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Epilepsy Syndromes

Idiopathic Generalized Epilepsy Syndromes

QuestionAnswer
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
Created by: 1042022037