Neurology Flashcards
1
Q
Most common type of pediatric seizure
A
Febrile seizure
2
Q
what percentage of patients will have recurrence of simple febrile seizure
A
1/3
3
Q
What is the biggest risk factor for febrile seizures
A
first or 2nd degree relative with history of febrile seizure
4
Q
Simple febrile seizure includes what 4 criteria
A
- generalized
- <15 min
- does not recur in 24 hours
- no post-ictal abnormalities
5
Q
Complex febrile seizure includes what 4 criteria
A
- focal
- > 15 min
- > or equal to 2 in 24 hours
- focal neurological deficits
6
Q
what is the risk of the general population for developing epilepsy? after a simple febrile seizure what is your risk?
A
1%
2-3% (2x baseline)
7
Q
What are 4 factors that increase your risk of epilepsy after a simple febrile seizure
A
- Family history
- complex febrile seizure
- age < 1
- developmental delay
8
Q
febrile convulsions >90 minutes have been associated with?
A
increased risk of medial temporal sclerosis
9
Q
what is the recurrence risk after 1 unprovoked seizure?
after 2 unprovoked seizures?
A
40%
80%
10
Q
What percentage of children with epilepsy will outgrow their disorder?
A
50%
11
Q
Name 5 treatable neonatal seizures
A
- Pyridoxine dependent seizures
- Pyridoxal phosphate dependent seizures
- biotinidase deficiency
- folinic acid responsive seizures
- glucose transporter type 1 syndrome
12
Q
What will you see on EEG for infantile spasms
A
Hypsarrythmia
13
Q
How do you treat infantile spasms (3)
A
Vigabatrin, ACTH, high dose oral steroids
14
Q
Name 2 syndromes associated with infantile spasms
A
TS and Down syndrome
15
Q
What is the classic EEG finding for absence seizures
A
3 hx spike and wave
16
Q
What do you use to treat Absence seizures
A
Ethosuximide first line
valproic acid second line
17
Q
What percentage of children will outgrow absence seizures by adolescence
A
70%
18
Q
What is the name of the seizure that presents with nocturnal focal seizures of semi-face lasting 1-2 min without loss of consciousness
A
Benign rolandic epilepsy
19
Q
What will you see on EEG for Benign rolandic epilepsy
A
Centro-temporal spikes
20
Q
At what age do seizures stop for Benign rolandic epilepsy
A
15 yo
21
Q
What is JME (Juvenile Myoclonic Epilepsy)
A
onset late in childhood/early adolescence
jerking in the morning (myoclonus)
later present with GTC seizure and can have absence seizures
tx: valproic acid or lamotrigine
22
Q
what is the prognosis of Juvenile Myoclonic Epilepsy
A
Lifelong epilepsy requiring treatment
23
Q
What is the general dosing for phenobarbital
A
3-5mg/kg/day at bedtime
24
Q
what is the most common adverse event for phenobarbital
A
sedation
25
why do you not use valproic acid <2 years of age
increase risk of liver toxicty
| If mitochondrial or metabolic disorder causing seizures, may trigger drug-induced liver failure
26
Name 2 contraindications to carbamazepine or phenytoin use
myoclonus/ absence seizures
27
Name one contraindication to valproic acid use
metabolic disease
28
Name side effects of valproic acid (8)
weight gain, hair loss, PCOS, thrombocytopenia, LFTS, pancreatitis, hyperammonia, tremor
29
Name 5 side effects of phenytoin
gingival hypertrophy, coarsening of facies, hirsutism, liver toxicity, SJS, ataxia
30
Name 4 side effects of carbamazepine
SJS, ataxia, agranulocytosis, SIADH
31
Name 1 side effect of Ethosuximide
agranulocytosis
32
Name 3 side effects of Topimax (topiramate)
metabolic acidosis, weight loss, kidney stones*
| cognitive dysfunction, fever, glaucoma, hypohidrosis
33
Which anticonvulsant has the greatest risk of SJS
Lamotrigine
34
Name 2 side effects of Keppra
behavioural changes- aggression/ "keppra rage"
| suicidal ideation
35
Breath holding spells are associated with
Iron deficiency anemia
36
By what age do breath holding spells resolve
100% resolve by 8 years pf age
37
Describe benign infantile myoclonus
sudden brief asymmetrical myoclonic jerks, only during sleep
38
What are the diagnostic criteria for pediatric migraine?
