neurology Flashcards
rate of epilepsy occurrence in canada
1-2% of canadians
40 000 people in BC
associated with cerebral palsy, autism, intellectual impairment
what is an epileptic seizure
paroxysmal interference of brain function due to abnormal electrical discharge in the brain
sudden disruption of the brains normal electrical activity
this may result in motor activity, sensory symptoms, behavior changes and/or altered consciousness depending on which areas of the brain are involved
how common are seizures
1 in 12 people will have a seizure
50% of people who do, have only 1
define epilepsy
2 or more unprovoked seizures
name the generalized seizures
tonic atonic absence clonic tonic-clonic myoclonic
name the focal seizures
dyscognitive
bilateral convulsive seizures
what are the 5 questions to ask about a first afebrile seizure?
- was the episode an epileptic seizure
- what was the cause of the seizure
- what investigations should i do
- does the child require treatment
- what else should i think about
how do you determine if a seizure was an epileptic seizure
clinical hx is key
detailed description of the event and circumstances around the event
- -setting in which it occurred
- -observation at onset of the event
- -how did the patient feel just prior to the event
- -other important factors like development, febrile seizures, family hx of epilepsy, history of brain injury/infection
what are the factors that favor an epileptic seizure
aura
brief
post ictal confusion
automatisms
amnesia of event
incontinence
tongue biting
events in sleep
eyes open
what are some non epileptic paroxysmal events
- syncope and reflex anoxic seizure
- day dreaming
- steroetypies
- panic/anxiety attacks
- psychogenic nonepileptic seizures
- sleep disorders
- migraine, complex
- transient ischemic attack/stroke
- paroxysmal movement disorder
what are some examples of syncope/reflex anoxic seizure episodes
vasovagal syncope
breath holding spells
orthostatic hypotension
cardiogenic (i.e prolonged QT, other arrhythmia)
Gastroesophageal reflux (sandifer syndrome)
what causes syncope
sudden decrease in oxygen availability to the brain (via reduced cerebral blood flow or oxygen content of blood)–> reflex anoxic seizures
NOT epileptic seizures
can result in LOC, GTC, incontinence
what causes epileptic seizures in childhood (categories)
50% genetic
30% structural-metabolic
10% unknown
what are the genetic causes of epileptic seizures in childhood
childhood and juvenile absence epilepsy
benign Rolandic epilepsy of childhood
juvenile myoclonic epilepsy
what are the structural-metabolic causes of epileptic seizures in childhood
malformation of brain development
neurocutaneous syndromes like tuberous sclerosis
vascular malformations
congenital or acquired CNS infections
hypoxic ischemic brain injury
stroke
traumatic brain injury
tumour
inborn error of metabolism
how do you distinguish between idiopathic and symptomatic epilepsy
idiopathic–> norma development, neuro exam. no family hx of epilepsy. no hx brain injury. characteristic EEG abnormalities
symptomatic–> developmental delay, hx brain injury, abnormal neuro exam, other congenital malformations
what investigations should be done in a child presenting with first epileptic seizure
a child presenting immediately after this first seizure should have a blood glucose checked
further labs should be guided by clinical judgement at the time i.e vomiting, fever, illness
consider toxicology
ECG with QTc interval (may be syncopal episode)
what is the most useful investigation for seizures
EEG
assessing risk of recurrent seizures
epileptic syndrome or seizures that may be associated with a structural lesion
can guide anticonvulsant meds
helps ID prognosis
when should you do the EEG
does not need to be done in ER
order an outpatient sleep deprived EEG
(may need sedation i.e autism)
does a normal EEG exclude epileptic seizures?
