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content:benign_familial_neonatal_epilepsy [2020/02/15 16:21] – [Electroencephalography] icna | content:benign_familial_neonatal_epilepsy [2020/02/23 20:32] – icna | ||
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====== Benign familial neonatal epilepsy ====== | ====== Benign familial neonatal epilepsy ====== | ||
Benign familial neonatal epilepsy is a rare autosomal dominant epileptic syndrome characterised by frequent brief seizures within the first days of life. | Benign familial neonatal epilepsy is a rare autosomal dominant epileptic syndrome characterised by frequent brief seizures within the first days of life. | ||
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===== Clinical features ===== | ===== Clinical features ===== | ||
* Seizures mainly occur in full-term normal neonates following a normal pregnancy and delivery and without precipitating factors. Seizures are brief, of 1–2 min and may be as frequent as 20–30 per day. | * Seizures mainly occur in full-term normal neonates following a normal pregnancy and delivery and without precipitating factors. Seizures are brief, of 1–2 min and may be as frequent as 20–30 per day. | ||
- | * Seizures usually start with tonic motor activity and posturing with apnoea followed by vocalisations, | + | * Seizures usually start with tonic motor activity and posturing with apnoea followed by vocalisations, |
* The tonic phase is followed by a clonic component which is usually asymmetrical and unilateral | * The tonic phase is followed by a clonic component which is usually asymmetrical and unilateral | ||
- | * The post-ictal state is brief and the interictally the neonate is normal | + | * The post-ictal state is brief |
- | * Rarely pure clonic or focal seizures are seen | + | * The neonate is asymptomatic interictally |
+ | * Pure clonic or focal seizures are rarely | ||
===== Electroencephalography ===== | ===== Electroencephalography ===== | ||
- | [{{ : | + | <WRAP right> |
- | * The inter-ictal EEG may be normal, discontinuous, | + | <figure fig1> |
+ | {{ : | ||
+ | < | ||
+ | </ | ||
+ | </ | ||
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+ | * The inter-ictal EEG may be normal, discontinuous, | ||
* The apnoea and tonic motor activity corresponds to synchronous and bilateral flattening of 5–19s on the ictal EEG | * The apnoea and tonic motor activity corresponds to synchronous and bilateral flattening of 5–19s on the ictal EEG | ||
* followed by bilateral and often asymmetrical discharges of spikes and sharp waves with a duration of 1–2 min, coinciding with the vocalisations, | * followed by bilateral and often asymmetrical discharges of spikes and sharp waves with a duration of 1–2 min, coinciding with the vocalisations, | ||
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===== Differential diagnosis ===== | ===== Differential diagnosis ===== | ||
* [[Benign neonatal seizures (non-familial)]] | * [[Benign neonatal seizures (non-familial)]] | ||
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^ ^ Benign (non-familial) neonatal seizures | ^ ^ Benign (non-familial) neonatal seizures | ||
| Main seizures | | Main seizures | ||
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| Interictal EEG | Usually theta pointu alternant | | Interictal EEG | Usually theta pointu alternant | ||
| Source: The Epilepsies: Seizures, Syndromes and Management. Panayiotopoulos CP. Oxfordshire (UK): [[http:// | | Source: The Epilepsies: Seizures, Syndromes and Management. Panayiotopoulos CP. Oxfordshire (UK): [[http:// | ||
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+ | * [[content: | ||
===== Prognosis ===== | ===== Prognosis ===== | ||
* Seizures remit between 1 and 6 months from onset, majority during the first 6 weeks[(: | * Seizures remit between 1 and 6 months from onset, majority during the first 6 weeks[(: | ||
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===== References ===== | ===== References ===== | ||
~~REFNOTES~~ | ~~REFNOTES~~ | ||
+ | {{tag> |