mnemonic SULTANS
5 or more attacks
lasting 1-72 hours
2/4 of the following characteristics or more:
S- severity (moderate-severe pain)
U- unilateral or bilateral (frontotemporal not occipital)
T- Throbbing/pulsating quality
A- Aggravated by activity or causing avoidance of physical activity
1 of these:
N- Nausea with or without vomiting
S- Photophobia AND phonophobia
Cannot be explained by another disorder
39
Name 4 childhood migraine variants
cyclic vomiting
abdominal migraine
paroxysmal torticollis of infancy
paroxysmal vertigo
40
what is a retinal migraine
transient episodes of blindness or scotoma
41
what is hemiplegic migraine
```
rare aura
transient hemiplegia (unilateral weakness) followed by headache
```
42
basilar migraine- now called migraine with brainstem aura
transient ataxia and or cranial nerve deficits
| vertigo, tinnitus, diplopia, blurred vision, scotoma, ataxia, occipital headache
43
Diagnostic criteria for tension headache
A. At least 10 episodes of headache occurring on < 1 day/mo on average (<12 days/yr) and fulfilling criteria B to D
B. Lasting from 30 min to 7 days
C. At least 2 of the following 4 characteristics:
1. Bilateral location
2. Pressing or tightening (nonpulsating) quality
3. Mild or moderate intensity
4. Not aggravated by routine physical activity, such as walking or climbing stairs
D. Both of the following:
1. No nausea or vomiting
2. No more than 1 of photophobia or phonophobia
E. Not better accounted for by another ICHD-3 beta diagnosis
44
What is the criteria for medication overuse headache
headache present for more than 15d/month for longer than 3 months and intake of simple analgesic on more than 15d/month and/or prescription medication (triptans or combination medications) for >10 d/month
often presents as chronic daily headache
45
Headache due to raised ICP present with
postural (worse in the morning and when lying down)
progressive worsening severity
optic disk edema +/- cranial VI palsy (papilledema on fundoscopy)
46
Causes of headache due to raised ICP (4)
tumor
sinus venous thrombosis
meningitis/encephalitis
pseudotumor cerebri (TCA use, steroids, obesity, SLE, hyper/hypothyroidism)
47
When should you image a child with headache? (8)
```
sudden onset of severe h/a (worst ever of their life)
focal abnormalities on neurological exam
pain that wakes patient from sleep
paint worst in mornings +/- vomiting
change in chronic headache type
acutely progressively worsening headache
papilledema
```
Nelsons:
abnormal neurologic exam
abnormal or focal neurologic signs/symptoms
seizures or very brief aura (<5min)
unusual headache in children (hemiplegic, basilar, cluster headache)
headache in children <6 or child who cannot adequately describe headache
headache worst on first awakening or awakens child from sleep
migrainous headache in a child with no family history of migraine
brief cough headache in a child or adolescent
48
Name lifestyle modifications/ supplements for headache management
```
sleep
exercise
avoid caffeine
regular meals
avoid triggers
magnesium, riboflavin, coQ10
```
49
Name one treatment for Pseudotumor cerebri
Diamox
50
What is the gene deletion for Duchenne Muscular Dystrophy
xp21 deletion
51
What is the dystrophin gene
structural protein in muscle cell
52
How do children with DMD present?
