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1
Neonatal Epileptic Syndromes

Neonatal epileptic seizures  - Demography 

High in preterm infants. Up to 10%. 

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Neonatal Epileptic Syndromes

Benign familial neonatal seizures  - Demography 

Rare.

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Neonatal Epileptic Syndromes

Benign neonatal seizures  - Demography 

 7% of all neonatal seizures 

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Neonatal Epileptic Syndromes

Early myoclonic encephalopathy  - Demography 

 Rare. Boys and Girls are equally effective. 

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Neonatal Epileptic Syndromes

Early infantile epileptic encephalopathy with suppression burst (Ohtahara syndrome) - Demography 

 Rare. Slight male predominance. 

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Neonatal Epileptic Syndromes

Neonatal epileptic seizures  - Age range of onset

80% occur in the first 1 or 2 days during the first week of life. 

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Neonatal Epileptic Syndromes

Benign familial neonatal seizures  - Age range of onset

Seizures mainly on the second or third day of life 

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Neonatal Epileptic Syndromes

Benign neonatal seizures  - Age range of onset

 Usually between days four and six. This syndrome is synonym with fifth day fits. Boys are affected slightly more than girls

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Neonatal Epileptic Syndromes

Early myoclonic encephalopathy  - Age range of onset

 Usually starts in the first days of life; sometimes immediately after birth. Majority starts before 10 days of age.

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Neonatal Epileptic Syndromes

Early infantile epileptic encephalopathy with suppression burst (Ohtahara syndrome) - Age range of onset

 Onset is mainly around the first 10 days of life; sometimes within the uterus or up to three months after birth.

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Neonatal Epileptic Syndromes

Neonatal epileptic seizures  -  Semiology 

Usually subtle; and difficult to recognize from normal behavior patterns. Seizures are brief and repetitive. Frequent autonomic manifestations such as changing heart rate or respiratory rate

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Neonatal Epileptic Syndromes

Benign familial neonatal seizures  -  Semiology 

 Occur in otherwise normal neonates. Seizures are brief – 1-2 min; 20-30/day. Usually start with tonic motor activity; posturing and apnea

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Neonatal Epileptic Syndromes

Benign neonatal seizures  -  Semiology 

 Repetitive lengthy seizure that constitutes a clonic status epilepticus which occurs in a otherwise normal full-term neonate. The median time is about 20 hours. Tonic seizures are incompatible with this syndrome

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Neonatal Epileptic Syndromes

Early myoclonic encephalopathy  -  Semiology 

 Triad of intractable seizures: myoclonus; followed by simple focal seizures; followed later by tonic epileptic spasms. Erratic (shifting from one part of the body to another in the random fashion) myoclonus that are nearly continuous and may affect a finger; corner of the mouth; toe; etc.

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Neonatal Epileptic Syndromes

Early infantile epileptic encephalopathy with suppression burst (Ohtahara syndrome) -  Semiology 

 Electrical clinical manifestations of tonic spasms and burst suppression patterns during sleep and waking states. Tonic seizures are brief and occur in clusters that can repeat several times a day. Some patients may have focal motor clonic seizures. Myoclonic seizures are rare. 

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Neonatal Epileptic Syndromes

Neonatal epileptic seizures  -  Etiology 

Several possibilities. Hypoxic ischemic encephalopathy accounts for 80% of cases 

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Neonatal Epileptic Syndromes

Benign familial neonatal seizures  -  Etiology 

 Autosomal dominant channelopathy with high degree of penetrance. Mutations in the voltage gated potassium channel subunit genes KCNQ2 or KCNQ3. Both form a potassium channel that determines the M – current. Note – mutations in the sodium channel SCN2A are specific to benign familial neonatal – infantile seizures

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Neonatal Epileptic Syndromes

Benign neonatal seizures  -  Etiology 

Unknown. Zinc deficiency has been postulated 

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Neonatal Epileptic Syndromes

Early myoclonic encephalopathy  -  Etiology 

 Multi-factorial disease. Inborn errors of metabolism are the most common causes: non-ketotic hyperglycinemia; propionic aciduria; methylmalonic acidemia; glyceric acidemia; xanthine oxidase deficiency; menkes dz; Zellweger sd.; molybdenium cofactor deficiency.

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Neonatal Epileptic Syndromes

Early infantile epileptic encephalopathy with suppression burst (Ohtahara syndrome) -  Etiology 

 Malformations of cortical development. Examples are hemimegalencephaly; focal dysplasias; Aicardi syndrome; agenesis of mammillary bodies; olivary dentate dysplasia. There are no familial cases. This syndrome is likely to be the earliest age-related specific epileptic reaction of the developing brain to heterogeneous insults

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Neonatal Epileptic Syndromes

Neonatal epileptic seizures  -  Genetic testing or metabolic screening 

None

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Neonatal Epileptic Syndromes

Benign familial neonatal seizures  -  Genetic testing or metabolic screening 

 Can be performed; but expensive and not routinely available 

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Neonatal Epileptic Syndromes

Benign neonatal seizures  -  Genetic testing or metabolic screening 

 None 

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Neonatal Epileptic Syndromes

Early myoclonic encephalopathy  -  Genetic testing or metabolic screening 

 Thorough metabolic screening is mandatory; including serum levels of amino and organic acids and amino acids in the CSF

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Neonatal Epileptic Syndromes

Early infantile epileptic encephalopathy with suppression burst (Ohtahara syndrome) -  Genetic testing or metabolic screening 

 Metabolic screening is mandatory imaging is normal.

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Neonatal Epileptic Syndromes

Neonatal epileptic seizures  - Imaging

Variable

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Neonatal Epileptic Syndromes

Benign familial neonatal seizures  - Imaging

 Normal 

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Neonatal Epileptic Syndromes

Benign neonatal seizures  - Imaging

 Normal 

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Neonatal Epileptic Syndromes

Early myoclonic encephalopathy  - Imaging

Normal at onset; with brain atrophy as the disease progresses.

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Neonatal Epileptic Syndromes

Early infantile epileptic encephalopathy with suppression burst (Ohtahara syndrome) - Imaging

 Usually shows severe abnormalities.