no
in patients with epilepsy, 1st EEG displays epileptiform activity only 50% of the time –> if you suspect epilepsy but EEG is normal, repeat EEG increases yield
what is the significance of an abnormal EEG in the setting of a seizure
if it is true epileptiform activity, there is a two fold increase in tendency to seizure
can epileptiform discharges alone dx epilepsy
no–not diagnostic without appropriate clinical history
certain epileptiform discharges are seen in 4% of children without epilepsy
what kids should get neuroimaging
children who have had more than 1 afebrile seizure
exception is if the clinical history and EEG findings are consistent with genetic etiology
**neuroimaging abnormalities occur in up to 1/3 of children with a first afebrile seizure, but only 2% will demonstrate a clinically significant abnormality that will influence management
**seizures are an uncommon presenting symptom of a brain tumour in kids
what are the indications for a pediatric neurologist
focal lesion on neuroimaging
uncertainty as to seizure type and/or epilepsy syndrome
seizure associated with developmental delay/regression
multiple seizure types
infantile spasms
failed appropriate therapy with two or more AEDs
how do behavioral problems in kids with epilepsy compare to those with diabetes
much more common in kids with epilepsy
how does a new epilepsy diagnosis in a kid affect the mom
1/3 of mothers of kids with newly dx epilepsy exhibit sx of PTSD or MDD
define benign febrile seizures
an idiopathic (inherited) epilepsy
tendency to seizures associated with fever or an intercurrent infection in normal children aged 6 mo-5 years (most 1-2 years)
occurence 2-4%–> most common inherited form of seizures
NOT associated with meningitis, encephalitis or toxins…
normal CT scan, often family history, usually developmentally normal
most common seizure type is generalized tonic-clonic
describe a typical febrile seizure
1 seizure in 24 hours
less than 5 min duration
no focal features
describe an atypical febrile seizure
2 or more seizures in 24 hours
last longer than 5 min
focal seizures
what are the recurrence risks of febrile seizures
1/3 will have 1+ seizure
1/11 will have 3+ seizures
75% of the recurrence happen within 12 mo
what puts a kid at increased risk of a recurrent febrile seizure
family hx
younger than 12 mo
low temp at seizure
*no increased risk of recurrence with atypical febrile seizures
what are the risk factors for developing Afebrile seizures after febrile seizures
developmentally abnormal prior to febrile seizure
atypical febrile seizure
positive family history of epilepsy
risk depends on number of risk factors–> 0 risk factors is less than 2% risk, 3 risk factors is 10% risk
do you start treatment after a single, brief febrile seizure?
no–> since the majority of kids will not have recurrent seizures
when should you give medication for a kid who had febrile seizures?
if the kid had a prolonged febrile seizure, teach parents to give VALIUM or buccal MIDAZOLAM to abort a seizure lasting longer than 5 min
what parental counseling should be done after a febrile seizure
first aid management of a seizure at home
safety precautions around water/bathing
risk of recurrence of febrile seizures
risk of recurrence of epilepsy
risks of brain damage (none for seizures lasting less than 30 min)
list primary headaches
migraine headaches–with and without aura, migraine equivalents
tension headaches
trigeminal autonomic cephalagias (TACs)
list secondary headaches
structural (tumour, Chiari etc)
infectious
hemorrhagic
traumatic
vascular
altered ICP without intracranial lesion (pseudotumour cerebri/idiopathic intracranial hypertention, post LP headache)
med overuse
what should you ask about on hx for headaches
- course
- -onset (new vs old)
- -frequency
- -duration
- -course (worsening, improving, stable, fluctuating) - onset and timing
- -triggers (sleep deprivation, illness, food)
- -timing (nocturnal, first thing in the morning, later in the day)
- -episodic vs continuous
- -rate of onset (sudden, gradual)
- -warning signs (aura)
- -exacerbating factors (valsalva, position) - characterstics
- -severity
- -quality (throbbing, pounding, pressure, squeezing, stabbing, burning, dull)
- -location (unilateral vs bilateral, generalized vs localized) - associated features
- -N/V
- -photo or phonophobia
- -neuro features/auras - systems review
- -loss of skills or developmental regression
- -neuro sx and signs
- -systemic illness (weight changes, fevers etc)
what should you pay attention to in the physical exam for a kid with headache
head circumference
high ICP? papilledema on fundoscopy or CN palsies
evidence of focal neuro abnormalities (hemiplegia, ataxia, vision or movement abnormalities, abnormal reflexes)
in what cases should you consider imaging in the setting of a headache
- recent onset of severe headache
- change in type of headache
- neuro dysfunction
- focal findings
- signs of increased ICP
- altered consciousness
- co existence of seizures
when is imaging not indicated in the setting of a headache
recurrent headaches
normal exam
are routine bloodwork or EEG indicated in evaluation of headache?
