Epilepsy Syndromes Flashcards
Neonates: Benign Neonatal Seizure (a.k.a fifth-day-fits) - family history, clinical presentation/course, EEG, genetics
- idiopathic or benign
- clonic, myoclonic, or apneic episodes
- usually stops by 6 weeks
- later 10-15% epilepsy or 33% febrile seizures
- treatment usually unncessary, but can phenobarbital for 1 month
- normal interictal EEG, theta point alternant
- KCNQ2 (chrom 20), KCNQ3 (chrom 8)
Neonates: Early Myoclonic Encephalopathy
- migrating myoclonus/erratic
- causes metabolic, inherited, various developmental
- EEG burst suppression, no correlate to myoclonus
- poor prognosis
Neonates: Ohtahara Syndrome
- frequent tonic spasms - difficult to control
- neurologic deterioration
- usually structural brain abnormality
- burst suppresion EEG
- NO myoclonic seizure as in early myoclonic epilespy
- vigabitrin early stages
- poor prognosis - often progresses to West syndrome or lennox gastaut
Neonates: Migrating Partial seizures of Infancy
- shifting multifocal seizures
- nearly continuous seizures at times
- progressive microcephaly/psychomotor deterioration
- poor response to treatment
- idiopathic
Neonates: Pyridoxine (Vitamin-B6)-dependent seizures
- autosomal recessive
- diminished GAD activity
- diagnosis by remission of seizure to B6
- treat with life-long B6; fatal if untreated
- differentiate from pyridoxine deficiency seizures AND b6-responsive epilepsy
Infants: Infantile Spasms
- seizure type
- poor outcome when symptomatic
- associated west’s syndrome
- interictal hypsarrythmia, ictal electrodecrement
- acth, vigabitrin
- differentiate from benign myoclonus of infancy (less frequent by 3 months, developmentally normal, normal EEG)
Infants: West Syndrome
- nonspecific triad (infantile spasms, hypsarrythmia, developmental arrest)
- etiology pre/peri/postnatal
- regression possible before seizures
- acth, vigabitrin, corticosteriod
- vigabitrin for Tuberous sclerosis related
Infants: Aicardi’s Syndrome
- infantile spasms, agenesis of corpus collosum, retinal malformation
- x-linked (lethal in boys)
Infants: Benign infantile seizures
-familial and non-familial
-partial seizures
-no postictal
NOTE: benign epilepsy usually idiopathic whereas idiopathic are not always benign
Infants: severe myoclonic epilepsy in infancy “Dravet Syndrome”
- myoclonic seizures worse with time - partial seizures later
- progressive developmental delay
- focal, multifocal, or generalized - photosensitive
- usually medically refractory - valproate and benzos should be tried
Children: Benign childhood epilespy with centrotemporal spine (“benign rolandic epilepsy of childhood”)
- autosomal dominant inheritence
- onset 2-12 years and usually resolved by 15-18 years - normal development
- history, clinical findings, labs negative despite clinical and electrographic focality
- motor or sensory simple seizures - can have secondary generalization
- grouped focal spikes (1.5-3Hz) in central/temporal areas and waves particularly during drowsiness and SLEEP
- EEG is otherwise NORMAL
- only 50-70% of children actually have seizures
- easy to treat - many left untreated
Children: early onset benign childhood occipital epilepsy (“Panayiotopoulos syndrome”, “epilepsy associated with ictal vomiting”, “childhood epilepsy with occipital paroxysms”)
- age 3-6
- autonomic,visual seizures often progress to partial tonic or generalized tonic clonic (autonomic status in ~50%)- NOCTURNAL
- excellent response to anticonvulsants
- interictal: high voltage rythmic occipital spikes and spike-wave complexes 1-3Hz with normal background - increases in non-REM and disappears with eye opening
- ictally: low-voltage fast activty (unilateral/bilateral)
- carbamezepine first line
Children: Late onset childhood occipital epilepsy (Gastaut type)
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Children: Epilepsy with myoclonic absences
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Children: myoclonic-astatic epilepsy of childhood
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