Neurology Flashcards
What are the recommendations for folic acid supplementation?
For all women of reproductive age 0.4 mg of folic acid a day
for women who have had a prior pregnancy with a neural tube defect or are on an anticonvulsant should take 4 mg of folic acid daily
What is an encephalocele and what is the treatment?
Herniation of the brain, meninges, CSF through a defect in the skull usually the occipital region.
Prognosis is dependent on what is herniated through the sac.
Eval with CT/MRI
Close defect as early as possible
What are the signs and symptoms of hydrocephalus
headache, early morning headache with nausea and vomiting
personality and behavior changes
nerve palsies particularly 3rd and 6th cranial nerves
excessive head growth
“setting sun sign” with limitation in upward gaze
How is hydrocephalus confirmed and how is it treated
US in infants
CT/MRI in children
Surgery with VP shunt
What is spina bifida occulta and what is the treatment
midline defect of the vertebral bodies with out protrusion of the spinal cord or meninges
most are asymptomatic
some have patches of hair, lipoma, skin color changes over the area
plain xr of the spine show the defect
no treatment is needed usually
What is a meningocele and what is the treatment
herniation of the meninges through a defect in the posterior vertebral arches
most are well covered with skin
you can delay surgery in those with normal neuro exam and complete skin covering the defect to allow for radiological review of spine
if leaking CSF or incomplete skin covering need immediate surgical repair to prevent meningitis
What is a myelomeningocele and what can the symptoms be?
neuro tube defect involving skin, vertebral bodies, nerve roots, spinal cord and meninges
defects depend on location
sacral: bowel, bladder incontinence, perineal anesthesia
lumbar: paralysis of lower extremities, no sensation, neurogenic bowel and bladder
What is the treatment of myelomeningocele and what is it associated with?
80-85% have hydrocephalus with Arnold Chiari type 2 malformation
surgery to correct herniation defect
many need VP shunt
many patients require bladder catheterization
What are the three types of Arnold-Chiari malformation
Type 1: cerebellar tonsils or vermis are pushed down below the foramen magnum, > 0.5 cm displacement
Type 2: 4th ventricle and lower medulla are pushed below the foramen magnum
Type 3: herniation of the cerebellum through a cervical spina bifida defect
What are the symptoms of Arnold Chiari malformation and what is the treatment?
Type 1: weakness, spasticity of the extremities, sensory loss, dysphagia, vertigo, sleep apnea, ataxia, headache and neck pain
no treatment, if asymptomatic
Type 2: have hydrocephalus and often myelomeningocele in addition to above, also have lower cranial nerve defects
surgical correction
What is Klippel-Feil Syndrome
- short neck
- limited neck motion
- low occipital hair line
cervical and upper thoracic vertebrae are fused into a boney block
often there are several other anomalies: macrocephaly, hydrocephalus, meningocele, intellectual disability, cardiac, GU, deafness, musculoskeletal
What is lissencephaly?
brain with no cerebral convolutions and a poorly formed sylvian fissure
those who survive to delivery: failure to thrive, profound delay, seizures
occurs with miller dieker syndrome
What is schizencephaly
unilateral or bilateral clefts with in the cerebral hemispheres
seizures, severe intellectual disability, spastic quadriparesis
What is Porencephaly
cysts or cavities with in the brain
may be a developmental defect or, may be secondary to infarction
“true cysts” are located in the sylvian fissure region and communicate with the subarachnoid space or ventricular system
seizures, intellectual disability, blindness, spastic quadriplegia
some may be asymptomatic
Pseudo porencephaly usually is due to infarction or hemorrhage
What is holoprosencephaly
defective cleavage of the prosencephalon
facial abnormalities including cyclopia are common
most infants die in the first few months
What is the prognosis for agenesis of the corpus callosum?
Can be AD or X linked recessive, associated with trisomy 8,18
linked with maternal cocaine use
some are asymptomatic others with severe intellectual disability
What are the four types of cerebral palsy
spastic
dyskinetic : chorea athetoid movements that can not be controlled but go away during sleep
ataxic: wide based gait, can’t do finger to nose, no functional decline, diagnosis of exclusion
mixed: spasticity, chorea athetoid movements
What are signs of spastic cerebral palsy
upper motor neuron signs, abnormal reflexes, extensor plantar response
spastic diplegia is associated with a history of PVL, or IVH
What is commonly associated with cerebral palsy?
seizures in 25-40%
intellectual disability
anticipate visual, hearing and speech disorders
How is cerebral palsy diagnosed and treated?
