Pediatric Neurology Flashcards

1
Q

Amblyopia

A

Decrease in visual acuity that occurs as a result of a lack of clear image on the retina
- can be unilateral (more common) or bilateral (less common)

Can’t be immediately corrected with glasses of surgery

  • instead they wear a patch over their good eye to force the bad eye to work and get better
  • can also where lens that blur that good eye

Age 6 years -8yrs is the highest likelihood
- 2-4% of North American population

Caused by anything that interferes with formation of a clear retinal image usually during the critical period of development before the cortex has become visually mature

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2
Q

What 3 abnormal visual experiences results in unilateral amblyopia

A

Strabismus
- eyes dont look in the same direction at the same time (crossed eyes)

Anisometropia
- unequal refractive errors

Monocular visual deprivation
- cataracts, corneal opacity, hemangioma, severe ptosis

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3
Q

Secondary results of amblyopia

A

Deviated eye

Unequal need for vision correction

A high refractive error in both eyes

Media opacity on the visual axis

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4
Q

Diagnosis of amblyopia

A

Kinda challenging

For preverbal children
- differences between eyes in fixation and following/preference can be diagnostic (but not 100%)

Automated photo-screeners are now gold standard

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5
Q

What abnormalities can a photo screener detect

A

Myopia

Hyperopia

Astigmatism

Anisometropia

Strabismus

Anisocoria

Amblyopia

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6
Q

Strabismus

A

Eyes are not aligned properly

  • bad eye can look inward/outward/upward/downward
  • eye that is misaligned can change or stay the same.

Leading cause of amblyopia

Can be convergent (esotropia) or divergent (exotropia) if horizontal

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7
Q

Pseudostrabismus

A

At a young age epicanthic folds in the inner eyelids of children can make it appear a child is cross eyed.

  • due to having a wide flat nose
  • pupil light reflex will be normal
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8
Q

Periorbital cellulites

A

Common in patients younger than 5 and have a decent history of upper respiratory tract infections

Possible Complication of sinusitis
- if this is the case, ethmoid and/or maxillary sinuses are affected

Must make sure the cellulitis is confined to the tissues outside the orbit only. (If inside orbit is worse orbital cellulitis)

  • ask if moving the eye under the swollen eyelid is possible and does it hurt if it can be moved
  • no movement or painful movement = orbital cellulitis
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9
Q

Symptoms of periorbital cellulitis

A

Sudden appearance of lid and periorbital swelling
- usually unilateral, indurated and tender to touch

Can show conjunctival injection and discharge

Often shows fever and uncomfortable patient (dont apper toxic or sick though)

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10
Q

Etiologies of periorbital cellulitis

A

50% idiopathic, 50% sinutis or bacteremia predisposed

Can also be caused by trauma to the orbit or via the following infections

  • impetigo
  • pustules
  • chalazions
  • infected dermatitis
  • specific insect bites

Generally in patients older than 5 yrs

  • the two most predominant pathogens associated are S. Aureus and GAS
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11
Q

Diagnosis of periorbital cellulitis

A

Requires a lot of cultures
- can be done via careful needle aspiration but is challenging

Nasopharyngeal and conjunctival drainage can have the affecting organism in 1/2-2/3 of cases

Blood cultures are positive in 1/3 or cases

CT scan is required if you cant tell if it’s periorbital or orbital As well as to show if any sinuses are compromised

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12
Q

Treatment for periorbital cellulitis

A

Broad spectrum empiric IV antimicrobial therapies

*must monitor for signs of complications

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13
Q

Orbital cellulitis

A

Worsened periorbital cellulitis (or develops on its own)
- often presents with subperiosteal abscesses which limits upward eye movement (or painful movement)

Possesses the following triad of symptoms:

  • proptosis (bulging of eyes
  • painful limitation of eye movement
  • decreased visual acuity

Increased chance of occurring in the winter as complicated sinusitis and also often follows respiratory viral infections
- being male also increases risk and mean age is 7yrs old

Requires CT to determine if any sinusitis is present
- most commonly compromised is the paranasla (ethmoid) sinusitis

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14
Q

How does the clinical presentation of orbital cellulitis differ from periorbital cellulitis?

A

Orbital cellulitis patients feel ill and are febrile toxic apperance

Periorbital only shows a fever, but does look toxic

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15
Q

When should you worry about intracranial hematogenous spread of orbital sinusitis?