present at 3-4 with toe-walking, waddling gait, difficulty risking from floor/walking stairs
progressive proximal muscle weakness
53
What would you look for on physical exam for patient with DMD
Gower's sign
gastrocnemius pseudohypoertrophy
CK >10 000
54
Diagnostic criteria for NF Type 1
2 of 7
C- cafe au lait (>6 and >5mm)
> 5 mm in greatest diameter in prepubertal individuals and > 15 mm in greatest diameter in postpubertal individuals
R- first degree relative with NF1
O- optic glioma
P- pseudoarthrosis
L- lisch nodules (2 or more)
A- axillary freckling or inguinal
N- neurofibromas (2 or more or one plexiform)
D- dysplasia of the sphenoid or cortical thinning of long bones with or without pseudoarthrosis
55
NF Type 1- what chromosome?
Autosomal dominant, chromosome 17 (same number of letters in neurofibromatosis)
100% penetrance by 5 yo
56
TS major criteria
```
At least 2 major or 1 major and 2 minor
A La Grass Hut
A- facial angiofibromas ≥ 3 or forehead plaque
L- lymphangiomyomatosis
A- Ash leaf spots (hypo pigmented macules ≥ 3, >5mm in diameter)
G- giant cell subependymal astrocytoma
R- rhabdomyoma in heart
A- angiomyolipoma of the kidney
S- shagreen patch
S- subependymal nodules
H- hemaratomas of the retina
U- ungal fibromas ≥ 2
T- tubers
```
periventricular calcification- can change into giant cell astrocytoma
57
What two things are required for SWS
capillary malformation in the face and the brain
1. port wine stain (capillary malformation)
2. leptomeningeal angioma (capillary venous malformation)
58
SWS involves which cranial nerve
angioma involving the leptomeninges and the skin of the face in the sensory territories V1 and V2 of trigeminal nerve (CN V)
59
What are the common symptoms of SWS (7)
```
S- seizures
S- stroke like episodes
H- hemiparesis
H- hemianopsia
H- headache
D- Developmental disabilities
G- Glaucoma
```
60
What is the most common cause of brachial plexus palsy
birth trauma
61
Term baby born via SVD, has brachial plexus palsy. Prognosis?
75% infants recover completely within first month of life
| 25% experience permanent impairment or disability
62
Name 2 treatment options for Bell's Palsy
1. oral steroids x 5 days (ideally within 72h)
2. eye drops and patching at night
3. treat the underlying cause (ex: OM or Lyme disease)
4. Consider acyclovir (within 72h) in patients with Ramsay hunt syndrome
63
Epidural hemorrhage occurs between..
bleeding between the dura and the skull
lucid interval
Biconvex on CT
64
Epidural hemorrhage is bleeding from which artery
middle meningeal artery
| ARTERIAL BLEED
65
Subdural hemorrhage occurs between...
Dura and arachnoid mater
| Concave on CT
66
Subdural hemorrhage is bleeding from what?
Bridging veins
| VENOUS BLEED
67
What type of bleed is seen with shaken baby syndrome
Subdural hemorrhage
68
What are the signs of basal skull fracture
Periorbital ecchymosis
Battle sign
Hemotympanum
CSF leaking from nose or ears
69
What is the criteria for a minor head injury
injury within the last 24 hours with LOC, persistent vomiting (>2 episodes 15 minutes apart), witnessed disorientation, amnesia, persistent irritability <2yo with GCS 13-15.
70
High risk CATCH (4)
GSC <15 2 hours after injury
Suspected open or depressed skull fracture
History of worsening HA
Persistent irritability
CT for any child with minor head injury plus 1 of the high risk or medium risk
71
Medium risk CATCH (3)
signs of basal skull fracture
large boggy hematoma of the scalp
Dangerous mechanism of injury ( fall >3 feet or 5 stairs, fall off bike with no helmet, MVA)
CT for any child with minor head injury plus 1 of the high risk or medium risk
72
What is the prognosis for Bell's palsy?
