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Diagnostic criteria for Dravet syndrome

Diagnostic criteria for Dravet syndrome
  Mandatory Alerts Exclusionary
Seizures
  • Recurrent focal clonic (hemiclonic) febrile and afebrile seizures (which often alternate sides from seizure to seizure), focal to bilateral tonic-clonic, and/or generalized clonic seizures
  • No history of prolonged seizures (>10 min)
  • Lack of fever sensitivity as a seizure trigger
  • Epileptic spasms
  • Early infantile SCN1A DEE
EEG  
  • Normal EEG background without interictal discharges after age 2 years
  
Age at onset
  • 1 to 20 months
  • 1 to 2 months or 15 to 20 months
  
Development at onset  
  • Developmental delay at seizure onset
  
Neurologic examination  
  • Focal neurologic findings (other than Todd paresis)
  
Imaging    
  • MRI showing a causal focal lesion
Other testing (eg, genetics)  
  • Lack of pathogenic SCN1A or other causal variant
  
Course of illness
  • Drug-resistant epilepsy
  • Intellectual disability
  • Good efficacy with prophylactic sodium-channel agents including carbamazepine, oxcarbazepine, and phenytoin
  
Is MRI or ictal EEG required for diagnosis?
  • An MRI is not required for diagnosis but is highly recommended to exclude other causes
  • An ictal EEG is not required for diagnosis
Possible evolving syndrome: In a child <12 months who presents with a prolonged hemiclonic or bilateral tonic-clonic seizure with fever, and no other underlying cause, the possibility of Dravet syndrome should be considered. Further convulsive seizures (often with fever, and if prolonged or hemiclonic) would allow more definitive diagnosis of Dravet syndrome. A diagnosis would be further supported by the finding of a pathogenic SCN1A variant.
Syndrome without laboratory confirmation: In resource-limited regions, Dravet syndrome can be diagnosed in children without alerts who meet all other clinical mandatory and exclusionary criteria, without EEG, MRI, and genetic testing
EEG: electroencephalography; MRI: magnetic resonance imaging.
From: Zuberi SM, Wirrell E, Yozawitz E, et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63:1349. Copyright © 2022 The Authors. Available at: https://onlinelibrary.wiley.com/doi/10.1111/epi.17239 (Accessed on November 14, 2022). Reproduced under the terms of the Creative Commons Attribution License 4.0.
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