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Comparative Study
. 2005 Jul;65(3):161-8.
doi: 10.1016/j.eplepsyres.2005.05.009.

Neonatal seizures with tonic clonic sequences and poor developmental outcome

Bernhard Schmitt  1 , Gabriele Wohlrab, Thomas Sander, Ortrud K Steinlein, Beatrice Latal Hajnal
Affiliations
Comparative Study

Neonatal seizures with tonic clonic sequences and poor developmental outcome

Bernhard Schmitt et al. Epilepsy Res. 2005 Jul.

Abstract

Seizures consisting of a tonic followed by a clonic phase have rarely been described in neonates and are not included in the current classifications of neonatal seizures. Our video archive of 105 neonates with seizures or suspected seizures revealed six neonates with such tonic clonic or tonic myoclonic sequences. Two of those neonates had pyridoxine dependent seizures. The other four neonates had drug refractory seizures and demonstrated similarities in electro-clinical pattern, clinical course and outcome. Their seizures started with tonic posturing and after 10-20s tonic posturing was superimposed by focal or multifocal cloni or myocloni. Ictal EEG started with voltage attenuation followed by bilateral or alternating focal epileptic discharges. The interictal EEG was abnormal. One child died, while the other three children became seizure free but had severe motor delay and mental retardation. In one of those three children, a de novo missense mutation was detected in the voltage gated potassium channel gene KCNQ2, indicating a genetic relationship between drug refractory neonatal seizures of unknown etiology with tonic clonic or myoclonic sequences and the well-known syndrome of benign familial neonatal convulsions (BFNC).

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