L7: Pediatric neurology Flashcards
Cerebral palsy subtypes
Spastic (most common) → stiff and tight
Ataxic→ shaky, affects balance and sense of positioning
Dyskinetic→ involuntary movements
Mixed
Cerebral palsy is caused by
Hypoxia Trauma Premature birth Infection Toxins Structural abnormalities
Most kids sit by ____ and walk by ____
8 months
18 months
Cerebral palsy presentation
Motor impairment Not reaching milestones Excessive irritability Poor feeding, drooling Poor visual attention Difficult to hold, cuddle Retained primitive reflexes
Asymmetrical tonic neck reflex (ATNR)
Primitive reflex retained in cerebral palsy
Moro reflex
Primitive reflex retained in cerebral palsy
startle on suddenly being released (normally gone at 4-6 mos)
Meds for cerebral palsy
anti-spasmodics
botulism toxin
In infants who die less than 1 year old
40% have congenital malformations of the CNS
Chiari Type I
Cerebellar tonsils displaced caudally below the foramen magnum +/- syringomyelia → fluid filled cyst within spinal cord
presents as a teen or adult
Chiari Type II
Cerebellar tonsils displaced caudally below the foramen magnum + myelomeningocele
Loss of abdominal reflex
Chiari type I
What causes neurological symptoms in chiari type I
presence of a syringomeylia
Chiari type II signs/symptoms
Hydrocephalus
Dysphagia
Upper extremity weakness
Apneic spells and aspiration
Spina bifida oculta
incomplete closure of the spinal canal, lower back most common
Spina bifida: meningocele
outpouching of the spinal fluid + meninges through vertebral cleft → mild problems with sac protrusion
Spina bifida: myelomeningocele
most severe. Spinal cord and/or nerve protrude from vertebral cleft.
Spina bifida oculta signs/symptoms
no/mild signs: hairy patch, dimple, dark spot, swelling at site of gap
Spina bifida: meningocele signs/symptoms
mild problems with sac protrusion
Spina bifida: myelomeningocele signs/symptoms
most severe→ weakness, loss of bladder/bowel control, hydrocephalus, inability to walk, learning problems
causes of spina bifida
Genetic
Medications during pregnancy
Low folate
Poorly managed DM
What can be done for spina bifida?
shunt, surgery
Hydrocephalus
Increased volume of CSF → ventricular dilation and increased intracranial pressure
Obstructive: blockage
Non-obstructive: impaired absorption, rarely overproduction
Hydrocephalus is caused by
CNS malformations Infection Intraventricular hemorrhage Genetic defects Trauma CNS tumors
Hydrocephalus symptoms
Asymptomatic Bradycardia, hypertension Altered respiratory rate HA, N/V Behavioral changes Papilledema, diplopia Macrocephaly Spasticity Spinal abnormalities
How do you diagnose hydrocephalus in infants/newborns?
Ultrasound
How do you diagnose hydrocephalus in infants/older children?
MRI or CT
Hydrocephalus management
refer to a neurosurgeon +/- shunt
Microcephaly and macrocephaly are treated by
Neurology referral
→ labs, imaging
Treat underlying cause
Microcephaly definition
Head circumference >2 standard deviations below mean, or <5th percentile
Primary (congenital)
→ lack of brain development
→ abnormal development due to timing of insult
Secondary (postnatal)
→ injury/insult to previously normal brain
Macrocephaly definition
Head circumference >2 standard deviations above mean, or >95th percentile
→ due to increase in size of any components of cranium: brain, CSF, blood, bone
Microcephaly is caused by
Genetic Pre/peri/post-natal injury Craniosynostosis Metabolic Toxin exposure
Microcephaly presentation
Delayed milestones Seizures Spasticity \+/- early fontanelle closure \+/- prominent sutures
Rapid growth in macrocephaly suggests
increased intracranial pressure
Catch-up growth macrocephaly occurs in
premature infants who are neurologically intact
Normal growth rate in macrocephaly suggests
familial macrocephaly or megalencephaly
Neurofibromatosis genetics
Autosomal dominant
NF1 most common form
Neurofibromatosis neurological abnormalities
macrocephaly
seizures
cognitive deficits
Neurofibromatosis signs
Cafe-au-lait macules by 1st year Axillary/inguinal freckling by 3-5 years Lisch nodules Optic glioma Neurofibromas
Primary headaches
