Tuesday, July 28, 2015

Miscellaneous facts about peds neurology: 

First line drugs for focal seizures that generalize 
- Keppra - clean drug, no interactions, nice side effect profile
- Lamotrigine - also pretty clean, few side effects - can't be loaded - must be titrated up slowly to avoid SJS
- Oxcarbazepine - very effective, can be loaded - can cause aggression like keppra

Generalized epilepsy 
- more likely to have EEG changes at baseline between ictal events - i.e. a negative routine EEG is more likely to be expected with focal rather than generalized epilepsy

EEG
- Blinking lights tends to trigger JME
- Hyperventilation brings out absence seizures
- Indications for long term EEG - management guidance in status (spot EEG is sufficient for diagnosis - if someone is really in status, you'll see it immediately), seizures that are spaced far apart and you want to catch something.
- Key to diagnosis of seizures is rhythmicity - when your EEG looks like an EKG, you're in trouble

DDx for neonatal seizures : 
- Presenting anytime: hypoglycemia, infection, bleeding
- Presenting immediately at birth: hypoxia (~80%), withdrawal from maternal drugs
- Presenting near the first week: "Fifth day fits" - benign familial neonatal seizures, commonly inherited in families (KCNQ2 mutation), severe inherited metabolic diseases
- Presenting after first week: inherited metabolic (inborn errors of metabolism, storage diseases, etc) - tend to occur after the infant starts eating

Neonatal seizures rarely present as grand-mal/tonic clonic seizures - if these movements occur, they are more likely something else. Neonatal seizures tend to be more subtle, and may even present as isolated disturbances to vital signs (tachycardia, apena, etc)

Phenobarb and phenytoin are often used to treat neonatal epilepsy. Neither are very effective (30-40% range) and they both have terrible side effect profiles, but have been historically used and there's more information on them than other drugs -- its almost impossible to get IRB/ethics clearance to do research on neonates, so we can't do any research on newer drugs that are probably better and more effective and less toxic, so we never have any data on them, and so we have to keep using these old, crappy drugs.

More about phenytoin: 
- first order clearance until you saturate enzymes (around blood level of 20) - then becomes zero order. So the same dose that will take you from 10 to 20 might take you from 20 to 60.
- younger kids metabolize phenytoin faster, so you have to give higher doses
- phenytoin is very hard to get into solution, so you have to use a very basic solution (i.e. pH ~ 11) and that causes all sorts of problems - cardiac dysrhythmias, if it extravasates in a peripheral IV It'll cause all the skin to slough off your hand "purple stocking syndrome"
- fos-pheny is safer bc it is a prodrug that can be given in a neutral solution - its metabolized to phenytoin in your liver
- fos-pheny can be loaded faster, but its a prodrug, so in the end the onset of action is probably not that different between fospheny and pheny - and its a a lot more expensive.
- ataxia, gingival hyperplasia (bc phenytoin is excreted in saliva and evaporates, irritating gums), coarsens facial features if used for a long time, hirsutism

Primitive reflexes: 
- Moro - you drop their head down quickly, and they bring their arms together in a C, should be symmetric
- Forced head turning - they posture into a fencer stance with the ipsilateral arm extended
- Grasp - hand and foot
- Upgoing babinski
- Snout - tap closed lips, will purse
- Root - touch cheek, infant will turn towards that side and suck
- Dazzle - bright light over closed eyes, will close eyes tighter and will extinguish with repeated light

Infantile Spasms
- Treated with prednisone/ACTH - if they receive early treatment, can prevent long-term neurological damage
- Associated with tuberous sclerosis
- Very consistent timing-- you could time a watch by the regularity of the spastic events

AE Drugs that come in an IV loadable formation
- Pheny/fospheny
- Keppra
- Oxcar
- Phenobarb
- Most benzos

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