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56 Cards in this Set
- Front
- Back
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What is the triad for Lennox-Gastaut syndrome?
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1) MR
2) multiple generalized seizure types 3) slow spike and wave activity |
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What is the diagnosis associated with acquired aphasia and epilepsy?
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Landau Kleffner syndrome
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What are the mutations associated with GEFS plus?
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3 sodium channels;
1 GABA |
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What is the common frequency of generalized spike and wave or polyspike and wave discharges in JME?
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3.5 to 5 hertz, usually triggered by photosensitivity
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What is the gene and chromosome associated with Unverricht-Lundborg disease or Baltic myoclonus?
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cystatin B, chromosome 21;
occipital spikes, progressive myoclonic epilepsy, ataxia and dementia; markedly photosensitive and giant SSEP |
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What is the chromosome, mode of inheritance nad gene involved in Lafora disease?
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autosomal recessive, laforin gene mutations, chromosome 6
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What are the clinical characteristics of Lafora disease?
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seizures between 10-18, rapid and relentless progression, severe myoclonus, dementia, spastic quadriparesis, death by 20; also has significant photosensitivity
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What percentage of patients with syncope have convulsive syncope?
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45% according to one small study of 18 patients with defibrillators
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What are some motor characteristics that can help suggest psychogenic non-epileptic seizures?
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bilateral movements with preserved awareness, pseudo-sleep, stop and go activity, asynchronous activity, weeping, pelvic thrusting, suggestibility, occuring with an "audience"
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What is the abnormality in Nocturnal frontal lobe epilepsy?
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autosomal dominant with acetylcholine receptor abnormality
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What is the abnormality in childhood absence epilepsy?
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3 HZ frontal predominant spike and wave, Ca channel, CACNA1 or Chloride channel
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What is the abnoramlity in juvenile absence epilepsy?
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chloride channel or glutamate receptor
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How does juvenile absence epilepsy differ from childhood absence epilepsy?
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age of onset- jae is 10-17, less frequent/severe absence spells, good response to therapy, = gender predilection; can have GTC, absence and myoclonic jerks, faster than 3 HZ spike and wave
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What is the relationship between CAE and JME?
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6-8% of CAE evolves into JME
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What is the most common generalized epilepsy in adulthood?
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JME
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What percentage of patients with JME respond to AED therapy?
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85%
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What are 4 agents that have shown success with JME treatment?
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VPA, ZNS, LEV, TOP
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Can AEDs ever be tapered off in patients with JME?
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AED therapy is usually lifelong
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What are 5th day fits?
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These focal seizure occur in healthy babies between days 4 and 6 of life with normal background and focal activity during the event, interictal rhythmic theta present, good prognosis, some with mild developmental deficits later in life
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What is the most common partial epilepsy of childhood?
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benign childhood epilepsy with centrotemporal spikes, accounts for 16% of epilepsies before 15 and 24% between age 5 and 14
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What does N9 correlate to on SSEP?
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spinal cord
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What does N13 correlate to?
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dorsal column nuclei in brainstem
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What n18 correlate to?
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thalamus
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What does n20 correlate to?
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cortex
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What is the surgical treatment for Landau-Kleffner syndrome?
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multiple subpial transactions because it leaves intact the cortical architecture necessary for functioning but disrupts lateral spread impt for seizures to spread
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Isoniazid can decrease the metabolism of which AEDs?
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VPA and CMZ
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Vertiginous seizures "feeling of room tilting" localize to what part of the cortex?
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lateral temporal region
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What mechanism leads to hyperthermia in predominantly children who are taking topiramate or zonisamide?
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Hypohidrosis
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What type of interactions occur between VPA and phenytoin?
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VPA displaces phenytoin from its protein binding sites, leading to increased levels of free pht, inhibits clearance of pht, and pht induces vpa metabolism
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What are 3 agents used to treat JME?
