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Epilepsy Syndromes
Idiopathic Generalized Epilepsy Syndromes
| Question | Answer |
|---|---|
| Benign neonatal Convulsions age of onset | Day 1 to 7, peak day 5 of life |
| Clinical features of Benign neonatal convulsions | PArtial clonic, may migrate, apnea, rare tonic, many progress to status epilepticus |
| EEG features of benign neonatal convulsions | Normal, excessively discontinous, focal or multifocal discharges |
| Genetics of benign neonatal convulsions | Idiopathic |
| Treatment of benign neonatal convulsions | Phenobarbital, Fosphenytoin, Benzodiazepins |
| Prognosis of Benign neonatal convulsions | Excellent, rare recurrence of seizures |
| Age of onset of BFNC | Majority durng neonatal period, typically during first week of life |
| EEG features of BFNC | May be normal or show transiet changes, including focal epileptiform discharges |
| Clinical features of BFNC | Tonic-clonic, apnea, autonomic phenomena, normal behavior interictally |
| Genetics of BFNC | Autosomal dominant, 85% penetrance |
| BFNC1 is due to mutation in... | KCNQ2, 20q13.3 |
| BFNC2 is due to mutation in... | KCNQ3, 8q24 |
| Treatment of BFNC | Resolves spontaneously |
| Prognosis of BFNC | Seizures usually remit by midinfancy, 10-16% will later develop epilepsy |
| Age at onset of Benign myoclonic epilepsy | 4 months to 3 years |
| Clinical features of Benign Myoclonic epilepsy | Myoclonic seizures, Absences, Rare GTC |
| EEG features of Benign Myoclonic epilepsy | Generalized polyspie-and-wave discharges |
| Genetics of Benign Myoclonic epilepsy | Unknown but 31% have a family history |
| Treatment of Benign Myoclonic epilepsy | Valproate |
| Prognosis of Benign Myoclonic Epilepsy | Excellent |
| Age at onset of GEFS+ Generalized epilepsy with febrile seizures plus | Infancy to adolescence |
| Clinical features of GEFS+ | Febrile seizures beyond age 6, and afebrile myoclonic, astatic, tonic-clonic, absence and complex partial seizures |
| EEG features of GEFS+ | May be normal or show generalized spike-and-wave or focal discharges |
| Genetics of GEFS+ | Autosomal dominant, up to 80% penetrance |
| Genes linked to GEFS+ | SCN1B on 19q13, SCN1A on 2q24, GABRG2 on 5q33-q34 |
| Age of onset of Myoclonic astatic epilepsy | 7m to 8y, peak 2-6y |
| Clinical features of MAE | Myoclonic, Atonic-astatic, Myoclonic-astatic, Absences, tonic |
| EEG features of MAE | Normal at onset, later slowing of the background and 2-3Hz generalized irregular spike-and-wave discharges |
| Genetics of MAE | Family history of seizures in 32% |
| Treatment of MAE | Valproate, ethosuximide, benzodiazepines... Lamotrigine may be used to treat GCT seizures |
| Drugs that must be avoided in MAE | Carbamazepine and vigabatrin |
| PRognosis of MAE | May be favorable in 50% to 89% although up to 41% may have borderline Iq or mental handicap, and some will develop intractable epilepsy |
| Age at onset of Childhood absence epilepsy CAE | 2 to 12 years |
| Clinical features of CAE | More common in girls, Many brief seizures per day. Staring, behavioral arrest without aura or postictal symptoms |
| EEG features of CAE | Normal background with 3Hz generalized spike-and-wave discharges |
| Genetics of CAE | Possibly autosomal dominant, linked to 20q and 8q24.3 |
| Treatment of CAE | Ethosuximide, Valproate, Lamotrigine |
| Prognosis of CAE | Patients typically have remission 2 to 6 years after |
| Age at onset of Eyelid myoclonia with and without absences | 2 to 14y, peak 6 to 8 years |
| Clinical features of Eyelid myoclonia with and without absences | Myoclonia of eyelids, upward deviation of eyes, and retropulsion fo the head with or without impairment of conciousness, mainly after eye closure. GTC |
| EEG features of eyelid myoclonia with and without absences | Frequent high-amplitude 3-6Hz generaliad spike and polyspike-and-wave discharges, typically seen with eye closure |
| Genetics of Eyelid myoclonia with and without absences | Unknown, but most have a family history of epilepsy |
| Treatment of Eyelid myoclonia with and without absences | Valproate, ethosuximide, clonazepam, possibly levetiracetam |
| Prognosis of Eyelid myoclonia with and without absences | Resistant to treatment and the syndrome is lifelong, even in those with well-controlled seizures |
| Age at onset of Perioral myoclonia with absences | 2-13 years, median 10 years |
| Clinical features of Perioral myoclonia with absences | Absence with ictal perioral myoclonia, GTC seizures, Frequent absence status epilepticus |
| EEG features of Perioral myoclonia with absences | Bursts of 4-7Hz generalized, but often asymmetric, spike and polyspike-and-wave, may have focal discharges, ictal EEG 3-4Hz irregular generalized spike and polyspike-and wave discharges |
| Genetics of Perioral myoclonia with absences | Unknown, but 50% have a first-degree relative with idiopathic generalized epilepsy and absences |
| Treatment of Perioral myoclonia with absences | Valrpoate alone or with ethosuximide, lamotrigine, clonazepam; absence status epilepticus should be trated with benzodiazepines |
| Prognosis of Perioral myoclonia with absences | Usually resistant to medication and may be lifelong |
| Age at onset of Epilepsy with myoclonic absences | 11m to 12y6m, mean 7y |