non progressive disorder, diagnosed clinically
occupational and physical therapy
diazepam, baclofen, dantrolene for spasticity
dorsal rhizotomy in children whose spasticity is worse in the lower extremities with relatively normal upper extremity function
How do strokes present children? Break it down by age group
Strokes in utero: early onset hand dominance or hemiplegic CP
Perinatal stroke: focal neonatal seizures; focal neuro deficits do not show for weeks to months
Children < 2: seizure and hemiparesis
Older children: acute focal neurological deficit. Fever and AMS
What three other things should one consider in a child with acute hemiplegia other than stroke
transient postictal hemiparesis (Todd paralysis) {MRI never shows infarction}
Complicated migraine
Alternating hemiplegia: children < 2, hemiplegia lasts for minutes to hours, with alternating weakness, seizures are common but do not occur during episodes of weakness
Name the major causes of ischemic stroke in children
cardiac disorders Prothombotic disorders sickle cell anemia cervicocephalic arterial dissection CNS vasculitis metabolic etiologies : fabry disease, homocystinuria
What defects cause prothrombotic ischemic strokes
1/3 of children with ischemic stroke
acquired antiphospholipid antibody, (anticardiolipin antibody or lupus anticoagulant)
OCP, nephrotic syndrome, liver disease
activated protein C resistance (factor v Leiden): more common than protein c or protein s deficiency
What age group with sickle cell disease has the highest incidence of stroke? What are the symptoms in these children? Can stroke be prevented?
2-5 year olds
many are asymptomatic but have changes on MRI
recurrence of stroke is very common,
prevention of recurrence with regular transfusion
and use transcranial doppler to predict which children are at greater risk of stroke
What is Moyamoya disease
chronic occlusive cerebrovascular disease
associated with sickle cell, neurofibromatosis type 1, trisomy 21 and cranial irradiation
extensive collateral vessels around the circle of Willis from prior occlusion of arteries
What is the most common cause of CNS vasculitis and what are some other causes of CNS vasculitis that results in stroke?
most common cause: bacterial meningitis
varicella causes a post infectious vasculopathy
CNS lupus, inflammatory bowel disease, Takayasu arteritis, cocaine and diet pill causing vasospasm
What are the causes of neonatal cerebral infarction and how does it present?
embolic in the left middle cerebral artery
maybe from the placenta, prothrombotic conditions
hypoxic ischemic encephalopathy
infant with seizures in first 3-4 days of life
What are some causes of hemorrhagic stroke in children
trauma
vascular malformations
venous malformations are the most common
What are saccular aneurysms associated with
coarctation of the aorta, polycystic kidney disease, Ehlers-Danlos syndrome and marfan syndrome
What are the symptoms of cerebral vein thrombosis
neonates: seizures
Older children: headache and vomiting, 6th nerve palsy depending on the location of the thrombus
basically signs of increased intracranial pressure,
When should you suspect an underlying intracranial lesion and thus would need to do a CT scan?
depressed or comminuted skull fracture loss of consciousness lasting at least 5 minutes altered mental status or irritability bulging fontanelle focal neurologic signs or deteriorating neurologic condition vomiting non impact seizures children < 6 months
How should a pond fracture be managed
do not require surgical intervention unless depressed > 0.5 cm
What is diffuse axonal injury
usually is seen with acceleration-deceleration injury
shearing at the junction of gray and white matter
causes prolonged alteration in cognitive function and alertness
What is an epidural hematoma and what are the symptoms?
bleeding between the skull and the dura
seen with temporal bone fracture and tearing of middle meningeal artery
tear of bridging veins or dural sinuses
looks like a lens on CT
usually loss of consciousness that is regained followed by deterioration
What is typically seen in a generalized seizure
involve both cerebral hemispheres from the beginning of the seizure
most common type in childhood
abrupt onset
loss or alteration of consciousness
no aura
EEG shows bilateral and synchronous epileptiform activity
bilateral symmetric motor activity associated with changes in muscle tone
What is seen in myoclonic seizures
short duration
rapid, bilateral symmetric muscle contractions
isolated or repetitive jerks
likelihood of the patient falling
what is seen in juvenile myoclonic epilepsy
morning myoclonic jerks \+/- history of absence seizures generalized tonic-clinic seizures occurring just after awakening or during sleep normal intelligence family history of similar seizures onset 8-20 years of age
What is seen in absence seizures
extremely short lapses of awareness amnesia during the episodes short duration (10 sec or less) abrupt onset and end flickering of the eyelids, staring or eye rolling
atypical or complex absence seizures can have brief jerks of eyelids and limbs, transient change in tone, pupillary dilation, skin color changes, tachycardia
What is seen in Atonic or akinetic seizures
sudden and complete loss of tone in the limbs, neck and trunk muscles
can be brief or longer duration
loss of consciousness
awareness returns quickly after the attack
myoclonic jerks may precede loss of muscle tone
What is seen in a simple partial seizure
focal motor, asynchronous tonic or clonic movements the involve the face, neck and extremities
aura, hallucinations, feelings of dread prior to the seizures
no loss of consciousness
What is seen in a complex partial seizure
alteration in consciousness
automatisms
can have an aura prior to the seizure
there is a post ictal time after the seizure
What are infantile spasms
children < 1 year of age
sudden simultaneous flexion of their head and trunk with flexion and adduction of their extremities
initiated or aggravated by transitions from sleep to wakefulness, feeding, handling or emotions
What is seen in the EEG of children with infantile spasms? What is the treatment and prognosis?