A

Patient has any of the following symptoms

  • headache
  • vomiting
  • focal neurologic findings
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16
Q

Complications of orbital cellulitis

A

Visual loss Due to increase intraocular pressure

Cavernous sinus thrombosis

Meningitis

Epidural or Subdural empyema

Optic atrophy

Retinal or choroid always ischemia

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17
Q

Treatment of orbital cellulitis

A

CT image is required

Lumbar puncture only if suspect meningitis as secondary complication

Systemic antibiotic therapy with broad spectrum is needed

  • ampicillin/sulbactam
  • IV clindamycin/ceftriaxone
  • cefepime

Give the vancomycin/cefotaxime/metronidazole combo therapy only if you suspect intracranial extension

  • use sinus drainage directly if antibiotics dont work*
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18
Q

Potts puffy tumor

A

Erosion of the frontal bone causes subperiosteal abscesses

Caused usually by untreated frontal sinusitis

Is red, swelling and dough consistency that has serious tenderness

Patients look toxic, febrile and uncomfortable

Must get a CT scan to ensure it has not leaked into the brain and other areas.

Once confirmed by CT, start surgical drainage and IV antimicrobial long-term therapy for the preceding osteomyelitis

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19
Q

What are the 3 most common causes of headaches in children that are not febrile?

A

Migraines

Tension headaches

Cerebellar tumors

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20
Q

Migraines in children

A

Most common cause of acute and recurrent headaches

Multiple triggers can cause it

Usually thought to be a cause of primary neuronal dysfunction (still idiopathic etiology for now)

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21
Q

Red flags for migraines in kids

A

Vomiting
- check for increased intracranial pressure (especially if the vomiting is daily and in the early morning)

Headaches that wake up the child from sleep
- pituitary tumor with increased intracranial pressure needs to be ruled out

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22
Q

Specific red flag for brain tumor in children

A

Vomiting and headache that is frequently present once waking up and goes away with maintenance of upright posture
- increased intracranial pressure is present and almost always caused by brain tumor in this case

  • if the child stands up once awakening and the headache/vomiting gets worse, this is a migraine instead of a tumor
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23
Q

Treatment of migraines

A

NSAIDS (not aspirin) and lifestyle is the gold standard of therapy
- almost always ibuprofen

can’t use triptans/ergots in kids (only in people 13 and above)

  • can use BBs if needed
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24
Q

Brain tumors in pediatric patients

A

Primary CNS tumors are most common type of brain tumor
- 20% of all childhood cancer

2nd most common cancer type in children

  • male and <20 yrs old
  • infratentorial location is most common in children 1-15 yrs

Mortality = 30%

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25
Q

What is most common site and types of brain tumors in children?

A

Site = infratentorially (50%)
- includes brainstem/cerebellum and 4th ventricle

Malignant = medulloblastoma

Benign = glioma

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26
Q

Clinical presentation for brain tumors

A

Usually doesnt start until Increased intracranial pressure is noted (usually caused by obstruction fo CSF)
- most common locations are 4th ventricle/posterior fossa/pineal gland.

Symptoms

  • headaches in the morning (get better once you start moving)
  • nausea/vomiting/fatigue
  • 6th cranial nerve palsy
  • papilledema
  • tense fontanelle/failure to thrive
  • developmental delay
  • paresis of upwards gaze (“sun setting” gaze is this with downward eye deviation)
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27
Q

Classic presenting symptoms for astrocytoma in the cerebellum in pediatric populations

A

Presents with:

  • ataxia
  • wide gait w/ falling tendencies
  • headache and vomiting
28
Q

Classic presenting symptoms for brain glioma in the brainstem in pediatric populations

A

Presents with

  • 6th/7th nerve palsy
  • contralateral limb weakness
  • headache
  • nausea/vomiting
29
Q

\What is the classic triad of symptoms for all midline or infratentorial tumors in pediatric patients

A

Headache (morning always)

Nausea

Vomiting

papilledema is also very common but isn’t included technically

30
Q

What classic symptoms are generally associated with infratentorial tumors

A

Blurred vision

Diplopia

Nystagmus

also torticollis (static head tilting) ONLY if cerebellar tonsil herniation occurs

31
Q

Evaluation and management for brain tumors

A

CT is almost always 1st (95% sensitive)
- looks for calcifications in tumors, hydrocephalus and hemorrhages

MRI is then next to confirm tumor (100% sensitive)

Then refer to a tertiary hospital for neurosurgery and oncology

32
Q

Common pediatric tumors and their general survival rates

A

Cerebrum Glioma
- best rates of survival (60-100%)

Medulloblastoma (PNET)
- 2nd best rates of survival (33-85%)

Ependyomas
- 3rd best rates of survival (22-80%)

Brain steam glioma

  • wide range of outcomes
  • pontine tumors = 9-13 months to live (0% to live)
  • localized = 80-90% chance of survival

Malignant glioma
- low survival rates (25%)

33
Q

Do you use radiation in kids?