migraine, tension
Secondary headaches
due to acute febrile illness
Migraine vs tension headache: location
Migraine: focal, unilateral or bilateral
Tension: diffuse, frontal or temporal
Migraine vs tension headache: duration
Migraine: 2-72 hours
Tension: 30 mins-1 week
Migraine vs tension headache: intensity
Migraine: moderate/severe
Tension: mild/moderate
Migraine vs tension headache: quality
Migraine: pulsatile/throbbing
Tension: pressure/non-throbbing
Migraine vs tension headache: activity
Migraine: aggravated with activity or reduced activity
Tension: not aggravated with activity
Concerning headaches signs/symptoms
Abnormal neurologic/visual exam
Vomiting
Daily symptoms with progressive worsening
Increased with coughing or bending
Acute onset without previous history
Severe upon awakening/awaken in middle of night
Pseudotumor cerebri
idiopathic intracranial hypertension without mass or hydrocephalus
Who gets Pseudotumor cerebri
obese teenage girls
Classic signs of Pseudotumor cerebri
HA + papilledema= hallmark
Visual field loss, acuity loss
Pulsatile tinnitus
Acetazolamide
Treatment for Pseudotumor cerebri
reduce rate of CSF production
Topiramate
Treatment for Pseudotumor cerebri
Control HA, adjust weight
Furosemide
Treatment for Pseudotumor cerebri to reduce fluid and pressure
Other options for treatment of Pseudotumor cerebri
Shunting
Optic nerve fenestrations
Weight loss + low sodium
Lumbar puncture of Pseudotumor cerebri has
elevated opening pressure
Seizure
sudden, transient disturbance of brain function→ involuntary sensory, motor, autonomic symptoms +/- LOC
Epilepsy
> 2 seizures more than 24 hours apart
Absence seizure
Sudden impairment in consciousness without loss of tone. Genetic
Febrile seizure
Convulsion with temp > 38 C (100.4 F)
6 months- 5 years
Virus
2 types of febrile seizures
Simple: most common, lasts <15 minutes
Complex: focal, last >15 minutes or occurs 2+ times in 24 hours
Absence seizures presentation/history
Provoked by hyperventilation
Ages 4-10 years old
Arrest in activity lasts 9-10 seconds, may occur 10x/day
only do an EEG for febrile seizures if
there is a risk of developing epislepsy
Ethosuximide
first line treatment for absence seizures
If a febrile seizure lasts longer than 5 minutes:
IV benzodiazepines
What’s the long term prognosis for absence seizures?
spontaneously remit by puberty
Guillain-Barre syndrome
Illness (campylobacter) → acute immune mediated polyneuropathy
Botulism
Honey, home canning → ingestion of clostridium botulinum spores→ neurotoxin binds ACh receptors
Guillain-Barre syndrome vs botulism: weakness
Guillain-Barre syndrome: ascending
Botulism: descending
Who gets botulism
kids <6 months
Most common cause of acute flaccid paralysis in healthy kids
Guillain-Barre syndrome
Guillain-Barre syndrome treatment
Hospitalization
IVIG or plasma exchange
Botulism treatment
Hospitalization
Monitoring
Botulism immune globulin (BIG-IV or BabyBIG)
Guillain-Barre syndrome diagnostics
EMG
CSF analysis: increased protein with normal WBC
Spinal MRI w/ and w/o contrast:
→ enhancement of spinal nerve roots and cauda equina
Botulism diagnostics
Spores in stool sample
EMG
Duchenne muscular dystrophy genetics
X-linked recessive defect in genes responsible for muscle function
Gower’s sign
Duchenne muscular dystrophy
use of hands to push up from floor
Duchenne muscular dystrophy vs Becker muscular dystrophy: onset
Duchenne muscular dystrophy: 2-3 years
Becker muscular dystrophy: later
Duchenne muscular dystrophy vs Becker muscular dystrophy: creatinine kinase levels
Duchenne muscular dystrophy: 10-20X
Becker muscular dystrophy: >5X
Duchenne muscular dystrophy vs Becker muscular dystrophy: cardiomyopathy
Duchenne muscular dystrophy: +/-
Becker muscular dystrophy: more predominant
Duchenne muscular dystrophy treatment
glucocorticoids
Duchenne muscular dystrophy progression
Onset ages 2-3
Wheelchair bound by 13
Mortality 18-20 years
Progressive weakness: proximal→ distal, lower extremities→ upper extremities
Duchenne muscular dystrophy: other signs/symptoms
Pesudohypertrophy of calves
growth delay
+/- orthopedic complications
+/-cognitive impairment