Pediatric Neurology Flashcards
Amblyopia
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
What 3 abnormal visual experiences results in unilateral amblyopia
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
Secondary results of amblyopia
Deviated eye
Unequal need for vision correction
A high refractive error in both eyes
Media opacity on the visual axis
Diagnosis of amblyopia
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
What abnormalities can a photo screener detect
Myopia
Hyperopia
Astigmatism
Anisometropia
Strabismus
Anisocoria
Amblyopia
Strabismus
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
Pseudostrabismus
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
Periorbital cellulites
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
Symptoms of periorbital cellulitis
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)
Etiologies of periorbital cellulitis
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
Diagnosis of periorbital cellulitis
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
Treatment for periorbital cellulitis
Broad spectrum empiric IV antimicrobial therapies
*must monitor for signs of complications
Orbital cellulitis
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
How does the clinical presentation of orbital cellulitis differ from periorbital cellulitis?
Orbital cellulitis patients feel ill and are febrile toxic apperance
Periorbital only shows a fever, but does look toxic
When should you worry about intracranial hematogenous spread of orbital sinusitis?
Patient has any of the following symptoms
- headache
- vomiting
- focal neurologic findings
Complications of orbital cellulitis
Visual loss Due to increase intraocular pressure
Cavernous sinus thrombosis
Meningitis
Epidural or Subdural empyema
Optic atrophy
Retinal or choroid always ischemia
Treatment of orbital cellulitis
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*
Potts puffy tumor
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
What are the 3 most common causes of headaches in children that are not febrile?
Migraines
Tension headaches
Cerebellar tumors
Migraines in children
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)
Red flags for migraines in kids
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
Specific red flag for brain tumor in children
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
Treatment of migraines
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
Brain tumors in pediatric patients
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%
What is most common site and types of brain tumors in children?
Site = infratentorially (50%)
- includes brainstem/cerebellum and 4th ventricle
Malignant = medulloblastoma
Benign = glioma
Clinical presentation for brain tumors
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)
Classic presenting symptoms for astrocytoma in the cerebellum in pediatric populations
Presents with:
- ataxia
- wide gait w/ falling tendencies
- headache and vomiting
Classic presenting symptoms for brain glioma in the brainstem in pediatric populations
Presents with
- 6th/7th nerve palsy
- contralateral limb weakness
- headache
- nausea/vomiting
\What is the classic triad of symptoms for all midline or infratentorial tumors in pediatric patients
Headache (morning always)
Nausea
Vomiting
papilledema is also very common but isn’t included technically
What classic symptoms are generally associated with infratentorial tumors
Blurred vision
Diplopia
Nystagmus
also torticollis (static head tilting) ONLY if cerebellar tonsil herniation occurs
Evaluation and management for brain tumors
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
Common pediatric tumors and their general survival rates
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%)
Do you use radiation in kids?
Usually no unless last resort
Causes increased toxicity and neuro cognitive defects as well as stunts growth
Supportive care in treatment for brain cancers
Hormonal replacement for neuroendocrine dysfunction
Antiepileptic drugs for seizure disorders
Physical/occupational/speech therapies
What is generally considered the most common type of seizures for kids?
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
Two types of febrile seizures
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
Common causes for febrile seizures
Exudative pharyngitis
Ottis media
Pneumonia
Rashes associated with viral syndromes
Meningitis
Do you do lumbar punctuates on kids for febrile seizures?
Usually not as long as they are immunized
- also aggressive use of acetaminophen and ibuprofen does not lower risk of febrile seizures
Botulism in children
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)
How to diagnosis infant botulism
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
Treatment of botulism in infants
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*
Shaken baby syndrome
Abusive head trauma
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%
What physical symptoms are certain evidence for child abuse
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%)
Tourette disorder diagnosis criteria
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
Chronic tic diagnostic criteria
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
Echopraxia
A type of complex motor tic where a child imitates someone else’s movements without wanting to
Copropraxia
A type of complex motor tic where a child makes a sexual or obscene gesture unwantingly
Difference between stereotypies and tics
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
Pathophysiology of tics
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
Treatment for Tourette’s syndrome and tics
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)
Partial arousal parasominas (sleepwalking, night terrors and confusional arousals)
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
Chorea
Sydenham chorea
“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
Treatment of Sydenham chorea
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
Guillain-Barré syndrome in pediatrics (GBS)
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
What vaccines can cause GBS?
Rabies
Influenza
Meningococcal (especially serogroup C)
*note these are ultra rare side effects as long as the patient has a normal immune system
What virus can mimic GBS?
West Nile virus
Sturge-Weber syndrome (SWS)
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
Diagnosis for SWS
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
Cerebral palsy (CP)
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
When should genetic and metabolic testing be conducted for cerebral palsy children?
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)
What is the most common type of CP?
Spastic cerebral palsy
Shows mild-severe spastic movement symptoms
- can affect legs/arms or the whole body
- symptoms do not get worse
Diskinetic CP
A type of CP which causes uncontrolled movements most commonly in the entire body (especially tongue and face)
Ataxic CP
Affects balance and coordination of movements (usually in the arm and hand)
Common causes of CP
- 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
Risk factors for CP
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
