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Epilepsy Epilepsy
Syncope -- <30s, +loc, +myoclonus, +\/bp, +\/hr Neurogenic syncope. Treat with midodrine
Syncope -- <30s, +loc, during exercise, +\/bp, +/\hr Cardiogenic syncope
Syncope -- <30s, +loc, random, +\/bp, variable hr Autonomic syncope. A/w Parkinsons and Lewy-Body dementia
Syncope -- <30s, +lightheaded, +/\hr, NO bp change POTS. Treat with exercise and fluids
Foci -- deja vu, automatisms Mesial temporal lobe
Foci -- speech apraxia Mesial temporal lobe
Foci -- epigastric rising sensation Mesial temporal lobe
Foci -- bilateral thrashing without loc Supplementary motor area in frontal lobe
Foci -- jacksonian march Starts with hand -> arm -> face -> hemibody -> generalization. Frontal lobe near motor cortex
Foci -- body image distortion Parietal lobe
Foci -- flashing lights Occipital lobe
Foci -- hallucinations Olfactory = mesial temporal. Auditory/visual = lateral temporal. Unformed/abstract = occipital
AED -- focal seizure Carbamazepine or oxcarbamazepine
What is strongest predictor of driving? Duration of seizure freedom
Corpus callosotomy is best for what type of seizure? Atonic, focal drop attacks
Foci -- eye/head deviation Frontal eye fields in frontal lobe. OR Occipital seizures in Panayiotopourlous syndrome
Foci -- eye blink iL hermis
Foci -- figure 4 posture cL frontal/temporal
Foci -- fencing posture cL SMA (supplementary motor area)
Foci -- nose rub iL temporal (ipsilateral to the hand that rubs the nose)
Foci -- uncontrollable laughing Gelastic seizure. A/w hypothalamic hammartoma
Foci -- spit/drool/cough/vomit Non-dominant temporal
Foci -- loud grunt/scream Frontal
Most common cause of seizure In age 20s = trauma. In age 40s = cancer. In age 60s = stroke
New onset seizures + rapid dementia Hashimotos thyroiditis. Note CJD, paraneoplastic, autoimmune syndromes all in differential
Treatment of new onset 1st time seizure If unprovoked = low recurrence = no treatment. If provoked = treat underlying etiology
When is epilepsy refractory? Failure of >2 medications due to efficacy (not side-effects) in compliant patient
VNS is best for what type of seizures? Generalized seizures originating from mesial temporal lobe
Empiric treatment for child with refractory seizures B6. If it is pyridoxine-dependent epilepsy, this is a quick diagnostic and therapeutic intervention
Age of breath holding spells 6 months to 6 years
Sandifeur syndrome Baby GERD -> stiffness + hyperflexion of back -> shaking/apnea/loc. Often ~30mins after meal
Age of febrile seizures 6 months to 6 years
Simple vs complex febrile seizure Old terminology. Complex = focal, >15mins, or >1 in 24hrs
Treatment of 1st time febrile seizure None. Treat etiology of fever. Note: 5-10% will develop epilepsy (>2 seizures) and require tx
Infant -- GTCs + nL development + nL interictal EEG Benign familial infantile epilepsy. A/w SCN1A and PRRT2. If PRRT2 = late dyskinesias
Infant -- myoclonic sz + nL develop + nL interictal EEG Benign myoclonus of childhood. NOT an epilepsy syndrome
Infant -- multiple sz types + nL develop + interictal slow Generalized epilepsy with febrile seiuzres (GEFS+).