EEG: hypsarrhythmia
resolve over time even with out therapy but those who survive have severe intellectual disability and other types of seizure disorders
Some use ACTH therapy, maybe prednisone
What is Lennox gastaut syndrome
severe seizures
intellectual disability
EEG shows generalized, bilateral synchronous sharp wave and slow wave complexes in a repetitive fashion in long runs
difficult to treat with poor prognosis
Describe Rasmussen syndrome
immunologic process involving one hemisphere of the brain
seizures become focal, unremitting and limited to one part or side of the body eventually deteriorates to a stable neurologic deficit of hemiparesis and diminished intelligence and hemianopia
the simple focal status epilepticus is also called epilepsia partialis continua
What type of seizures would you use valproic acid to treat?
idiopathic generalized tonic-clonic seizures
> 1 type of seizure
what are some side effects of valproic acid
hepatotoxicity in children < 3 especially if they have a metabolic disorder
teratogen
decreases l-carnitine levels, L-carnitine can be used to treat valproic acid toxicity
What type of seizures would you use carbamazepine to treat?
simple partial seizures
complex partial seizures
What are some side effects of carbamazepine
dizziness, drowsiness, SIADH, hepatoxicity
avoid erythromycin as it elevates levels
What is the drug of choice for absence seizures
ethosuximide
What is status epilepticus
repeated seizures with out regaining consciousness or seizure prolonged for at least 20 minutes
What are some causes of neonatal seizures
perinatal complications like HIE intraventricular hemorrhage hypoglycemia hypocalcemia infectious (all babies with seizure should get a lumbar puncture) congenital brain malformations benign neonatal sleep myoclonus familial neonatal seizures
what is the treatment for neonatal seizures
correct potential electrolyte abnormalities
diazepam
if occurring frequently: phenobarbital
can wean anti-seizure medication 1 month after the last seizure in many neonates
What is a febrile seizure
seizure between 6 months and 60 months
no intracranial infection
child has never had a seizure before with out a fever
what is a simple febrile seizure
lasts for less than 15 minutes
is non focal
does not recur
What is a complex febrile seizure
focal findings
last > 15 min
recurs in 24 hours
When should an LP be performed in a child with a suspected febrile seizure
presence of meningeal signs and symptoms
children 6-12 months with unknown or incomplete immunization status
those who received antibiotics prior to seizure
What are the risks for recurrence of a febrile seizure
first seizure before 18 months
1st degree relative with febrile seizures
history of seizure with only a modest temperature elevation
suffered a seizure after having the fever for only a very short duration
What is the criteria for migraine without aura
a) at least five attacks fulfilling B through D
B) migraine attack lasting 2-72 hours
C) at least two of the following
1. unilateral or bilateral location
2. pulsating quality
3. moderate to severe intensity
4. aggravation by routine physical activity
D) at least 1 of the following
1. nausea and/or vomiting
2. photophobia and phonophobiaWhat are the criteria for migraine with aura
A) at least 2 attacks lasting 2-72 hours
B) at least 3 of the following
1. 1 or more fully reversible aura or symptoms indicating focal cortical and or brain stem dysfunction
2. at least 1 aura developing gradually over more than 4 minutes or 2 or more symptoms occurring in succession
3. no auras lasting more than 60 min
4. headache follows in less than 60 minutes
In what kind of headache in children should prompt a work up?
occipital headaches as these are rare and usually have a structural cause
When are some times to order a CT/MRI for a child with a headache?
recent school failure or change in behavior
abnormal neurologic signs
fall off in growth
headache awakens child from sleep
early morning headache with increase in severity and frequency
headache with focal seizures
migraine followed by seizure
headache associated with vomiting in the absence of family history of migraine
cluster headaches in children
brief coughing episode that results in a headache
increasing or “crescendo headache”
persistent focal headaches
increase in head circumference
increase with valsalva
what is spinal muscular atrophy
AR, SMN-1 gene
symmetric muscle weakness
proximal muscles affected more, legs more affected than arms
no sensory or cognitive deficits
what are the three types of spinal muscular atrophy
Type 1: severe infantile (werdnig-hoffman) presents < 6 months, tongue fasciculations, hypotonia and difficulty feeding
Type 2: healthy at birth and meet milestones then, lose these skills by 2.
Type 3: mild SMA (Kugelberg-Welander) presents between 2 and 17 degree of deficit corresponds to age of onset,