~70% complete recovery
Better prognosis if some signs of recovery of facial function within first 21 days of onset
Chance of recurrence 7-15%
73
How do you distinguish between positional and craniosynostosis
i. Presence of bony ridge
ii. Position of ear on affected (flat) side of head – anterior in posit. plag., unchanged or posterior in craniosyn.
iii. Bulging of ipsilateral forehead in posit. plag.
74
Name two treatment options for Guillain Barre
i. IVIG
ii. Plasmapheresis
iii. Supportive – esp. respiratory, assisted ventilation, paint management
75
List 2 investigations for Guillain Barre
i. LP – CSF protein is usually elevated to more than twice the upper limit of normal, the glucose level is normal, and there is no pleocytosis; there should be fewer than 10 white blood cells/mm3. Bacterial cultures are negative
ii. MRI head and spine – contrast enhancement of CN roots, spinal nerve roots, cauda equina
Nelsons MRI spine- thickening of the cauda equina and intrathecal nerve roots with gadolinium enhancement (extensive contrast enhancement of nerve roots)
iii. Nerve conduction studies – motor conduction block, slowing (or decreased amplitude) of conduction, temporal dispersion, prolonged latencies
EMG- acute denervation of muscle
Nelsons- Motor and sensory nerve conduction velocities are reduced to a variable extent, reflecting the patchy nature of nerve involvement in this disorder, which is also reflected in the presence of focal conduction block and dispersed responses. Electromyography may show acute denervation of muscle.
iv. Serum auto-Ab – anti-GQ1b
76
What gene mutation is associated with Rett syndrome
MeCP2
77
What is the hallmark of Rett syndrome
repetitive hand wringing movements
loss of purposeful and spontaneous use of the hands
development may proceed normally to 1 year of age then regression of language and motor milestones
78
A 3 y/o girl with regression of milestones is noted to have microcephaly. What is the most likely diagnosis:
Rett Syndrome
79
Classic triad of botulism
1. symmetric flaccid descending paralysis
2. No paresthesias
3. No fever
80
Name 5 contraindications to LP
1. skin infection near the site of the lumbar puncture
2. suspicion of increased ICP due to cerebral mass
3. unstable
4. uncorrected coagulopathy
5. acute spinal cord trauma
81
What is spastic diplegia and what causes it
spasticity in lower limbs bilaterally
caused by periventricular leukomalacia (damages fibres running closest to ventricles)
PVL occurs at 24-32 weeks
82
what is spastic hemiplegia and what is the most common cause
unilateral spasticity and motor deficit in upper and or lower limb
75% associated with PRENATAL insult (often in utero MCA stroke or IVH)
look for early hand preference**
83
Patients with TS are at risk of what brain tumor?
subependymal giant cell astrocytoma
| therefore brain MRI every 1-3 years for monitoring
84
where are shagreen patches typically located
lumbosacral region
| orange peel consistency
85
what is TAND and name 4 conditions
```
tuberous sclerosis–associated neuropsychiatric disorders
Intellectual disability
Autism
ADHD
anxiety
depression
```
86
Minor criteria for TS
Don't I regret coming NM
```
Dental enamel pits (>3)
Intraoral fibromas (≥2)
Retinal achromic patch
Confetti skin lesions
Nonrenal hamartomas
Multiple renal cysts
```
87
what tumors are characteristic of VHL
hemangioblastomas are characteristic of von Hippel-Lindau syndrome
- cerebellar Hemangioblastomas
- retinal capillary hemangioblastomas
88
what are common eye complications associated with SWS
buphthalmos
| glaucoma
89
what is the key feature of myotonic muscular dystrophy
myotonia- very slow relaxation of muscle after contraction
| most common form of muscular dystrophy that begins in adulthood
90
what is required for the diagnosis of guillian barre
progressive weakness in legs and arms
arreflexia (or decreased tendon reflexes) in weak limbs
ADDITIONAL SYMPTOMS
• Progressive phase lasts days to 4 wk (often 2 wk).
