Pediatric Spinal Dysraphisms 

Meningocele: 
- Protrusion of meninges filled with CSF, NO spinal cord tissue within
- Associated with hydrocephalus

Myelomeningocele: 
- Associated with hydrocephalus, chiari II (almost all), syrinx, lipomas, dermoid/epithelioid cysts, ventral and vertebral anomalies, diastematomyelia ("split cord" malformations)
- Kids who have myelo and chiari II who have hydrocephalus and fail their shunts tend to present with mild symptoms, and then clinically deteriorate very rapidly into coma and death. Perhaps the presence of the chiari II makes for less space in the posterior fossa and thus less room for progression of symptoms before acute decompensation.
- Chiari II associated with learning disability / decreased cognitive functioning, although the vast majority will be within the range of normal intelligence
- 70% will have bowel or bladder incontinence.
- Tx: prophylactic antibiotics until closure of neural tube defect to prevent infection, neurosurgical closure within 72 hours of life -- approximate edges of neural placode to create a tube.

A side note on hydrocephalus and shunt failure: its not a question of absolutes but relatives; ventriculomegaly does not have to be present, its more a function of deviation from baseline. Kids with shunts in typically have slit or very small ventricles, so a shunted kid who presents with "normal" ventricles who previously had slit ventricles may be in acute shunt failure. Likewise a kid with a relatively moderate headache who never has headaches and has had progressively worsening pain is concerning, even if the pain is not bad on an absolute scale.

Both brainstem compression or acute hydrocephalus (from shunt failure) can occur in these kids, and they can present with similar symptoms -- make sure to r/o hydrocephalus before doing a suboccipital decompression, because that in the setting of hydro can cause hernation.

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

Myelopathy + a diffuse T2-hyperintense region of cord

Playing the odds 
- most likely is compression - from disc, tumor (met), trauma
- in the absence of that, most likely is demyelinating, esp MS - esp if its short segments, and there are multiple disparate lesions in random-seeming locations.
- long-segment disease - think NMO (esp if there are optic sx) or transverse myelitis (not a dz, a description of a constellation of symptoms that are caused by other things)

Transverse Myelitis etiology:
- Viral - HSV, VZV, CMV, EBV, enteroviruses (echo, coxsackie, polio), HIV, influenza, rabies, HTLV (human T cell lymphoma virus),
- Bacterial - TB, Lyme
- Autoimmune - Sarcoid, Sjogrens, Lupus
- Post vaccine - rabies, cowpox
- MS
- Paraneoplastic
- Vascular - DAF, AVM, clot of spinal aa, heroin vasculitis

Intramedullary Spine tumors
- In adults, ependymoma > astrocytoma
- In kids, astrocytoma > ependymoma
- Astrocytomas tend to look more like diffuse, +/- enhancing regions of cord with a good bit of swelling, and they tend to be associated with slowly progressive symptoms - in the context of an enhancing lesion with rapidly progressive sx and optic symptoms - think NMO, rather than astrocytoma.
- Ependymomas and Hemangioblastomas tend to look more like actual tumors
- Ependymomas often have a cap of hemosiderin at the top and bottom - from prior bleeds - and may have a cystic component

Super helpful diagrams from radiologyassistant.nl:

MS 

- Active lesions in the spine, tend to NOT enhance - in the brain, can enhance 

- If you suspect spine MS (i.e. multiple non-enhancing lesions that are small, and separate in time and space) - then get a brain MRI - if you see characteristic lesions in the corpus callosum/periventricular/pons/subcortical area, increases likelihood of MS

- 1/3 will have spinal symptoms, 95% will have spinal lesions (path dx) - irrespective of symptoms 

- 1/3 of people will have spinal MS ONLY and no brain MS 

Decompressive Hemicraniectomy in Ischemic Stroke 

RCTs:

	DESTINY	DECIMAL	HAMLET	DESTINY II
N (final)	32	38	64	112
Year	2004-2005	2001-2005	2002-2007	2009-2013
Age range	18-60	18-55	18-60	>60
Median age	45	43 (mean)	47-50	70
NIHSS (R/L)	>18/20	>16	>16/21	>14/19
Infarct size (CT)	> 2/3 MCA territory	>1/2 MCA territory + DWI vol > 145 cm2	> 2/3 MCA territory	> 2/3 MCA territory
Median Time to surgery	24 hours	20 hours	>41 hours	28 hours
Exclusion Criteria	mRS >1, bilateral blown pupils, GCS <6, IPH, other serious illness.	mRS>1, big contralateral infarct, big IPH	thrombolysis w/in 12 hrs, mRS > 1, bilateral blown pupils, entire hemisphere stroke	mRS >1, bilateral blown pupils, GCS <6, IPH, other serious illness.

Pooled results from DESTINY, DECIMAL, AND HAMLET: 

- half of the HAMLET trial pts excluded for being randomized too late (> 45 hours after symptom onset -- assuming >48 hours to treatment) 

Thoughts: 

- Decompressive hemicraniectomy (DH) is associated with a significantly reduced proportion of death, but a significantly increased proportion of severe disability (mRS 4-5). 

- Incidence of very good outcome (mRS) is much higher in the surgical intervention group - 14% than the nonoperative group - 2% 

DESTINY II: