Peds Neuro Flashcards
LMN or UMN?
- Flaccid paralysis
- Decreased tone
- Absent DTR
- Fasciculations
- Atrophy
LMN
LMN or UMN?
- Spastic paralysis
- Increased tone/DTRs
- Minimal atrophy
- Stiffness
UMN
Prophylaxis for migraine headaches <6 y/o
Cyproheptadine
Prophylaxis for Migraine headaches >6y/o
Propanolol
Topiramate
Amitryptiline
Etc
- HA, blurred vision
- Diplopia, vision loss
- worse at night
- PE- Papilledema
Pseudotumor cerebri
Main complication of Pseudotumor cerebri?
Vision loss
How do you tx Pseudotumor cerebri? (3 pharm, 2 sx)
- Pharm:
- Decrease Na intake
- Acetazolamide
- Topiramate
- Sx:
- Optic nn. sheath fenestration
- CSF shunt
Who is Pseudotumor cerebri MC in?
Females of childbearing age (usually 11+)
Which type of CP?
- MC type
- UMN lesion/sxs
Spastic
What are the 4 types of CP
- Spastic (MC)
- Athetoid/Dyskinetic
- Ataxic (most rare)
- Atonic
Which type of CP?
- Slow, smooth, writhing movements that involve distal muscles
Athetoid
WHich type of CP?
- Decreased spontaneous movement
- Hypotonia
- Suppressed primitive reflexes
Dsykinetic CP
Which type of CP?
- Wide-based gait
- intention tremor
- slow
- jerking movments
Ataxic CP (most rare)
Which type of CP?
- Severe hypotonia
- Never stand or walk
- Microcephaly
- Profound intellectual disability
Atonic CP
What is a myelomeningocele
- This is a type of spina bifida
- Meninges and spinal cord exposed
What is a meningocele
Spinal canal and meninges exposed
Underlying spinal cord intact
Type of spina bifida
What does a dimple or tuft of hair indicate?
Spina bifida Occulta
How do you manage Spina Bifida? (2)
Surgical closure
VP shunt
Almost all patients with a myelomeningocele (type of spina bifida) also have what type of formation?
Most also have associated what?
Chiari II malformation (cerebellum herniates)
Associated hydrocephalus
What is seen on vitals in a patient with Hydrocephalus? (3)
- Bradycardia
- HTN
- Altered respiration
What 5 things are seen on PE in a pt with Hydrocephalus
1. Increased head circumference
2. Bulging anterior fontanelle
- Abnormal skull contour
- CN dysfunction
- Papilledema
How do you tx hydrocephalus? (2 for sxs, 2 surgical interventions)
- sxs- Loop diuretic, Acetazolamide
- Sx: VP shunt, remove obstructive lesion
What confirms dx of spinal muscular atrophy? (related to genes)
Homozygous deletion of exon 7 of SMN1
What is spinal muscular atrophy? How is it inherited
Progressive weakness of LMNs
Autosomal recessive
- Decreased spont. movement
- floppiness
- Decreased facial expression
- Drooling
Spinal muscular atrophy
When does spinal muscular atrophy begin?
6mo-6yrs
What 3 tests are done to dx spinal muscular atrophy?
- EMG
- Muscle bx
- DNA testing
How do you tx spinal muscular atrophy?
No tx-
symptom therapy (improve flexibilty, prevent infections, maintain language/intellectual stimulation)
What causes Guillain Barre Syndrome?
Campylobacter Jejuni
Hallmarks of what condition?
- Ascending weakness
- Loss DTRs
- No walking/leg pain
GBS
How do you dx GBS? (2)
- LP w/ increased CSF protein WITHOUT increase in WBC
- EMG
What is death due to in GBS?
from autonomic dysfunction, respiratory failure
How do you tx GBS? (2)
IVIG
Plasmapheresis
How is Duchenne Muscular Dystrophy inherited?
X-linked recessive- only boys
What protein is missing in Duchenne Muscular Dystrophy>
No dystrophin protein
Gower sign
Calf hypertrophy
Waddling gait
Duchenne Muscular Dystrophy
HOw do you dx Duchenne Muscular Dystrophy? (4)
- Muscle Biopsy
- EMG
- Serum CK (10-20x higher than normal)
- Genetic testing
How do you tx Duchenne Muscular dystrophy?
- Glucocorticoids (extend independent ambulation by 2.5yrs)- started b/w 4-8y/o
- Supportive
Pts with Duchenne Muscular Dystrophy are usually wheelchair bound by what age?
12 y/o
How do pts w/ Duchenne Muscular dystrophy die and at what age? What is the avg lifespan?
Death due to PNA or CHF in early 20s
Avg lifespan=25 yrs
Duchenne Muscular Dystrophy- Loss of UE movement by what age
16
Type 1 Neurofibromatosis assoc. w/ what chrom
chrom 17
Type 2 Neurofibromatosis assoc. w/ what chrom
Chrom 22
Neurofibromatosis type 1 or 2?
- Skin and brain abnormalities= ____
- More severe, spinal cord abnromalities, intracranial and spinal tumors=____
- Skin and brain abnormalities= type 1
- More severe, spinal cord abnromalities, intracranial and spinal tumors=type 2
Neurofibromatosis type 1 or 2?
- Cafe-au-lait spots
- Neurofibromas
- Seizures
- macrocephaly
*
Type 1
Neurofibromatosis type 1 has increased incidence of what?
CNS tumors- astrocytoma, meningioma, schwannoma, etc
How do you tx Neurofibromatosis type 1
Surgical excision of tumors
Genetic screening for fam
What are 6 complications of Neurofibromatosis type 1
- Seizures
- Deafness
- Short stature
- Early puberty
- HTN
- Optic Glioma
Neurofibromatosis type 1 or 2?
- Autosomal dominant
- Vastibular Schwannoma
- NO Cafe-au-lait spots
- May present as loss of hearing in kids
Neurofibromatosis type 2
Febrile seizures are seen at what age?
6mo-5y/o
How do you tx Febrile sz if lasting longer than 5 min
IV benzos or Buccal Midazolam if no IV access