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Epilepsy
@NeuroFOD | @NeuroFOD |
---|---|
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 |