content:benign_non-familial_neonatal_epilepsy

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content:benign_non-familial_neonatal_epilepsy [2020/02/15 18:15] – [Pathophysiology] icnacontent:benign_non-familial_neonatal_epilepsy [2020/02/23 20:31] – icna
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 Benign neonatal seizure (nonfamilial) is a benign syndrome characterized by clonic seizures that begin around the fifth day of life and may recur during the following 2 to 3 days. Benign neonatal seizure (nonfamilial) is a benign syndrome characterized by clonic seizures that begin around the fifth day of life and may recur during the following 2 to 3 days.
  
-===== History ===== +===== History & terminology ===== 
-  * Declan et al(1977) first reported a neonatal convulsive disorder of unknown etiology that occurs around the fifth day of life and associated with a favorable outcome.+  * Dehan et al 1977[(:cite:931532>{{pubmed>long:931532}})] first reported a neonatal convulsive disorder of unknown etiology that occurs around the fifth day of life and associated with a favorable outcome. 
 +  * Prior & Macourt (1981)[(:cite:7305412>{{pubmed>long:7305412}})] described the features in 90 infants with the condition and coined the term "fifth day fits"
   * In 1989, the ILAE Commission on Classification and Terminology proposed the term "benign neonatal seizures"   * In 1989, the ILAE Commission on Classification and Terminology proposed the term "benign neonatal seizures"
-  * In the updated ILAE 2017 classification it is categorized under //Conditions with epileptic seizures that are traditionally not diagnosed as a form of epilepsy per se//.+  * The [[content:epilepsy_classification|updated ILAE 2017 classification]] categorizes it under //Conditions with epileptic seizures that are traditionally not diagnosed as a form of epilepsy per se//.
 ===== Genetics ===== ===== Genetics =====
   * Claes et al 2004 reported three de novo mutations in KCNQ2 in 4 patients with benign neonatal seizures without a family history (Claes et al 2004).    * Claes et al 2004 reported three de novo mutations in KCNQ2 in 4 patients with benign neonatal seizures without a family history (Claes et al 2004). 
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 ===== Clinical features ===== ===== Clinical features =====
   * seizures are mainly clonic   * seizures are mainly clonic
 +  * median duration is around 20hrs (status epilepticus)[(:cite:panayiotopoulos2005>)]
   * onset is around fifth day of life   * onset is around fifth day of life
 ===== EEG ===== ===== EEG =====
   * ictal EEG usually shows localised spikes   * ictal EEG usually shows localised spikes
-  * interictal EEG usually shows theta pointu alternant+  * interictal EEG usually shows theta pointu alternant. It may also show focal or multifocal, non-specific abnormalities or a discontinuous pattern or it may be normal in ~10%.
 ===== Pathophysiology ===== ===== Pathophysiology =====
   * probably environmental   * probably environmental
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   * [[Benign infantile epilepsy]]   * [[Benign infantile epilepsy]]
 ===== Prognosis ===== ===== Prognosis =====
-* risk of subsequent seizures is practically nil (o.5%)[(:cite:panayiotopoulos2005>Panayiotopoulos CP. (2005). The Epilepsies: Seizures, Syndromes and Management. Oxfordshire (UK): Bladon Medical Publishing)]+  * excellent with risk of subsequent seizures practically nil(0.5%)[(:cite:panayiotopoulos2005>Panayiotopoulos CP. (2005). **The Epilepsies: Seizures, Syndromes and Management**. Oxfordshire (UK): Bladon Medical Publishing)] 
 +  * normal neurodevelopmental outcome
 ===== References ===== ===== References =====
 ~~REFNOTES~~ ~~REFNOTES~~
 +{{tag>neonatal_seizures}}
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