| Clinical features of Epilepsy with myoclonic absences | Myoclonic absence seizures. GTC seizures. Absence. Seizures with fall. Absence status |
| EEG features of Epilepsy with myoclonic absences | Generalized 3Hz with a strict correlation between EEG discharge and myoclonia |
| Genetics of Epilepsy with myoclonic absences | 20% have a family history of epilepsy |
| Treatment of Epilepsy with myoclonic absences | Valproate with ethosuximide, Benzodiazepines, Lamotrigine |
| PRognosis of Epilepsy witn myoclonic absences | epilepsy improves in approx. 1/3 Presence of GTC seizures is a poor prognosis |
| Age at onset of Juvenile myoclonic epilepsy, JME | Late childhood to late adolescence |
| Clinical features of JME | Absence seizures in childhood. Myoclonus early adolescence. GTC adolescence |
| EEG features of JME | Normal background with 3-4HZ generalized atypical spike-and-wave discharges |
| Genetics JME | Complex inheritance linked to 6p, 18, 15q14, and 8q23.3-q24.1 |
| Treatment of JME | Valproate, Lamotrigine, Levetiracetam |
| Prognosis of JME | Response to AED is usually good, but lifelong treatment is frequently required |
| Age at onset of Juvenile absence epilepsy JAE | Adolescence |
| Clinical features of JAE | Daily brief absence seizures, fewer than childhood absence epilepsy, GTC seizures |
| EEG features of JAE | Normal bacground with 3-4Hz generalized spike-and-wave discharges |
| Genetics of JAE | Possible localization to 21q22.1 |
| Treatment of JAE | Valproate, Lamotrigine, Ethosuximide |
| Prognosis of JAE | Response to AED is usually good but lifelong treatment is frequently required |
| Age at onset of Epilepsy with grand mal on awakening | 6-35 years |
| Clinical features of Epilepsy w/GM on awakening | Seizures occur shortly after waking up, may be associated with absence or myoclonic seizures |
| EEG features of Epilepsy w/GM on awakening | Slow background, generalized spike-and-wave discharges, photosensitivity |
| Genetics of Epilepsy w/GM on awakening | Thought to be genetic; 3.3% have a first-degree relative w/epilepsy |
| Prognosis of Epilepsy w/GM on awakening | Response to AED is good, but relapse occurs with AED discontinuation and sleep deprivation |
| EEG features of Perioral myoclonia with absences | Bursts of 4-7Hz generalized, but often asymmetric, spike and polyspike-and-wave, may have focal discharges, ictal EEG 3-4Hz irregular generalized spike and polyspike-and wave discharges |
| Genetics of Perioral myoclonia with absences | Unknown, but 50% have a first-degree relative with idiopathic generalized epilepsy and absences |
| Treatment of Perioral myoclonia with absences | Valrpoate alone or with ethosuximide, lamotrigine, clonazepam; absence status epilepticus should be trated with benzodiazepines |
| Prognosis of Perioral myoclonia with absences | Usually resistant to medication and may be lifelong |
| Age at onset of Epilepsy with myoclonic absences | 11m to 12y6m, mean 7y |
| Clinical features of Epilepsy with myoclonic absences | Myoclonic absence seizures. GTC seizures. Absence. Seizures with fall. Absence status |
| EEG features of Epilepsy with myoclonic absences | Generalized 3Hz with a strict correlation between EEG discharge and myoclonia |
| Genetics of Epilepsy with myoclonic absences | 20% have a family history of epilepsy |
| Treatment of Epilepsy with myoclonic absences | Valproate with ethosuximide, Benzodiazepines, Lamotrigine |
| PRognosis of Epilepsy witn myoclonic absences | epilepsy improves in approx. 1/3 Presence of GTC seizures is a poor prognosis |
| Age at onset of Juvenile myoclonic epilepsy, JME | Late childhood to late adolescence |
| Clinical features of JME | Absence seizures in childhood. Myoclonus early adolescence. GTC adolescence |
| EEG features of JME | Normal background with 3-4HZ generalized atypical spike-and-wave discharges |
| Genetics JME | Complex inheritance linked to 6p, 18, 15q14, and 8q23.3-q24.1 |
| Treatment of JME | Valproate, Lamotrigine, Levetiracetam |
| Prognosis of JME | Response to AED is usually good, but lifelong treatment is frequently required |
| Age at onset of Juvenile absence epilepsy JAE | Adolescence |
| Clinical features of JAE | Daily brief absence seizures, fewer than childhood absence epilepsy, GTC seizures |
| EEG features of JAE | Normal bacground with 3-4Hz generalized spike-and-wave discharges |
| Genetics of JAE | Possible localization to 21q22.1 |
| Treatment of JAE | Valproate, Lamotrigine, Ethosuximide |
| Prognosis of JAE | Response to AED is usually good but lifelong treatment is frequently required |
| Age at onset of Epilepsy with grand mal on awakening | 6-35 years |
| Clinical features of Epilepsy w/GM on awakening | Seizures occur shortly after waking up, may be associated with absence or myoclonic seizures |
| EEG features of Epilepsy w/GM on awakening | Slow background, generalized spike-and-wave discharges, photosensitivity |
| Genetics of Epilepsy w/GM on awakening | Thought to be genetic; 3.3% have a first-degree relative w/epilepsy |
| Prognosis of Epilepsy w/GM on awakening | Response to AED is good, but relapse occurs with AED discontinuation and sleep deprivation |
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