Neurology

Question 1

What are the recommendations for folic acid supplementation?

Answer 1

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

Question 2

What is an encephalocele and what is the treatment?

Answer 2

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

Question 3

What are the signs and symptoms of hydrocephalus

Answer 3

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

Question 4

How is hydrocephalus confirmed and how is it treated

Answer 4

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

Question 5

What is spina bifida occulta and what is the treatment

Answer 5

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

Question 6

What is a meningocele and what is the treatment

Answer 6

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

Question 7

What is a myelomeningocele and what can the symptoms be?

Answer 7

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

Question 8

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

Answer 8

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

Question 9

What are the three types of Arnold-Chiari malformation

Answer 9

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

Question 10

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

Answer 10

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

Question 11

What is Klippel-Feil Syndrome

Answer 11

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

Question 12

What is lissencephaly?

Answer 12

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

Question 13

What is schizencephaly

Answer 13

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

Question 14

What is Porencephaly

Answer 14

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

Question 15

What is holoprosencephaly

Answer 15

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

Question 16

What is the prognosis for agenesis of the corpus callosum?

Answer 16

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

Question 17

What are the four types of cerebral palsy

Answer 17

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

Question 18

What are signs of spastic cerebral palsy

Answer 18

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

Question 19

What is commonly associated with cerebral palsy?

Answer 19

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

Question 20

How is cerebral palsy diagnosed and treated?

Answer 20

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

Question 21

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

Answer 21

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

Question 22

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

Answer 22

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

Question 23

Name the major causes of ischemic stroke in children

Answer 23

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

Question 24

What defects cause prothrombotic ischemic strokes

Answer 24

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

Question 25

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

Answer 25

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

Question 26

What is Moyamoya disease

Answer 26

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

Question 27

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

Answer 27

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

Question 28

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

Answer 28

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

Question 29

What are some causes of hemorrhagic stroke in children

Answer 29

trauma vascular malformations venous malformations are the most common

Question 30

What are saccular aneurysms associated with

Answer 30

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

Question 31

What are the symptoms of cerebral vein thrombosis

Answer 31

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

Question 32

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

Answer 32

``` 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 ```

Question 33

How should a pond fracture be managed

Answer 33

do not require surgical intervention unless depressed > 0.5 cm

Question 34

What is diffuse axonal injury

Answer 34

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

Question 35

What is an epidural hematoma and what are the symptoms?

Answer 35

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

Question 36

What is typically seen in a generalized seizure

Answer 36

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

Question 37

What is seen in myoclonic seizures

Answer 37

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

Question 38

what is seen in juvenile myoclonic epilepsy

Answer 38

``` 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 ```

Question 39

What is seen in absence seizures

Answer 39

``` 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

Question 40

What is seen in Atonic or akinetic seizures

Answer 40

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

Question 41

What is seen in a simple partial seizure

Answer 41

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

Question 42

What is seen in a complex partial seizure

Answer 42

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

Question 43

What are infantile spasms

Answer 43

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

Question 44

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

Answer 44

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

Question 45

What is Lennox gastaut syndrome

Answer 45

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

Question 46

Describe Rasmussen syndrome

Answer 46

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

Question 47

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

Answer 47

idiopathic generalized tonic-clonic seizures | > 1 type of seizure

Question 48

what are some side effects of valproic acid

Answer 48

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

Question 49

What type of seizures would you use carbamazepine to treat?

Answer 49

simple partial seizures | complex partial seizures

Question 50

What are some side effects of carbamazepine

Answer 50

dizziness, drowsiness, SIADH, hepatoxicity | avoid erythromycin as it elevates levels

Question 51

What is the drug of choice for absence seizures

Answer 51

ethosuximide

Question 52

What is status epilepticus

Answer 52

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

Question 53

What are some causes of neonatal seizures

Answer 53

``` 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 ```

Question 54

what is the treatment for neonatal seizures

Answer 54

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

Question 55

What is a febrile seizure

Answer 55

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

Question 56

what is a simple febrile seizure

Answer 56

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

Question 57

What is a complex febrile seizure

Answer 57

focal findings last > 15 min recurs in 24 hours

Question 58

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

Answer 58

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

Question 59

What are the risks for recurrence of a febrile seizure

Answer 59

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

Question 60

What is the criteria for migraine without aura

Answer 60

``` 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 ```

Question 61

What are the criteria for migraine with aura

Answer 61

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

Question 62

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

Answer 62

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

Question 63

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

Answer 63

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

Question 64

what is spinal muscular atrophy

Answer 64

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

Question 65

what are the three types of spinal muscular atrophy

Answer 65

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,