECSWS)\\ | ||
- | Epileptic encephalopathy with continuous spike-waves during slow-wave sleep (CSWS) | ||
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+ | The term [[content: | ||
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+ | Epileptic encephalopathy with continuous spike-waves during sleep (ECSWS)\\ | ||
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* 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. | + | * 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, |
- | 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, cognitive dysfunction, | + | |
**Electrical status epilepticus during sleep** | **Electrical status epilepticus during sleep** | ||
- | Patry et al. in 1971[(: | + | 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[(: | + | ESES can be divided into symptomatic and idiopathic forms[(: |
- | The idiopathic form was characterized by early normal development, | + | 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 ===== | ===== ESES: Clinical features ===== | ||
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* cognitive function impairment | * cognitive function impairment | ||
* neuropsychological impairment (behaviour problems) | * neuropsychological impairment (behaviour problems) | ||
* motor impairment (ataxia, dyspraxia, dystonia, or some unilateral deficit) | * motor impairment (ataxia, dyspraxia, dystonia, or some unilateral deficit) | ||
- | * all types of generalized seizures may occur, often changing over time ( secondarily generalised tonic-clonic seizures SGTC seizures, atypical absences, and negative myoclonus or hemiclonic, automotor, or focal adverse | + | * all types of generalized seizures may occur |
- | movements with vomiting, and focal attacks are seen). | + | |
* improvement in seizures usually is associated with improvement in cognition followiing AED institution | * improvement in seizures usually is associated with improvement in cognition followiing AED institution | ||
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- | ==== EEG ==== | ||
- | * nearly continuous, irregular generalized spike–wave complexes, seen in at least 85% of the sleep record and with minimal abnormalities during wakefulness[(: | ||
===== EEG ===== | ===== EEG ===== | ||
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* 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 ===== | ||
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