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Topiramate, Valproate and Levetiracetam; Lamictal has the potential to exacerbate myoclonic seizures
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What are 4 things that suggest good seizure control following temporal lobectomy?
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predominantly unilateral ictal & interictal EEG abnormalities, history of febrile seizures, and mesial temporal sclerosis on mri
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What is the efficacy of vagus nerve stimulation in patients with refractory secondary generalized epilepsy?
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50% chance of a 50% reduction of seizures, mood may improve, few become seizure free
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What are 3 medications that have been shown that can treat absence epilepsy?
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balproate, ethosuximide, lamotrigine
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What 2 agents carry a risk of nephrolithiasis and are relatively contraindicated?
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topiramate and zonisamide; 1.5% risk of kidney stones
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What medications can increase levels of carbamazepine?
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isoniazid, propoxyphene, macrolids, calcium channel blockers
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What medication can cause signs of cmz toxicity without increasing the level of cmz?
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vpa can do this because it causes cmz metabolite to accumulate
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What type of epilepsy is the GABA A receptor subunit alteration associated with?
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GEFS plus
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What type of epilepsy is the voltage gated sodium channel subunit associated with?
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GEFS plus
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What type of epilepsy is the neuronal nicotinc acetylcholine receptor subunit associated with?
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benign autosomal dominant nocturnal frontal lobe epilepsy
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Mutations of P/Q type calcium channel have been associated with what type of epilepsy?
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childhood absence epilepsy
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What is the interaction of oral contraceptives and lamotrigine?
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Oral contraceptives decrease the half life of lamotrigine and may lead to break through seizures
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Explain zero order kinetics
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Zero order kinetics means that a constant fraction of the drug is not eliminated per unit time but at some point the enzymes become saturated so small increases in dose may lead to high increases in level
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Choking and strider associated with salivation and repetitive swallowing in seizures have localized where?
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insular cortex
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What is the chance of seizure freedom in patients after anterior temporal lobectomy?
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60 to 80% chance seizure free
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What medications cause osteoporosis or osteomalacia?
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All the enzyme inducers lead to osteomalacia because they alter the metabolism of vitamin D
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How could VA lead to osteomalacia?
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It may increase bone turnover
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What is autonmic seizures and autonomic status epilepticus?
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Panayiotopoulos syndrome
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Leucine rich glioma inactivated agene abnormality leads to what type of epilepsy?
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autosomal dominant partial epilepsy with auditory features
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Name 7 broad spectrum AEDs.
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1. VPA
2. LEv 3. TOP 4. ZNS 5. LTG 6. BZDZ 7. RUF |
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Name 5 AEDs that are renally metabolized.
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1. TOP
2. GBP 3. LTG 4. LEV 5. FELBAMATE |
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What is the rate of seizure freedom after monotherapy with 1 AED according to Kwan and Brodie?
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60%
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What is the conclusion of the VA Cooperative Geriatric Epilepsy study comparing LTG, CBZ and GBP?
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CBZ is least tolerated.
LTG with least side effects in this age group. All equally efficacious although French editorial states cannot conclude this as trial was not designed that way. |
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What is Dravet's syndrome?
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severe myoclonic epilepsy
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Why should one be cautious regarding the use of vigabatrin?
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It may worsen absence and myoclonic seizures
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What are five differences between epileptic and eiee?
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1. sz type: myoclonic vs. frequent partial
2. EEG suppression burst during sleep not wake for EME both sleep and wake for EIEE 3. Duration SB 1-6 months then goes away for EMG persists longer for EIEE 4. Evolution of EEG SB then to hypsarrhythmia SB persists 5. Etiology EME: mitochondrial/metabolic EIEE: unkonwn |
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What are 3 difference between cryptogenic and symptomatic West syndrome?
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cyptogenic: normal development until 3-6 months.
Treatment with ACTH outcome MR symptomatic developmental delay prior to seizures Treatment with Vigabatrin LGS/generalized seizures |
