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Epilepsy Borrowed Decks > Neonatal Epileptic Syndromes > Flashcards

Neonatal Epileptic Syndromes Flashcards

1
Q

Neonatal Epileptic Syndromes

Neonatal epileptic seizures - Demography

A

High in preterm infants. Up to 10%.

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2
Q

Neonatal Epileptic Syndromes

Benign familial neonatal seizures - Demography

A

Rare.

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3
Q

Neonatal Epileptic Syndromes

Benign neonatal seizures - Demography

A

7% of all neonatal seizures

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4
Q

Neonatal Epileptic Syndromes

Early myoclonic encephalopathy - Demography

A

Rare. Boys and Girls are equally effective.

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5
Q

Neonatal Epileptic Syndromes

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

A

Rare. Slight male predominance.

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6
Q

Neonatal Epileptic Syndromes

Neonatal epileptic seizures - Age range of onset

A

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

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7
Q

Neonatal Epileptic Syndromes

Benign familial neonatal seizures - Age range of onset

A

Seizures mainly on the second or third day of life

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8
Q

Neonatal Epileptic Syndromes

Benign neonatal seizures - Age range of onset

A

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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9
Q

Neonatal Epileptic Syndromes

Early myoclonic encephalopathy - Age range of onset

A

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

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10
Q

Neonatal Epileptic Syndromes

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

A

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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11
Q

Neonatal Epileptic Syndromes

Neonatal epileptic seizures - Semiology

A

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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12
Q

Neonatal Epileptic Syndromes

Benign familial neonatal seizures - Semiology

A

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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13
Q

Neonatal Epileptic Syndromes

Benign neonatal seizures - Semiology

A

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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14
Q

Neonatal Epileptic Syndromes

Early myoclonic encephalopathy - Semiology

A

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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15
Q

Neonatal Epileptic Syndromes

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

A

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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16
Q

Neonatal Epileptic Syndromes

Neonatal epileptic seizures - Etiology

A

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

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17
Q

Neonatal Epileptic Syndromes

Benign familial neonatal seizures - Etiology

A

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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18
Q

Neonatal Epileptic Syndromes

Benign neonatal seizures - Etiology

A

Unknown. Zinc deficiency has been postulated

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19
Q

Neonatal Epileptic Syndromes

Early myoclonic encephalopathy - Etiology

A

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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20
Q

Neonatal Epileptic Syndromes

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

A

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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21
Q

Neonatal Epileptic Syndromes

Neonatal epileptic seizures - Genetic testing or metabolic screening

A

None

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22
Q

Neonatal Epileptic Syndromes

Benign familial neonatal seizures - Genetic testing or metabolic screening

A

Can be performed; but expensive and not routinely available

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23
Q

Neonatal Epileptic Syndromes

Benign neonatal seizures - Genetic testing or metabolic screening

A

None

24
Q

Neonatal Epileptic Syndromes

Early myoclonic encephalopathy - Genetic testing or metabolic screening

A

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

25
Q

Neonatal Epileptic Syndromes

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

A

Metabolic screening is mandatory imaging is normal.

26
Q

Neonatal Epileptic Syndromes

Neonatal epileptic seizures - Imaging

A

Variable

27
Q

Neonatal Epileptic Syndromes

Benign familial neonatal seizures - Imaging

A

Normal

28
Q

Neonatal Epileptic Syndromes

Benign neonatal seizures - Imaging

A

Normal

29
Q

Neonatal Epileptic Syndromes

Early myoclonic encephalopathy - Imaging

A

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

30
Q

Neonatal Epileptic Syndromes

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

A

Usually shows severe abnormalities.

31
Q

Neonatal Epileptic Syndromes

Neonatal epileptic seizures - Interictal EEG

A

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

32
Q

Neonatal Epileptic Syndromes

Benign familial neonatal seizures - Interictal EEG

A

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

33
Q

Neonatal Epileptic Syndromes

Benign neonatal seizures - Interictal EEG

A

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

34
Q

Neonatal Epileptic Syndromes

Early myoclonic encephalopathy - Interictal EEG

A

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
Q

Neonatal Epileptic Syndromes

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

A

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

36
Q

Neonatal Epileptic Syndromes

Neonatal epileptic seizures - Ictal EEG

A

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
Q

Neonatal Epileptic Syndromes

Benign familial neonatal seizures - Ictal EEG

A

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

38
Q

Neonatal Epileptic Syndromes

Benign neonatal seizures - Ictal EEG

A

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

39
Q

Neonatal Epileptic Syndromes

Early myoclonic encephalopathy - Ictal EEG

A

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

40
Q

Neonatal Epileptic Syndromes

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

A

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
Q

Neonatal Epileptic Syndromes

Neonatal epileptic seizures - Differential diagnosis

A

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

42
Q

Neonatal Epileptic Syndromes

Benign familial neonatal seizures - Differential diagnosis

A

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

43
Q

Neonatal Epileptic Syndromes

Benign neonatal seizures - Differential diagnosis

A

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

44
Q

Neonatal Epileptic Syndromes

Early myoclonic encephalopathy - Differential diagnosis

A

Ohtahara syndrome

45
Q

Neonatal Epileptic Syndromes

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

A

Early myoclonic encephalopathy

46
Q

Neonatal Epileptic Syndromes

Neonatal epileptic seizures - Prognosis

A

Depends on the underlying cause.

47
Q

Neonatal Epileptic Syndromes

Benign familial neonatal seizures - Prognosis

A

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
Q

Neonatal Epileptic Syndromes

Benign neonatal seizures - Prognosis

A

Normal development and no recurrence of seizures

49
Q

Neonatal Epileptic Syndromes

Early myoclonic encephalopathy - Prognosis

A

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
Q

Neonatal Epileptic Syndromes

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

A

Devastating syndrome with high mortality and mobility.

51
Q

Neonatal Epileptic Syndromes

Neonatal epileptic seizures - Management

A

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

52
Q

Neonatal Epileptic Syndromes

Benign familial neonatal seizures - Management

A

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

53
Q

Neonatal Epileptic Syndromes

Benign neonatal seizures - Management

A

Convulsions remit spontaneously without medication

54
Q

Neonatal Epileptic Syndromes

Early myoclonic encephalopathy - Management

A

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

55
Q

Neonatal Epileptic Syndromes

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

A

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

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