no not really
what are the headache red flags (12)
- sudden, worst, thunderclap headache
- progressive headache
- occipital headache
- positional
- worse with valsalva, exertion
- present first thing upon waking up
- wakes patient at night
- neuro or devel regression
- seizures
- systemic illness
- focal findings on exam
- findings suggestive of increased ICP
what are migraines
paroxysmal headaches separated by pain free episodes
what are associated features of migraine
aura nausea abdo pain throbbing quality unilateral family history
what are the diagnostic criteria for migraine without aura
- at least 5 attacks fulfilling the next 3 criteria
- headache lasts 2-72 hours
- headache has at least two of–> unilateral location (may be bilateral or frontotemporal but not occipital), pulsating quality, moderate or severe pain, aggravation by or causing avoidance of routine physical activity
- during headache at least 1 of–> nausea and/or vomiting, photophobia and/or phonophobia which may be inferred from behavior
- not attributable to another disorder
what is an aura
attacks of reversible focal neuro sx
usually develop gradually over 5-20 min
lasts less than 60 min
migraines usually follow but may be absent
most commonly visual–> binocular scotoma, distortion/hallucinations, monocular
what are the diagnostic criteria for migraine with aura
- at least two attacks fulfilling the next 2 criteria
- one or more of the following fully reversible aura symptoms
- -visual, sensory, speech and/or language, motor, brainstem, retinal - at least two of the following 4 characteristics–>
- -at least one aura symptoms spreads gradually over more than 5 minutes and/or two or more symptoms occur in succession
- -each individual aura symptom lasts 5-60 minutes
- -at least one aura symptom is unilateral
- -the aura is accompanied or followed within 60 min by a headache - not better accounted for by another ICDH-3 diagnosis and TIA has been excluded
what are other types of migraine with aura
brainstem aura
hemiplegic migraine
retinal migraine
alice in wonderland syndrome
describe a basilar migraine
typically younger (mean age 7y)
posterior fossa involvement
vertigo, dizzyness, ataxia, nausea, diplopia, tinnitus, hearing loss etc
- *neuroimaging to rule out structural posterior fossa lesion recommended
- *seizures and ingestions are also on the ddx
describe a hemoplegic migraine
aura has “stroke like” quality–typically hemiplegia
may also include hemisensory changes, aphasia, visual field deficits
several familial forms
- *neuroimaging and other tests to rule out vascular, intracranial etiologies
- *ddx is stroke, TIA, seizure (post ictal todd’s paresis), demyelinating disease, tumour, hemorrhage, metabolic disturbance
what is alice in wonderland syndrome
visual distortions, may be bizarre/vivid
macropsia–> object appear larger
micropsia–> objects appear smaller
metamorphopsia–> objects appear distorted
teleopsia–> objects appear far away
what are “childhood periodic syndromes” and why do we care about them
commonly precursors of migraine
cyclical vomiting, abdo migraine, benign paroxysmal vertigo of childhood
what is the pathophysiology of migraine generally
inherent vulnerability with hyperexcitable cortex
often inherited
inherited vulnerability plus triggering events cause attacks
what happens in the brain to cause a migraine
- cortical spreading depression
- -neuronal event of neuronal depolarization, spreading across the cortex–> pathophysiologic correlate of migraine aura - accompanied by oligemia, later followed by hyperemia
- extravasation of proteins (sterile inflammation) into dural vessels–> activation of meningeal afferents–> trigeminal activation–> pain
- role of central brainstem structures in modulating pain (locus ceruleus–adrenergic, dorsal raphe–serotoninergic)
- sensitivity to activities and cutaneous allodynia due to sensitization of trigeminal afferents
what are the principles of migraine treatment
parents must recognize “typical” headaches
best to treat headache at ONSET of pain
–delay makes it harder to treat and more likely to have chronic or status migrainosus
use adequate dosing for age and weight
goal to relieve symptoms as best as possible and enable patient to return to normal function within 1-2 hours
