Pediatric Neurology Flashcards
What condition involves a non-progressive motor impairment due to fetal/infantile brain injury?
Cerebral Palsy
What is the most common type of Cerebral Palsy? How does this present?
Spastic
- Stiff/tight muscles
What condition involves abnormal tone/posture, retained primitive reflexes?
Cerebral Palsy
What condition involves increased CSF volume → ventricular dilation, increased ICP?
Hydrocephalus
What are the two subtypes of Hydrocephalus?
- Obstructive (blockage)
- Non-obstructive (impaired absorption)
What is the preferred test for a newborn/infant with Hydrocephalus? What about a older infant/child?
- Newborn/infant = US
- Older infant/child: MRI or CT
What sign/symptom is evident with Hydrocephalus?
Macrocephaly
What is Occipitofrontal Circumference (OFC) reflective of? How often should it be measured?
OFC reflects brain growth
- Measure EVERY well visit from birth to 3 years
What two conditions are considered Microcephaly?
Head circumference
- 2+ standard deviations BELOW mean
OR
- <5th percentile
What two conditions are considered Macrocephaly?
Head circumference
- 2+ standard deviations ABOVE mean
OR
- 97th+ percentile
What is the etiology for Microcephaly? What are the two subtypes?
BRAIN
- Primary (congenital, lack brain development)
- Secondary (brain injury post-natal)
What is the etiology for Macrocephaly?
ANY component of cranium (brain, CSF, blood, bone)
How do the etiology subtypes of Macrocephaly present (rapid growth, catch-up growth, normal growth rate)?
- Rapid growth: high ICP
- Catch-up growth: premature but neuro intact
- Normal growth rate: family history of large heads
If there is a concern regarding OFC, what should be your next step in evaluation?
REMEASURE
What are the two Congenital Malformation of CNS? How are each diagnosed?
- Chiari Malformations
- Spinal Dysraphisms (Spina Bifida)
BOTH diagnosed with MRI
What are the two types of Chiari Malformation?
- Type I: displaced caudally below foramen magnum
- Type II: TYPE I + myelomeningocele +/- hydrocephalus
What age group does each type of Chiari Malformation typically present?
- Type I: teen, adult
- Type II: prenatally, at birth
What condition involves neck pain, HA, CN abnormalities, high ICP worse with cough, sneeze, Valsalva?
Type I Chiari Malformation
What condition involves dysphagia, apneic spells, and often requires surgery as treatment?
Type II Chiari Malformation
What condition can be caused by low folate, genetics, fever, uncontrolled DM, obesity?
Spina Bifida
What are the two subtypes of Spina Bifida?
- Closed (occulta)
- Open (meningocele, myelomeningocele)
What condition involves incomplete closure of spinal cord? In what three ways might this present?
Spina Bifida Occulta (closed)
- Hairy patch
- Dark spot
- Back swelling
How does Meningocele differ from Myelomeningocele? Which is more severe?
- Meningocele: outpouching of spinal fluid and meninges from vertebral cleft
- Myelomeningocele: meninges AND spinal cord protrude from vertebral cleft; MORE SEVERE
What condition involves outpouching of spinal fluid and meninges from vertebral cleft?
Meningocele (open Spina Bifida)
What condition involves meninges AND spinal cord protrude from vertebral cleft
Myelomeningocele (open Spina Bifida)
Which condition is often associated with Type II Chiari Malformation and Hydrocephalus?
Myelomeningocele (open Spina Bifida)
What two conditions are often associated with Myelomeningocele (open Spina Bifida)?
- Type II Chiari Malformation
- Hydrocephalus
What type of genetic disease is Neurofibromatosis (NF1)?
Autosomal dominant
Which condition involves café-au-lait macules, neurofibromas, axillary/inguinal freckling?
Neurofibromatosis (NF1)
Which type of HA involves focal; 2-72 hours?
Migraine HA
Which type of HA involves diffuse; 30 minutes to 7 days?
Tension HA
What condition involves moderate/severe pulsatile and throbbing HA?
Migraine HA
Which type of HA is worse with activity?