• Relative symmetry.
• Mild sensory symptoms or signs (not present in acute motor axonal neuropathy).
• Cranial nerve involvement, especially bilateral weakness of facial muscles.
• Autonomic dysfunction.
• Pain (common).
91
what are the most common causes of guillain-barre syndrome?
The onset of weakness usually follows a nonspecific gastrointestinal or respiratory infection by approximately 10 days.
GI- Campylobacter jejuni, but also Helicobacter pylori
Resp- mycoplasma pneumoniae
92
what is Guillain-barre
post infectious polyneuropathy
Guillain-Barré syndrome is an autoimmune disorder that is thought to be a "postinfectious polyneuropathy", involving mainly motor but also sensory and sometimes autonomic nerves.
93
Name 3 medications that can cause idiopathic intracranial hypertension
```
vitamin A
retinoic acid
minocycline
OCP
tetracyclines
sulphonamides
cytabarabine
```
94
what medications can cause acute dystonic reactions? what is the treatment?
```
haloperidol
risperidone
antiemetics (metoclopramide, prochlorperazine)
```
IV diphenhydramine (IV Benadryl)
95
what is gabapentin used for and some side effects
| what's an alternative?
neuropathic pain
somnolence, dizziness, and ataxia. Children occasionally demonstrate side effects not reported in adults, such as impulsive or oppositional behavior, agitation, and occasionally depression.
alternative: pregabalin
96
bilateral optic neuritis in children may be associated with what?
neuromyelitis optica (NMO or Devic disease)
97
optic neuritis is associated with what in the future?
MS (risk of MS is 19% within 20 yr)
98
what are some risk factors for deformational plagiocephaly? (10)
Male
First-born child
Prematurity
Limited passive neck rotation at birth (e.g., congenital torticollis)
Developmental delay
Sleep position is supine at birth and at 6 wk
Bottle feeding only
Tummy time < 3 times/day
Lower activity level, slower milestone achievement
Sleeping with head to same side, positional preference
99
what is the difference between central and peripheral facial paralysis
Peripheral facial paralysis, involves the entire side of the face, including the forehead. When the infant cries, movement occurs only on the nonparalyzed side of the face, and the mouth is drawn to that side. On the affected side the forehead is smooth, the eye cannot be closed, the nasolabial fold is absent, and the corner of the mouth droops.
Central facial paralysis spares the forehead. The infant with central facial paralysis usually has other manifestations of intracranial injury, most often 6th nerve palsy from the proximity of the 6th and 7th cranial nerve nuclei in the brainstem.
100
What is the prognosis for brachial plexus palsy? What are some risk factors?
75% of infants recover completely within first month of life
25% experience permanent impairment or disability
75% INFANTS RECOVER COMPLETELY WITHIN FIRST
risk factors: LGA, shoulder dystocia, maternal diabetes or obesity, forced delivery
101
CHILD PRESENTS WITH ATAXIA AND INABILITY TO SIT UP TWO WEEKS AFTER HAVING CHICKEN POX. WHAT IS THE DIAGNOSIS?
HOW DO YOU DIFFERENTIATE THIS FROM MENINGOENCEPHALITIS?
CHILD PRESENTS WITH ATAXIA AND INABILITY TO SIT UP TWO WEEKS AFTER HAVING CHICKEN POX. WHAT IS THE DIAGNOSIS?
POST INFECTIOUS ACUTE CEREBELLAR ATAXIA
HOW DO YOU DIFFERENTIATE THIS FROM MENINGOENCEPHALITIS?
- ABSENCE OF FEVER, NUCHAL RIGIDITY, ALTERED LOC AND SEIZURES.