tx encompasses lifestyle mods/prevention, meds (abortive and prophylactic meds) and non pharma intervention
what lifestyle mods can be used to manage migraine
ID and eliminate triggers
improve sleep
ensure adequate food and fluid intake
avoid stressors
what is used for abortive therapy in migraines
- ibuprofen (10mg/kg/dose)
- -FIRST line, superior to acetaminophen
- -may use up to 15mg/kg/dose - acetaminophen (15mg/kg/dose)
- -possible faster than ibuprofen
- -use in those with poor tolerance to ibuprofen or contraindications
- -avoid exceeding 60mg/kg/day due to hepatotoxicity - triptans
- -recommended in patients whose headaches respond poorly to NSAIDs or acetaminophen
what are triptans
migraine specific agents
selective serotonin receptor AGONIST at 5-HT1B and 1D sites
4 recommended for pediatric use
- -almotriptan (12 and older)
- -rizatriptan (6-17 years old)
- -sumatriptan
- -zolmitriptan
what are the contraindications to triptans
- vascular disorders
- -stroke, TIA, HTN, angina pectoris, MI, peripheral artery occlusive disease, reynaulds - vascular risk factors
- MAOI use within two weeks
- ergotamine use
- impaired renal or liver function
- pregnancy
- lactation
what is the emergency treatment for a migraine
- analgesics (naproxen, diclofenac, ketorolac… IV or IM)
- dopamine antagonists (help N/V, specific antimigraine)
- triptans
- ergots (i.e DHE)
**do not use ergots and triptans together
why do you admin dopamine antagonists in migraine emergency
help with N/V
interferes with dopaminergic system–> specific antimigraine effect
example: metoclopramide, chlorpromazine
risk of EPS (dytonia, hyperkinesis)… can give diphenhydramine prophylaxis
why do you administer ergots in migraine emergency
i.e DHE
are 5-HT agonists wth wide range of effects
side effects are N, dizziness, paresthesias, abdominal/chest pain, rebound
contra-indications are pregnancy, HTN, renal and hepatic failure
DO NOT USE WITH TRIPTANS
little pediatric data
when should you use prevention meds for migraines
- frequent (more than 1-2 per week or 3-4 a month)
- headache related disability that interferes with school attendance, daily routine, daily activities
also if…
acute tx options are ineffective or not tolerated or overused, or if the patient has extremely intensive, prolonged (more than 48 hour), hemiplegic or basilar type migraines or severe aura
what natural products can prevent migraine
- riboflavin (vitamin B2)
- magnesium
- coenzyme Q10–expensive with questionable efficacy
- butterbur
- -currently not recommended due to risk of liver toxicity
what are the preventative migraine meds
- amitriptylline
- -TCA with antichoinergic SEs and need to rule out long QT before starting - propanolol
- -beta blocker… avoid in patients with asthma and heart block and in highly athletic patients - flunarazine
- -calcium channel blocker, efficacy high in hemiplegic migraine - cyproheptadine
- -FIRST LINE in less than 6 years old…often useful for migrain equivalents, but causes sedation adn weight gain - valproic acid
- -causes weight gain, risk of hepatotoxicity, plately dysfunction, teratogen - topiramate
- -can cause anorexia, renal stones, cognitive slowing
what are tension type headaches
may be similar pathophysiologic spectrum as migraine
bilateral, pressing/tightening quality
mild to moderate severity
not exacerbated by activity
no N/V usually
photophobia OR phonophobia may be present but NOT BOTH
tx with simple analgesics; risk of med overuse and rebound
what is another name for idiopathic intracranial HTN
pseudotumour cerebri or benign intracranial HTN–not not benign
what is idiopathic intracranial HTN
raised pressure (above 2oo mm/water) in absence of intracranial lesions
caused by/associated with obesity, endocrinopathies (hypothyroid, addisons), meds (tetracycline, contraceptives, oral steroids), vitamin A intox, SLE
if untreated can lead to vision loss
how do you treat idiopathic intracranial HTN
acetazolamide
optic neuronal sheath fenestration
therapeutic LP
name the trigeminal autonomic cephalagies
cluster headaches
paroxysmal hemicrania
what are cluster headaches
uncommon in kida/adolescents
m:f is 3:1
severe unilateral retroorbital pain lasting 15-180 minutes
