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Pediatric Board > Neurology > Flashcards

Neurology Flashcards

1
Q

What are the recommendations for folic acid supplementation?

A

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

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2
Q

What is an encephalocele and what is the treatment?

A

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

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3
Q

What are the signs and symptoms of hydrocephalus

A

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

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4
Q

How is hydrocephalus confirmed and how is it treated

A

US in infants
CT/MRI in children
Surgery with VP shunt

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5
Q

What is spina bifida occulta and what is the treatment

A

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

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6
Q

What is a meningocele and what is the treatment

A

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

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7
Q

What is a myelomeningocele and what can the symptoms be?

A

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

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8
Q

What is the treatment of myelomeningocele and what is it associated with?

A

80-85% have hydrocephalus with Arnold Chiari type 2 malformation
surgery to correct herniation defect
many need VP shunt
many patients require bladder catheterization

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9
Q

What are the three types of Arnold-Chiari malformation

A

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

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10
Q

What are the symptoms of Arnold Chiari malformation and what is the treatment?

A

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

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11
Q

What is Klippel-Feil Syndrome

A
  1. short neck
  2. limited neck motion
  3. 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
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12
Q

What is lissencephaly?

A

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

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13
Q

What is schizencephaly

A

unilateral or bilateral clefts with in the cerebral hemispheres
seizures, severe intellectual disability, spastic quadriparesis

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14
Q

What is Porencephaly

A

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

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15
Q

What is holoprosencephaly

A

defective cleavage of the prosencephalon
facial abnormalities including cyclopia are common
most infants die in the first few months

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16
Q

What is the prognosis for agenesis of the corpus callosum?

A

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

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17
Q

What are the four types of cerebral palsy

A

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

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18
Q

What are signs of spastic cerebral palsy

A

upper motor neuron signs, abnormal reflexes, extensor plantar response
spastic diplegia is associated with a history of PVL, or IVH

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19
Q

What is commonly associated with cerebral palsy?

A

seizures in 25-40%
intellectual disability
anticipate visual, hearing and speech disorders

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20
Q

How is cerebral palsy diagnosed and treated?

A

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

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21
Q

How do strokes present children? Break it down by age group

A

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

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22
Q

What three other things should one consider in a child with acute hemiplegia other than stroke

A

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

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23
Q

Name the major causes of ischemic stroke in children

A 
cardiac disorders
Prothombotic disorders
sickle cell anemia
cervicocephalic arterial dissection
CNS vasculitis
metabolic etiologies : fabry disease, homocystinuria
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24
Q

What defects cause prothrombotic ischemic strokes

A

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

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25
Q

What age group with sickle cell disease has the highest incidence of stroke? What are the symptoms in these children? Can stroke be prevented?

A

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

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26
Q

What is Moyamoya disease

A

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

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27
Q

What is the most common cause of CNS vasculitis and what are some other causes of CNS vasculitis that results in stroke?

A

most common cause: bacterial meningitis
varicella causes a post infectious vasculopathy
CNS lupus, inflammatory bowel disease, Takayasu arteritis, cocaine and diet pill causing vasospasm

28
Q

What are the causes of neonatal cerebral infarction and how does it present?

A

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

29
Q

What are some causes of hemorrhagic stroke in children

A

trauma
vascular malformations
venous malformations are the most common

30
Q

What are saccular aneurysms associated with

A

coarctation of the aorta, polycystic kidney disease, Ehlers-Danlos syndrome and marfan syndrome

31
Q

What are the symptoms of cerebral vein thrombosis

A

neonates: seizures
Older children: headache and vomiting, 6th nerve palsy depending on the location of the thrombus
basically signs of increased intracranial pressure,

32
Q

When should you suspect an underlying intracranial lesion and thus would need to do a CT scan?

A 
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
33
Q

How should a pond fracture be managed

A

do not require surgical intervention unless depressed > 0.5 cm

34
Q

What is diffuse axonal injury

A

usually is seen with acceleration-deceleration injury
shearing at the junction of gray and white matter
causes prolonged alteration in cognitive function and alertness

35
Q

What is an epidural hematoma and what are the symptoms?

A

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

36
Q

What is typically seen in a generalized seizure

A

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

37
Q

What is seen in myoclonic seizures

A

short duration
rapid, bilateral symmetric muscle contractions
isolated or repetitive jerks
likelihood of the patient falling

38
Q

what is seen in juvenile myoclonic epilepsy

A 
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
39
Q

What is seen in absence seizures

A 
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

40
Q

What is seen in Atonic or akinetic seizures

A

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

41
Q

What is seen in a simple partial seizure

A

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

42
Q

What is seen in a complex partial seizure

A

alteration in consciousness
automatisms
can have an aura prior to the seizure
there is a post ictal time after the seizure

43
Q

What are infantile spasms

A

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

44
Q

What is seen in the EEG of children with infantile spasms? What is the treatment and prognosis?

A

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

45
Q

What is Lennox gastaut syndrome

A

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

46
Q

Describe Rasmussen syndrome

A

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

47
Q

What type of seizures would you use valproic acid to treat?

A

idiopathic generalized tonic-clonic seizures

> 1 type of seizure

48
Q

what are some side effects of valproic acid

A

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

49
Q

What type of seizures would you use carbamazepine to treat?

A

simple partial seizures

complex partial seizures

50
Q

What are some side effects of carbamazepine

A

dizziness, drowsiness, SIADH, hepatoxicity

avoid erythromycin as it elevates levels

51
Q

What is the drug of choice for absence seizures

A

ethosuximide

52
Q

What is status epilepticus

A

repeated seizures with out regaining consciousness or seizure prolonged for at least 20 minutes

53
Q

What are some causes of neonatal seizures

A 
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
54
Q

what is the treatment for neonatal seizures

A

correct potential electrolyte abnormalities
diazepam
if occurring frequently: phenobarbital
can wean anti-seizure medication 1 month after the last seizure in many neonates

55
Q

What is a febrile seizure

A

seizure between 6 months and 60 months
no intracranial infection
child has never had a seizure before with out a fever

56
Q

what is a simple febrile seizure

A

lasts for less than 15 minutes
is non focal
does not recur

57
Q

What is a complex febrile seizure

A

focal findings
last > 15 min
recurs in 24 hours

58
Q

When should an LP be performed in a child with a suspected febrile seizure

A

presence of meningeal signs and symptoms
children 6-12 months with unknown or incomplete immunization status
those who received antibiotics prior to seizure

59
Q

What are the risks for recurrence of a febrile seizure

A

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

60
Q

What is the criteria for migraine without aura

A 
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 phonophobia
61
Q

What are the criteria for migraine with aura

A

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

62
Q

In what kind of headache in children should prompt a work up?

A

occipital headaches as these are rare and usually have a structural cause

63
Q

When are some times to order a CT/MRI for a child with a headache?

A

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

64
Q

what is spinal muscular atrophy

A

AR, SMN-1 gene
symmetric muscle weakness
proximal muscles affected more, legs more affected than arms
no sensory or cognitive deficits

65
Q

what are the three types of spinal muscular atrophy

A

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,

Pediatric Board (11 decks)

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