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What is benign childhood epilepsy?
- Presents between 3-13
- With tonic activity of the face, salivation and unable to speak.
- Secondary generalisation can occur
- They are usually nocturnal and occur after sleep onset or before awakening.
- Seizures resolve by adolescence
- Classic EEG = central or centrotemporal spikes most prominent during sleep
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What Hz spike and wave are assoc with what epilepsies?
- 2-2.5 = Lennox-Gastaut
- 3hz = Childhood absence epilepsy
- 3-5hz = JME
Lets Cuddle Jellyfish
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What medication works very well for absences and what medication makes them worse?
- Ethosuxamide is very good
- Valproate and Lamotrigine are also options if they have GTCS as well.
- Carbamazepine puts them into absence status
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A young man in his teens with nocturnal seizures in which he thrashes around in his bed. Father and paternal grandfather had the same thing? Whats the syndrome and what genes are assoc with it?
- Autosomal dominant nocturnal frontal lobe epilepsy
- CHRNA4
- Cholinergic nicotinic receptor
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How does Dravet's present?
- Child is usually well.
- Then in the first year of life, they get a fever and a seizure.
- They then develop generalised and focal Tonic clonics, absences, non-convulsive status...
- Difficult to treat but basically avoid all the old drugs - Phenytoin, phenobarb, carbamazepine and lamotrigine
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Topiramate definitely causes kidney stones, but what else does?
Zonisamide - theyre both carbonic anhydrase inhibitors
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In the absence of Focal neurological deficits and witha normal EEG - whats a persons chance of seizure recurrence at 2 years after first fit?
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Some rules of thumb about Head injuries and seizures?
- Severe head injuries should get 7 days of prophylactic anti-epileptic.
- Early seizures occur within the first 24 hours and occur in children under 5 even with mild head trauma
- The more severe the head injury, the later the seizures present.
- Seizures that develop within the first year are more likely to remit.
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What is Panyiotopoulos Syndrome?
- Occurs in children between 2-8 with peak at 5.
- Female preponderance.
- EEG reveals occipital spikes, mainly during sleep
- They present with autonomic symptoms and sometimes LOC
- Imaging is normal
- They all get better in 1-2 years
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Which tumors are assoc with Seizures and in order of most to least epileptogenic?
The slower growing, the more likely to cause seizures....
- DNETs(100%)
- Gangliocytoma(80-90%)
- Low grade astrocytoma (75%)
- Meningioma (30-60%)
- GBM
- Mets
- Leptomeningeal tumors
- Primary CNS lymphoma (10%)
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Which factors distinguish a complex febrile seizure from a simple one?
- Duration >15mins
- Focal manifestations
- Abnormal neurological exam
- recurrence within 24hrs
- History of afebrile seizures in the family
Complex febrile seizures have a greater chance of going on to cause epilepsy in adulthood
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What medication aggravates myoclonic jerks?
Lamotrigine
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Seizures that start with a frightened facial expression, screaming and agitation?
Ventro-medial prefrontal region
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Multiple seizure types, Learning difficulties, low creatinine, bilateral Globus pallidus hypertintensity and speech difficulties?
- GAMT deficiency
- Guanidineacetate methyltransferase deficiency
- Responds well to creatinine replacement
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Which Antibodies are assoc with Rassmusens?
AntiGluR3
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Whats Stiripentol for?
Adjunct in Dravet’s
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What proportion of pregnant ladies have their seizures get worse in pregnancy?
15-30%
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Which AED is most significantly decreased by the COCP?
- Lamotrigine.
- And its reduced by pregnancy
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Seizures and Mute?
- Landau Kleffner syndrome
- 'Acquired epileptic aphasia'
- Really quite normal up to age 6 and then regress quickly.
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Prevalence of Post ictal psychosis
2-7% of people with epilepsy
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Risk factors for Post ictal psychosis
- Family history
- Left temporal onset
- Hipocampal sclerosis
- Previous hx of status
- Poorly controlled epilepsy
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Risk factors for SUDEP
- Young adults with Learning difficulties
- Being on 2 or more AEDs
- More than 15 seizures/month
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Treatment of eclampsia?
- IV mag sulphate
- Labetalol to bring down BP
- Deliver the baby
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Diagnostic criteria for Eclampsia
- DBP >90mmHg
- Proteinuria 1+
- Raised AST
- Thrombocytopaenia
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What is idiopathic hypersomnia?
- Daytime sleepiness that is less irresistible than narcolepsy.
- PSG should show less than 2 SOREMP
- MSLT<8
- Total sleep time 12-14 hrs
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What aspect of night time events should ‘raise’ suspicion for nocturnal frontal lobe epilepsy?
Raised arms!
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Which medications are implicated in causing Rem sleep behaviour disorder?
TCAs, SSRIs, SNRIs, MAOIs
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What is exploding head syndrome?
- Should of a gunshot as you wake up.
- Reassure and discharge, even if there’s a headache that goes with it.
- They can have benzos, TCAs or topiramate of they want but exercise or meditation might be better.
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The sleep wake cycle is regulated by?
Suprachiasmatic nucleus in the hypothalamus
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What is the difference between night terrors and Nightmares?