ipsilateral conjunctival injection, lacrimation, nasal congestion, rhinorrhea, edema, sweating, miosis, ptosis
may have attacks that last weeks to months
how do you treat cluster headaches
inhaled oxygen
sumatriptan SC or IM
verapamil, valproic acid, lithium
what is paroxysmal hemicrania
intense periorbital or temporal unilateral pain for 2-30 min
up to dozens of times a day
may also have ipsilateral autonomic features
tx of paroxysmal hemicrania
responsive to indomethacin
what is an ice pick headache
momentary (seconds)
sharp/jabbing
once or several times a day at irregular intervals
periorbital or temporal
more common in patients with migraine or cluster headaches
treatment of ice pick headache
indomethacin
what are the two components of conciousness
wakefulness and awareness
what is obtundation
mild to mod reduction in alertness
decreased interest in environment
appear sleepy, may sleep if left alone
what is stupor
unresponsiveness with little to no spontaneous movement
aroused only by vigorous stimulation
what is coma
unrousable, sustained unconsciousness
dysfunction of ascending reticular activating system and/or both cerebral hemispheres
what is a vegetative state
complete unawareness of self
sleep wake cycle present though irregular
some preservation of autonomic functions
cannot interact with environment
irregular periods of wake that cycle with sleep
may exhibit spontaneous sounds or emotional expressions–but sustained response to enviro stimuli is lacking
no visual tracking
what is a minimally conscious state
minimal awareness of self or environment but CAN interact with the environment
may follow simple commands
yes and no answers
purposeful behavior in respnose to stimuli like smiling, vocalization, reaching
what is brain death
permanent, irreversible absence of all brain function
includes brainstem functions
no respiratory drive
hypothermia, anesthesia, sedatives may mimic this though the brainstem responses and respiratory drive are often present in comatose patients
how is the glasgow coma scale adjusted for children
- eye opening–> out of 4. 4–spontaneous, 3–to SOUND (rather than command)
- verbal–> out of 5
5–age appropriate verbalization
4–cries but consolable
3–irritable, uncooperative, aware of environment
2–irritant persistent cry, inconsistently inconsolable
1–unaware of environment/parents, inconsolable - motor–> out of 6
6–obeys or has spontaneous movement
dilated, symmetric pupils suggests…
hypoxic ischemic injury
dilated, asymmetric pupils suggests..
compression (i.e herniation)
small, reactive pupils suggests…
drug ingestions, metabolic disorders, hypothalamic injury
midposition and unresponsive pupils suggests…
midbrain lesion
what does decorticate (flexion) posturing suggest
higher lesions (midbrain and above)
what does decerebrate (extensor) posturing suggest
lower brainstem lesion (midbrain or below)
tends to carry poorer prognosis
what is tone
resistance to passive muscle stretch
how is tone controlled
mediated by elements of both central and peripheral nervous systems
facilitators and inhibitors–> sum total results in clinically observed and measured tone
lesion localization results from functional anatomy
what are you looking at in particular in the exam of a floppy infant
dysmorphic features other affected systems (cardiomyopathy etc) joint contractures pectus excavatum flat occiput hip subluxation
by what age should head track with body when being lifted by the arms from supine position (traction response)
by one month
what are some causes of hypotonia in the infant
- brain–>benign congenital hypotonia, genetic (i.e prader willi, trisomy), HIE, malformation, inborn error of metabolism
- spinal cord–> ischemia, trauma
- anterior horn cell–> SMA
- peripheral nerve–> hereditary neuropathy
- NMJ–> infantile botulism, transient neonatal MG, congenital MG
- muscle–> congenital myopathy, congenital muscular dystrophy, congenital myotonic dystrophy, metabolic myopathy
what signs would suggest a central cause of hypotonia
impaired consciousness or seizures
dysmorphic features
fisting of hands/scissoring of legs
presence or exaggeration of moro reflex
norma or brisk tendon reflexes
what signs would suggest lower motor neuron cause of hypotonia
absent or decreased tendon reflexes
absent moro
fasciculations
muscle atrophy
