Neurology Flashcards
central hypotonia is dysfunction of
upper motor neurons
peripheral hypotonia is dysfunction of
lower motor neurons
common history found in hypotonia
decreased fetal movements
breech presentations
seizure in neotnatal period
PE finding for hypotonia
frog-leg position
decrease spontaneous movement
what studies should be worked up for central hypotonia
- head CT
- serum electorlytes
- chromosome study
studies for peripheral hypotonia
- Creatinine kinase
- DNA tests
- nerve conduction studies
- muscle biopsy
Spinal muscular atrophy
anterior motor horn cell degeneration
Werdnig-Hoffman disease
type I spinal muscular atrophy
infantile form
inheritance of spinal muscular atrophy
AR
SNM1 chromosome 5
clinical feature of spinal muscular atrophy
weak cry tongue fasciculations bell-shaped chest frog-leg position normal eye movement and sensory
Diagnosis for spinal muscular atrophy
DNA testing
muscle biopsy
management for spinal muscular atrophy
supportive
toxin of infantile botulism MOA
prevents presynaptic release of acetylcholine
classic first symptoms of infantile botulism
constipation
clinical feature of infantile botulism
- ophthalmoplegia and hyporeflexia
- paralysis is symmetric and descending
Management for infantile botulism
Botulism immune globulin
NO ANTIBIOTICS
myotonia
inability to relax muscles
all survivors of congenital myotonic dystrophy have what
mental retardation
how is congenital myotonic dystrophy inherited
AD
from mom
Hydrocephalus ex vacuo
ventricular enlargement caused by brain atrophy
Chiari type II
downward displacement of cerebellum and medulla through foramen magnum
Chiari type II is associated with
lumbosacral myelomeningocele
Dandy-Walker malformation
absent of hypoplastic cerebellar vermis
- cystic enlargement of 4th ventricle
sunset sign
tonic downward deviation of bot eyes caused by pressure from enlarged third ventricle
Myelomeningocele
herniation of spinal cord tissue and meninges
Spina bifida occulta
no hernication
lumbosacral myelomeningoceles assoicated wtih
Chiari type II malformation and hydrocephalus
what serum is elevated in spina bifida
alpha-fetoprotein
Cheyne stroke breathing indicates
bilateral cortical injury
Apneustic breathing indicates
pontine damage
unilateral dilatred nonreactive pupil suggests
uncal herniation
bilateral constricted reactive pupils suggests
opiate ingestions
pontine
oculocephalic maneuver (doll’s eyes)? if this does not work , what does that mean?
turn head of unconconsious person
- eyes should follow back to midline
- injured brainstem
Caloric irrigation
head at 30 degrees
cold water in ear
- eye should move to irrigated side
what must be checked immediately in a camotose patient
glucose
2 types of generalized seizures
- tonic clonic
2. Absence seziure
simple partial seizure
consciousness not impaired
complex partial seizure
consciousness is decreased
first time afebrile seizure in a healthy child with normal neurologic examination
no further investigation
what must be ruled out for febrile seizure
CNS infection
treatment for status epilepticus
- IV short acting Benzo
- Phenobarbital or phenytion
treatment for absence epilepsy
ethosuximide ``
alternative treatment options for epilepsy
- vagal nerve stimulator
- ketogenic diet
management for recurrent frequent febrile seizure
daily anticonvulsant prophylaxis
rectal diazepam
3 common epileptic syndrome
- infantile spasms
- absence
- benign rolandic epilepsy
common cause of infantile spasms
tuberous sclerosis
Hypssarrhytmia pattern
west syndrome ( infantile spasms)
Management of West syndrome
ACTH
Valproic acid
Vigabatrin
outlook for West syndrome
poor, mental retardation
generalized 3-Hz spike and wave discharge
absence seizure
second line treatment for absence
valproic acid
spike and sharp wave disturbance in mid-temporal and central regions
Benign rolandic epilepsy
clinical feature of Rolandic epilepsy
early morning hours
oral buccal manifestations
migrane
unilateral associated with nausea, vomiting or visual changes