102
what is the most common type of craniosynostosis
```
saggital craniosynostosis (most common type 80%)
more common in males
long and narrow skull
```
103
what would you see on exam for saggital craniosynostosis?
frontal bossing
prominent occiput
palpable keel ridge
decreased biparietal diameter
104
where do you see calcifications with CMV and toxoplasmosis?
CMV (periventricular calcifications)
| toxoplasmosis (intracranial calcifications)
105
what is the treatment for adrenoleukodystrophy?
bone marrow transplant
| ADHD= classic symptoms
106
what are 3 tests you would do to diagnose TS
MRI brain- cortical tubers
Genetic testing- TSC1 and 2
Woods lamp
107
Injury to what nerves cause Erbs Palsy? how do they hold their hand?
injury to C5, C6
waiters tip
shoulder internally rotated, arm adducted, elbow extended, hand pronated
108
Injury to what nerves cause Klumpke's palsy? how does their hand present?
Klumpke’s palsy = C8, T1 Deficits of small muscles of hand, presents as claw hand, usually permanent deficits.
109
Child with headache, list 5 features that you make you think this was a basilar migraine:
- Vertigo
- Tinnitus
- Diplopia
- Blurred Vision
- Scotoma (blind spot)
- Ataxia
- Occipital Headache
110
In a patient admitted with head injury what are 4 things that would cause increased risk of adverse outcomes?
Clinical severity at initial presentation (especially GCS <5)
· Raised ICP
· Presence and severity of injuries at other body site
· Pre-injury attention-deficit-hyperactivity disorder
· Socioeconomic status
111
what is the main side effect for vigabatrin
retinal toxicity
112
what is the main side effect for lamotrigine
rash
113
what drugs are associated with teratogenic effects? what should you start before pregnancy?
neural tube defects
start folate!!
VPA> phenytoin> topiramate>carbamazepine
114
Meds for primary generalized seizures
```
PRIMARY GENERALIZED:
• Ethosuxamide (only absence)
• Valproic acid (VPA)
• Lamotrigine (LTG)
• Levetiracetam (LEV)
• Topiramate (TPM)
```
115
which meds do you NOT use for primary generalized
carbamazepine (will worsen)
| phenytoin
116
Medications for SECONDARILY GENERALIZED
| (FOCAL-ONSET):
* First line:
* Levetiracetam (keppra)
* Carbamazepine / oxcarbamazpine
117
which meds do you not use for secondarily generalized
Ethosuxamide (ineffective)
118
Sudden (thunderclap) headache or “worst
headache of my life”
what is this a red flag for??
Aneurysmal sentinel bleed
119
Occipital location, red flag for what?
not seen with migraines (usually frontal or frontotemporal)
| Chiari 1 malformation
120
what is the genetic test for SMA
SMN 1 gene deletion testing (survival motor neuron 1)
| it is autosomal recessive
121
what is the most common cause of death in a child due to a genetic cause
Spinal muscular atrophy!!
| used to be CF
122
How is Duchenne muscular dystrophy passed on
x linked
| primarily only affects boys
123
what is a milder phenotype of Duchenne muscular dystrophy
Beckers muscular dystrophy
124
A 7 year old boy presents with increasing falls and
difficulty getting up from the floor. He has a mild
learning disability. He has proximal weakness and
prominent calf muscles. His serum CK is 20,000 U/L.
What is the most appropriate test:
DMD duplication deletion analysis
125
```
You see a newborn girl with
seizures & vesicular lesions in a
dermatomal distribution. HSV,
VZV testing are negative. What is
the most likely diagnosis?
```
Incontinenti pigmenti
126
What is the inheritance pattern for Sturge Webber
sporadic
127
```
Cerebral palsy is most likely to arise following a
hypotensive event at what time?
1. 5-8 weeks GA
2. 16-20 weeks GA
3. 26-30 weeks GA
4. 40-42 weeks GA
```
26-30 weeks
128
when does PVL occur
PVL only occurs between 24-32 weeks
causes spastic diplegia
due to watershed injury