Terrors - happen in children, Occur early in the night and are not recalled. Also have autonomic symptoms.
Mares - Occur in the last 1/3d of the night, remembered in great detail, no autonomic symptoms, not limited to children.
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What is the other name for Benign neonatal epilepsy?
- Fifth day fits
- Resolve spontaneously by age 4-6 weeks
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Benign myoclonic epilepsy of infancy is different to Benign neonatal epilepsy….?
It comes on later and resolves within a year
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What are the nasty childhood epilepsy syndromes?
Otahara’s and Lafora’s
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Where do absence seizures originate from in Juvenile absence epilepsy?
Thalamus
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What is the other name for Benign childhood epilepsy with centrotemporal spikes?
What do you treat it with?
- Benign Rolandic epilepsy
- Carbamazepine
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What percentage of kids with simple febrile seizures will develop epilepsy?
<5%
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How many kids experience simple febrile seizures?
When do they experience them?
- 5%
- between 5 months and 5 years
- 90% before age 3
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What conditions are associated with Hypothalamic hamartoma?
Precocious puberty
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Which AED in conjunction with Valproate will give you hyperammonemic encephalopathy?
- Topiramate
- Valproate on its own will do it too but topiramate just makes it worse.
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Seizures due to POLG mutations have a predilection for...?
The occipital lobe!
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Whats the best way to investigate if you suspect Dravet's?
- Gene testing.
- SCN1A mutation
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Whats Rufinamide good for?
Lennox Gastaut!!
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2Hz spike and wave discharges?
Lennox Gastaut
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Which neuropsychological deficit is most prominent in hippocampal sclerosis?
Deficits in Verbal learning
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The HLA B1502 allele predisposes to SJS with which drugs?
- Carbamazepine and its derivatives
- Lamotrigine
- Phenytoin
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Which AED inhibits Liver enzymes and therefore causes increased levels of LMT?
SVP
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Which anti-psychotic causes seizures at low or standard levels?
Clozapine
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What is each phase of a clinical trial?
- I - Safety
- II - Efficacy
- III - Comparitive
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EEG hallmark of landau-kleffner?
- Normal when awake
- Continuous slow spike and wave during sleep.
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Landau-Kleffner gene mutation?
GRIN2A
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Which dementia drugs decrease seizure threshold?
- ACHEI
- Donepezil, galantamine, Rivastigmine
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In what way do automatisms and posturing help with localisation?
- Automatisms are usually ipsilateral
- Posturing will be contralateral
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What is Jet lag Disorder?
- Insomnia and excessive daytime sleepiness
- Worst when travelling east
- Avoid sleeping during the day
- Avoid bright light in the early morning when travelling east
- Take bright light in the evening.
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Fencing posture?
- Localises to the frontal lobe
- Bent arm is ipsilateral, straight arm is contra
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Automatisms or ictal nose rubbing lateralisation?
- Temporal lobe
- Ipsilateral to the hand thats moving
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Whats Kleine-Levin syndrome?
- Episodic hypersomina with hyperphagia, irritability, confusion.
- Completely normal between bouts
- They can last for days
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Recurrent febrile seizures in a female infant. Severe clusters of focal onset seizures with secondary generalisations. Often have developmental delay.
- Protocadherin 19 related epilepsy
- Female restricted epilepsy with mental retardation
- X-linked dominant
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Medication options for narcolepsy/cataplexy?
- First line: TCAs (but be carful in glaucoma), SSRIs/SNRIs
- Second line: Sodium oxybate
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What gene is mutated to cause GLUT-1 problems?
SLC2A1
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What kind of seizures does Ring 20
Frontal lobe
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Which antibiotics reduce serum valproate levels?
- Carbapenems
- Need to include a 2nd agent during this time.
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In a patient in ITU, in the absence of EEG, which clinical signs suggest ongoing electrographic seizure activity?
- Pupillary dilatation
- Hypertension
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Lafora's
- EPM2A mutation - encodes laforin - ribosomal protein
- Presents with myoclonus, atypical absences, atonic, complex partial
- Predominance of occipital seizures with transient blindness and visual hallucinations
- Later - dysarthria, ataxia, cognitive decline
- Leads to PAS positive inclusions everywhere including skin cells which are often used for diagnosis.
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Unverricht-lundborg
- Epilepsy with Progressive Myoclonus 1
- Mutation in Cystatin B
- Often mistaken for JME
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What characterises otahara syndrome?
- Progressive epileptic encephalopathy with the first few days of life.
- Characterised by burst suppression on EEG
- They die by age 3
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Whats the best medication for treatment of SEGAs in TSC?
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Keppra mechanism of action?
Synaptic vesicle protein (SV2A) modulator
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Perampanel MoA and SE?
- Partial AMPA antagonist
- Suicidal and homicidal ideation
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Whats the best predictor of risk of seizures on drug withdrawal?
Being on more than 1 AED
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Management of neurocystersercosis?
- Generally - anticonvulsant only
- If theres any evidence of oedema or mass effect - will need to give albendazole/praziquantel + steroid
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What is the only hormonal contraceptive not affected by inducers?
Depo-provera
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