Migraine HA
What condition involves N/V, photophobia and phonophobia +/- aura?
Migraine HA
What condition involves mild/moderate pressure and NON-throbbing?
Tension HA
What condition involves photophobia or phonophobia (not both)?
Tension HA
What condition involves idiopathic intracranial HTN → high ICP?
Pseudotumor Cerebri
What population is Pseudotumor Cerebri most common?
Obese teen girl
What condition presents with papilledema, also HA and vision issues?
Pseudotumor Cerebri
Which peds neuro condition is a diagnosis of exclusion?
Pseudotumor Cerebri
What is the treatment for Pseudotumor Cerebri?
Acetazolamide
What condition involves sudden, transient brain function disturbance?
Seizure
How can you differentiate seizure from epilepsy?
Epilepsy: 2+ unprovoked seizures more than 24 hours apart
What are the three phases of a seizure?
- Aura (warning signs/pre)
- Ictal (active/during)
- Post-ictal (recovery/after)
Which seizure type involves localized region of brain; often brief?
Focal Seizure
Which type of seizure involves unilateral sxs or inappropriate emotions/behaviors?
Focal Seizure
How can you differentiate simple from complex Focal Seizure?
- Simple = conscious; seconds to minutes
- Complex = impaired awareness; 3+ minutes
Which seizure type involves both hemispheres simultaneously?
Generalized Seizure
Which type of seizure involves LOC/impaired awareness; varying post-ictal state?
Generalized Seizure
What is the most common type of Generalized Seizure?
Clonic/tonic
What type of seizure involves rhythmic jerking?
Clonic/tonic (Generalized Seizure)
What type of seizure involves arrest in activity lasting ~10 seconds, can occur 10x/day?
Absence (Generalized Seizure)
How do you diagnose an Absence (Generalized Seizure)?
EEG
What is the first line treatment for Absence (Generalized Seizure)?
Ethosuximide
What provokes an Absence (Generalized Seizure)?
Hyperventilation
What type of seizure is associated with genetic predisposition?
Febrile Seizures
Which type of Febrile Seizure is more common? What is the other kind?
Simple = MORE common
- Also Complex
How can you differentiate simple from complex Febrile Seizure?
- Simple: lasts less than 15 minutes
- Complex: focal, lasts 15+ minutes OR more than once per day
How do you treat a Febrile Seizure lasting longer than 5 minutes?
IV benzodiazepines
What often precedes Guillain-Barre Syndrome, and what is an example?
ILLNESS
- Campylobacter jejuni
What condition involves ascending symmetric weakness; neuropathic pain?
Guillain-Barre Syndrome
What is the recommended diagnostic test for Guillain-Barre Syndrome? What else can be used?
EMG/NCS
- Can also do CSF analysis
What is the recommended treatment for Guillain-Barre Syndrome (2)?
Hospitalization!
- IVIG or plasma exchange
What condition involves descending weakness; constipation?
Botulism
What is the recommended diagnostic test for Botulism?
Stool sample
What is the recommended treatment for Botulism (2)?
Hospitalization!
- BIG-IV or BabyBIG
What type of genetic disease is Duchenne Muscular Dystrophy (DMD)?
X-linked recessive
What condition involves onset at 2-3 years and progressive (death: 20 years)?
Duchenne Muscular Dystrophy (DMD)
What condition involves Gower’s sign?
Duchenne Muscular Dystrophy (DMD)
What condition involves progressive weakness, calves pseudohypertrophy?
Duchenne Muscular Dystrophy (DMD)
What condition involves profoundly elevated CK (10-20 times normal)?
Duchenne Muscular Dystrophy (DMD)
What is the recommended treatment for Duchenne Muscular Dystrophy (DMD)?
Steroids
What condition is associated with Becker Muscular Dystrophy (BMD)?
Cardiomyopathy
What condition involves CK less elevated (only 5x)?
Becker Muscular Dystrophy (BMD)
How can you differentiate Duchenne Muscular Dystrophy (DMD) from Becker Muscular Dystrophy (BMD)?
- DMD: earlier onset, more severe
- BMD: later onset, less severe than DMD