A

Usually no unless last resort

Causes increased toxicity and neuro cognitive defects as well as stunts growth

34
Q

Supportive care in treatment for brain cancers

A

Hormonal replacement for neuroendocrine dysfunction

Antiepileptic drugs for seizure disorders

Physical/occupational/speech therapies

35
Q

What is generally considered the most common type of seizures for kids?

A

Febrile seizures

  • occurs 2-5% of kids
  • is classified only if no intracranial infection/metabolic disturbance/traumatic cause or history of afebrile seizures is present.

Risk factors:

  • high fever >104F (40C)
  • developmental delay
  • family history of febrile seizures

Treatment:

  • prognosis is excellent w/ no cognitive impairment
  • doesnt need work up as long as you can diagnosis a febrile illness as the cause
36
Q

Two types of febrile seizures

A

Simple

  • short seizure (<15 min)
  • returns to baseline mental status within 1 hour
  • less dangerous

Complex

  • prolonged seizure (>15 min)
  • either doesn’t return to baseline within 1 hr or has recurrent seizures within 24 hrs of 1st seizure
  • dangerous
37
Q

Common causes for febrile seizures

A

Exudative pharyngitis

Ottis media

Pneumonia

Rashes associated with viral syndromes

Meningitis

38
Q

Do you do lumbar punctuates on kids for febrile seizures?

A

Usually not as long as they are immunized

  • also aggressive use of acetaminophen and ibuprofen does not lower risk of febrile seizures
39
Q

Botulism in children

A

Common in breast fed infants who have constipation
- also most common in children 3weeks- 6 months of age

Common sources are:

  • honey
  • soil
  • dust
  • corn syrup

Common states for botulism in children

  • Hawaii
  • Utah
  • California
  • Pennsylvania

Symptoms

  • NO FEVER
  • consitpation
  • poor feeding
  • weak cry
  • autonomic dysfunction s
  • *respiratory arrest is possible (so dangerous)
40
Q

How to diagnosis infant botulism

A

Isolation of C. Botulinum in stool
- gold standard but takes forever

Clincial history and physical tests can be enough as well because of the length to confirm for botulism
- “rag doll”/“floppy baby” infants is a gold standard sign

41
Q

Treatment of botulism in infants

A

Call infant botulism treatment program

Give baby IV botulism immune globulin (babyBIG) immediately
- save money and time in the hospital

  • dont give Aminoglycosides antibiotics (makes is worse*
42
Q

Shaken baby syndrome

Abusive head trauma

A

Combination of brain injury and hemorrhages as a result of shaking an infant severely

10% of all cases of child abuse

Most common finding is retinal hemorrhages

Mortality rate = 25%

43
Q

What physical symptoms are certain evidence for child abuse

A

Avulsion of the optic nerve or vitreous base

Retinal brushes

Orbital bone fractures

Corneal or sclera lacerations

Sub conjunctival hemorrhage (technically not always but like 90%)

44
Q

Tourette disorder diagnosis criteria

A

1) both multiple moron and one or more multiple vocal tics are present at some time (doesnt have to be concurrent)
2) must last for more than 1 yr from onset
3) onset must be before age 18yr
4) disturbance is not attributable to physiologic effects of a substance or other medical condition

45
Q

Chronic tic diagnostic criteria

A

1) single or multiple motor OR vocal tics must be present (NOT BOTH)

2) must have persisted > 1 yr from onset
- < 1 yr is a provisional tic

3) onset must be before 18 yrs
4) disturbance is not attributable to substances or physiologic effects/medical conditions
5) criteria for Tourette’s has never been met

46
Q

Echopraxia

A

A type of complex motor tic where a child imitates someone else’s movements without wanting to

47
Q

Copropraxia

A

A type of complex motor tic where a child makes a sexual or obscene gesture unwantingly

48
Q

Difference between stereotypies and tics

A

Stereotypies resemble tics but are rhythmic movements and do not demonstrate change in body locations or movement type over time
- tics usually changes types over time

49
Q

Pathophysiology of tics

A

Dysfunction in the corticostriatal-thalamocortical motor pathways

Also associated with dopamine, serotonin an NE neurotransmitter systems

Is male predominant

Onset is 4-6 yrs
Peak severity is 10-12 yrs
Marked severity in 18-20 yrs
Almost never worsens into adulthood

50
Q

Treatment for Tourette’s syndrome and tics

A

Based on severity
- mild tics are not treated usually

Need HRT therapy if treating

Medications:

  • haloperidol and primozide
  • aripiprazole
  • Clonidine and guanfacine (1st line agents)
51
Q

Partial arousal parasominas (sleepwalking, night terrors and confusional arousals)

A

More common in school age children
- occur during the 1st 3rd of the night (nightmares in the last 3rd)

Appears to be a genetic component for sleepwalking and night terrors

Scheduled awakenings are the most helpful intervention (wake the child up 15-30 minutes before the normal scheduled event)

if using pharmacotherapy (which is almost never done) use benzos or tricyclic antidepressants

52
Q

Chorea

Sydenham chorea

A

“Dance-like” chaotic movements that seem to flow from 1 body part to another
- increases exponentially with stress and disappears in sleep

Caused by a recent GAS infection (usually acute rheumatic fever)
- the cross-reactive antibodies affect cerebellar and basal ganglia functions

Risk factors:

  • family history for chorea or rheumatic fever
  • being a girl
  • ages 5-15
  • gets GAS/rheumatic fever

*clinical hallmarks are chorea with hypotonia and emotional lability

53
Q

Treatment of Sydenham chorea

A

Diagnosis requires antistreptolysin titers and evaluation for carditis (negative results for both does NOT exclude diagnosis, but can confirm)

Treatment = long term antibiotic prophylaxis (penicillin G IM admission every 4 weeks or penicillin V PO admission 2x daily) until the chorea dissolves on its own

54
Q

Guillain-Barré syndrome in pediatrics (GBS)

A

Post-infections polyneuropathy

Usually only affects motor tracts (but can do autonomic nerves also

Causes: (usually GI and respiratory infections)

  • campylobacter jejuni
  • helicobacter pylori
  • mycoplasma pneumonia
  • Zika and haemophilus influenza
  • CMV
  • mononucleosis
  • Lyme disease
  • listeria

Common children specific clincial symptoms:

  • very irritable
  • refuses to walk
  • flaccid tetrapolegia (late stage only)
  • can affect bulbar muscles which initiates respiratory failure if not treated
55
Q

What vaccines can cause GBS?

A

Rabies

Influenza

Meningococcal (especially serogroup C)

*note these are ultra rare side effects as long as the patient has a normal immune system

56
Q

What virus can mimic GBS?

A

West Nile virus

57
Q

Sturge-Weber syndrome (SWS)

A

Patients that have a facial capillary malformation with buphthalmos (port wine stain) and appear to have epilepsy
- generates excessive blood vessel growth

Caused by a GNAQ gene mutation

Other symptoms:

  • seizures (opposite side of port wine stain)
  • TIA-like symptoms
  • headaches
  • intellectual disabilities
  • hemiparesis (opposite side of port wine stain)
  • glaucoma
58
Q

Diagnosis for SWS

A

MRI w/ contrast of brain

  • shows extension of leptomeningeal capillary malformations
  • shows white matter abnormalities
  • shows cerebrum atrophy ipsilateral to the port wine stain
  • choroid plexus is usually enlarged
59
Q

Cerebral palsy (CP)

A

Group of nervous system disorders that cause abnormal movements, body positions and poor balance

Symptoms are often present at both and are born with the condition (not inherited throughout life)

Motor defect is ALWAYS present
- no loss of function, just doesnt develop

Treatment is the symptoms that develop, there is no cure

60
Q

When should genetic and metabolic testing be conducted for cerebral palsy children?

A

1) evidence of determination or episodes of metabolic decompensation
2) neuro imaging results in no etiology
3) family history of a childhood disorder associated with CP
4) a developmental malformation on neuroimaging is noted (especially if a stroke looks prominent)

61
Q

What is the most common type of CP?

A

Spastic cerebral palsy

Shows mild-severe spastic movement symptoms

  • can affect legs/arms or the whole body
    • symptoms do not get worse
62
Q

Diskinetic CP

A

A type of CP which causes uncontrolled movements most commonly in the entire body (especially tongue and face)

63
Q

Ataxic CP

A

Affects balance and coordination of movements (usually in the arm and hand)

64
Q

Common causes of CP

A
  • note this doesn’t include all causes*

Reduced blood/oxygen supply at birth or in the womb

A brain infection at birth

Brain trauma

High levels of bilirubin at birth or before 5 yrs

Abnormal development of the brain

65
Q

Risk factors for CP

A

Premature births/ low birth weight

Mother has had multiple births

Conceived through infertility treatments

Mother has a viral or bacterial infection during pregnancy

Severe jaundice

Complicated births

Did not get routine vaccinations