content:dravet_syndrome

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content:dravet_syndrome [2020/02/23 19:50] – [History & Clinical Manifestations] icnacontent:dravet_syndrome [2020/02/23 19:55] – [Epilepsy surgery] icna
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   * localized findings are usually seated in the central areas, bilaterally, synchronous or asynchronous, and in the vertex, and may spread to the entire hemisphere but can be also recorded in the posterior regions, occipital or temporal.   * localized findings are usually seated in the central areas, bilaterally, synchronous or asynchronous, and in the vertex, and may spread to the entire hemisphere but can be also recorded in the posterior regions, occipital or temporal.
   * The EEGs contain more generalized paroxysms when myoclonic jerks are present. The relationship between the seat of the interictal paroxysms and the ictal discharges are not always clear.   * The EEGs contain more generalized paroxysms when myoclonic jerks are present. The relationship between the seat of the interictal paroxysms and the ictal discharges are not always clear.
-  * The response to **Hyperventilation:** is variable.+  * **Hyperventilation:** variable response
   * **Eye closure:** The eye closure may facilitate the occurrence of localized and generalized abnormalities.   * **Eye closure:** The eye closure may facilitate the occurrence of localized and generalized abnormalities.
   * **Sleep:** Sleep is usually well structured with physiological patterns and cyclic organization, except when several seizures occur during the night. The paroxysmal, generalized as well as localized, activities are enhanced or appear.   * **Sleep:** Sleep is usually well structured with physiological patterns and cyclic organization, except when several seizures occur during the night. The paroxysmal, generalized as well as localized, activities are enhanced or appear.
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 ===== Epilepsy surgery ===== ===== Epilepsy surgery =====
 Andrade et al (2010)[(:cite:19919661>{{pubmed>19919661}})] reported two adults aged 19&34 with Dravet syndrome who were treated with thalamic deep brain stimulation (DBS) , where the 19yr old showed marked improvement over a 10yr follow up, while the other did not.Small case series have also reported that palliative epilepsy surgery including Vagal Nerve Stimulation (VNS) and corpus callosotomy (CC) can be effective at reducing seizure frequency in Dravet syndrome[(:cite:27465677>{{pubmed>long:27465677}})]. Andrade et al (2010)[(:cite:19919661>{{pubmed>19919661}})] reported two adults aged 19&34 with Dravet syndrome who were treated with thalamic deep brain stimulation (DBS) , where the 19yr old showed marked improvement over a 10yr follow up, while the other did not.Small case series have also reported that palliative epilepsy surgery including Vagal Nerve Stimulation (VNS) and corpus callosotomy (CC) can be effective at reducing seizure frequency in Dravet syndrome[(:cite:27465677>{{pubmed>long:27465677}})].
 +
 +The presumed topography of the epileptogenic areas involves preferentially the mesial frontal lobe, the central area, sometimes the parietal and, even, the occipital lobes. Few interictal foci are localized in the temporal area. Surprisingly a hippocampal sclerosis has not been shown in the MRI of these patients who had prolonged and repeated severe FS[(:ref:todo)].
 ===== Cannabidiol ===== ===== Cannabidiol =====
   * Clinical trials show that cannabidiol reduces the number of convulsive and non-convulsive seizures when compared with usual care[(:cite:28813226>{{pubmed>28813226}})][(:cite:32040850>{{pubmed>32040850}})].   * Clinical trials show that cannabidiol reduces the number of convulsive and non-convulsive seizures when compared with usual care[(:cite:28813226>{{pubmed>28813226}})][(:cite:32040850>{{pubmed>32040850}})].
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 There is a wide variation among the phenotypes in these conditions and is possibly due to the strong influence of the genetic background. There is a wide variation among the phenotypes in these conditions and is possibly due to the strong influence of the genetic background.
  
-===== Classification ===== +===== References ===== 
-Dravet syndrome is classified under Electroclinical syndromes in the Revised ILAE Classification and Terminology (2010). In the majority of cases, no etiology has been found. A mitochondrial cytopathy has been reported in rare cases.+~~REFNOTES~~ 
  
-The presumed topography of the epileptogenic areas involves preferentially the mesial frontal lobe, the central area, sometimes the parietal and, even, the occipital lobes. Few interictal foci are localized in the temporal area. Surprisingly a hippocampal sclerosis has not been shown in the MRI of these patients who had prolonged and repeated severe FS. 
  
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