Infant <1yo -- multiple sz types + regression + slow EEG Myoclonic epilepsy of infancy (Dravet)
Infant 2-6yo -- multiple sz types + regression + slow EEG Myoclonic-Atonic Epilepsy (Doose). Later onset than Dravet, more atonic seizures
AEDs to avoid in GEFS+, Dravet, Doose Avoid Na-channel AEDs
Best AEDs for GEFS+, Dravet, Doose Valproate, Clobazam, keto diets. Avoid Na-channel AEDs
Fifth day fits Benign familial neonatal convulsions. A/w KCNQ2/KCNQ3. EEG theta pointu alterant
Benign familial neonatal convulsions gene KCNQ2 / KCNQ3
EEG -- theta pointu alterant Benign familial neonatal convulsions. A/w KCNQ2/KCNQ3
Otahara syndrome pathophysiology Cortical dysplasia or malformation
Tonic spasms lasting ~2mins, occur 100s of times /day Otahara syndrome
EEG -- burst suppression in infant Otahara syndrome
Pathophysiology of pyridoxine dependent epilepsy B6 is required cofactor for Gluatamic acid decarboxylase. No B6 = /\glutamate, \/GABA
Sudden brief <5s jerks in infant, clusters on awakening Infantile spasms. A/w West syndrome, Aicardi, and Tuberous sclerosis
EEG -- hypsarrythmia Infantile spasms. A/w West syndrome, Aicardi, and Tuberous sclerosis
Treatment of infantile spasms ACTH is better than steroids. Use vigabatrin only if etiology is from tuberous sclerosis
West syndrome Infantile spasms + developmental delay
Aicardi syndrome Infantile spasms + corpus callosum agensis + retinal malformations
EEG -- unilateral centrotemporal spikes, /\ with sleep Benign epilepsy with centrotemporal spikes (BECTS) = aka Rolandic epilepsy
Nocturnal sz, unilateral, focal face/arm Benign epilepsy with centrotemporal spikes (BECTS) = aka Rolandic epilepsy
Nocturnal sz, unilateral, focal face/arm. +verbal delay Landau-Kleffner syndrome. OR Epilepsy Status Epilepticus of Sleep ESES
EEG -- bilateral centrotemporal spikes in NREM sleep Landau-Kleffner syndrome. BECTS (Rolandic) has unilateral spikes
Landau-Kleffner vs Autism clinically L-K = verbal regression only. Autism = verbal regression + social/repetitive behaviors
Panayiotopoulous syndrome Occipital epilepsy w/ tonic eye deviation (+nausea/vomit). A/w dysautonomia
Panayiotopoulous syndrome treatment No AEDs required. But if severe = CBZ is best
Gastaut syndrome Late-onset Panayiotopoulous in children >8yo. Less dysautonomia, but worse epilepsy
Absence epilepsy genes GABRG2, CACNA1A, GLUT1
Absence epilepsy age 2-12yo
Absence epilepsy treatment Ethosuximide if ONLY absence seizures. If also GTCs = Lamotrigine 1st, Valproate 2nd
EEG -- 3Hz spike-wave Absence epilepsy
Absence epilepsy trigger Hyperventillation. Photic stimultation is more for JME
Juvenile Myoclonic Epilepsy JME age 12-18yo. Older than absence that's 2-12yo
Myoclonic seizures on awakening Juvenile myoclonic epilepsy
Myoclonic seizures that worsen with alcohol Juvenile myoclonic epilepsy
Juvenile Myoclonic Epilepsy JME treatment Valproate 1st. Can Lamotrigine but worsens myoclonic jerks. Avoid GBT/Pheny/CBZ
Juvenile Myoclonic Epilepsy JME EEG 4-6Hz polyspike and wave
Lafora Body disease Mitochondrial. Myoclonic+atonic epilepsy w/ ragged red fibers. Histo= targetoid cerebellar cells
Unverricht-Lundborg disease Mitochondrial. Myoclonic+atonic epilepsy with ragged red fibers
AEDs to avoid in mitochondrial epilepsy Avoid valproate
Lennox Gastaut EEG Slow 2.5Hz spike-wave
Rasmussen's encephalitis Unilateral hemisphere inflammation/degeneration. Intractable focal seizures, cognitive decline.
Rasmussen's encephalitis treatment Steroids/IVIG (because it is inflammatory process, NOT neurodegenerative)
Rasmussen's encephalitis gene Anti-GlutR3
Status epilepticus treatment protocol Benzos q5mins x3 -> Pheny/VPA/Keppra load -> rpt load x2 -> propofol/pentobarb
Created by: amitchaudharimd
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