Neonatal Epileptic Syndromes Flashcards Preview

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Flashcards in Neonatal Epileptic Syndromes Deck (55):
1

Neonatal epileptic seizures  - Demography 

High in preterm infants. Up to 10%. 

2

Benign familial neonatal seizures  - Demography 

Rare.

3

Benign neonatal seizures  - Demography 

 7% of all neonatal seizures 

4

Early myoclonic encephalopathy  - Demography 

 Rare. Boys and Girls are equally effective. 

5

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

 Rare. Slight male predominance. 

6

Neonatal epileptic seizures  - Age range of onset

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

7

Benign familial neonatal seizures  - Age range of onset

Seizures mainly on the second or third day of life 

8

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

9

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.

10

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.

11

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

12

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

13

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

14

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.

15

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. 

16

Neonatal epileptic seizures  -  Etiology 

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

17

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

18

Benign neonatal seizures  -  Etiology 

Unknown. Zinc deficiency has been postulated 

19

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.

20

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

21

Neonatal epileptic seizures  -  Genetic testing or metabolic screening 

None

22

Benign familial neonatal seizures  -  Genetic testing or metabolic screening 

 Can be performed; but expensive and not routinely available 

23

Benign neonatal seizures  -  Genetic testing or metabolic screening 

 None 

24

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

25

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

 Metabolic screening is mandatory imaging is normal.

26

Neonatal epileptic seizures  - Imaging

Variable

27

Benign familial neonatal seizures  - Imaging

 Normal 

28

Benign neonatal seizures  - Imaging

 Normal 

29

Early myoclonic encephalopathy  - Imaging

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

30

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

 Usually shows severe abnormalities.

31

Neonatal epileptic seizures  - Interictal EEG

Spikes are not reliable at this age. Other patterns such as burst suppression; hemispheric asymmetry; or theta – pointu alternans are more helpful 

32

Benign familial neonatal seizures  - Interictal EEG

 Normal or with discontinuity and multifocal abnormalities; including theta-pointu alternans

33

Benign neonatal seizures  - Interictal EEG

 Normal or with discontinuity and multifocal abnormalities; including theta-pointu alternans

34

Early myoclonic encephalopathy  - Interictal EEG

 Repetitive burst suppression pattern without physiological rhythms. The bursts are short and the suppression is long. Later; a hypsarhythmic pattern may appear; but it is replaced again by burst suppression.

35

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

 Burst suppression pattern with long bursts (with high amplitude slow waves mixed with spikes) and short suppressions. 

36

Neonatal epileptic seizures  - Ictal EEG

Repetitive patterns (multiple frequencies). Usually associated with tonic; clonic or subtle seizures. More commonly on Central temporal; central and occipital regions. Only 21% are associated with clinical manifestations (electro clinical dissociation)

37

Benign familial neonatal seizures  - Ictal EEG

Synchronous and bilateral flattening coinciding with clinical symptoms followed by asymmetrical discharges off epileptiform patterns 

38

Benign neonatal seizures  - Ictal EEG

Rhythmic spikes or slow waves mainly in the rolandic regions (but may also occur elsewhere) 

39

Early myoclonic encephalopathy  - Ictal EEG

 The myoclonia do not have an ictal EEG expression and may follow bursts 

40

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

 The tonic spasms are concomitant with the burst phase.  The ictal pattern may also occur as a diffuse desynchronization or with a more frequent burst suppression pattern. Hypssarhythmia emerges after 3-6 months; later progressing to the slow spike wave pattern. Tonic seizures during the awake and sleep stages in the early days or weeks of life are almost pathognomonic of Ohtahara syndrome.

41

Neonatal epileptic seizures  - Differential diagnosis

 As a rule; any suspicious behavior should be investigated by video EEG.

42

Benign familial neonatal seizures  - Differential diagnosis

 A family history of similar seizures is a prerequisite for the diagnosis 

43

Benign neonatal seizures  - Differential diagnosis

 This diagnosis can be made only after other causes of neonatal seizures have been excluded 

44

Early myoclonic encephalopathy  - Differential diagnosis

 Ohtahara syndrome 

45

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

 Early myoclonic encephalopathy 

46

Neonatal epileptic seizures  - Prognosis

Depends on the underlying cause. 

47

Benign familial neonatal seizures  - Prognosis

 Seizures usually remit in the first six weeks to six months. 10 to 14% may later develop other types of seizures. Normal psychomotor development. 

48

Benign neonatal seizures  - Prognosis

 Normal development and no recurrence of seizures 

49

Early myoclonic encephalopathy  - Prognosis

 Psychomotor developmental abnormalities may occur at the onset of seizures or deteriorate rapidly afterwards. There is hypotonia and hypertonia; deconjugate eye movements and decerebrate posturing with pyramidal signs. Usually patients aren't able to follow objects with their eyes. Dreadful prognosis; with more than half of the patients dying within weeks or months of onset. 

50

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

 Devastating syndrome with high mortality and mobility. 

51

Neonatal epileptic seizures  - Management

Treatment of the underlying cause. Pharmacological treatment is controversial among specialists. Phenobarbita and phenytoin are the most commonly used drugs. 

52

Benign familial neonatal seizures  - Management

 There is no consensus. The use of AED does not influence outcome 

53

Benign neonatal seizures  - Management

Convulsions remit spontaneously without medication 

54

Early myoclonic encephalopathy  - Management

 There is no effective treatment. ACTH or antiepileptic drugs are of no benefit. A trial with pyridoxine is justifiable

55

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

 There is no effective treatment. ACTH or antiepileptic drugs are of no benefit.