BRS- Neuro Flashcards
Contrast hypotonia and weakness
- hypotonia: decreased resistance of movement during passive stretch
- weakness: less force for active contraction
Cause of central vs peripheral hypotonia
- central: upper motor neuron dysfxn
- peripheral: LMN dysfxn
Antenatal/neonatal signs of hypotonia
antenatal: decreased fetal movement, breech presentation (peripheral)
neonatal: seizures (central)
PE findings in both central and peripheral hypotonia
decreased movement
frogleg posture
muscle contractures
Central hypotonia workup
- CT
- serum electrolytes
- chromosome studies
Peripheral hypotonia workup
- CK levels
- DNA tests
- EMG
- muscle biopsy
Four possible areas lesioned in peripheral hypotonia
1) spinal cord
2) peripheral nerves
3) NMJ
4) muscle
Three disorders affecting the NMJ/ causing hypotonia
- botulism
- myasthenia gravis
- mag tox
Disorder affecting the spinal cord causing hypotonia?
spinal muscular atrophy
Disorder affecting the peripheral nerves causing hypotonia
familial dysautonomia
Two most common neuromuscular disorders?
#1: DMD #2: SMA
SMA:
- affects what cells?
- chromosome
- gene
- inheritance
- anterior horn cells
- chromosome 5
- SMN1 (survival motor neuron)
- AR
Three types of SMA:
1: infantile/ Werdnig Hoffman (less than 6 mos)
2: intermediate 6-12 mos
3: juvenile, less than 3 years
3 PE findings in SMA
- tongue fasiculations
- bell shaped chest
- normal sensation
Gold standard for diagnosis of SMA
DNA testing
Prognosis for SMA:
type 1: rare to live beyond 1st year
II-II: survive to adolescent/adult hood
Botulism:
- weakness type
- mechanism of weakness
- time from ingestion to onset
- bulbar weakness, symmetric and descending
- prevents presynaptic release of Ach
- 12-48 hours after ingestion of spores
Treatment botulism
How long until recovery?
IVIG
abx contraindicated
full recovery in weeks –> months
Congenital myotonic dystrophy:
- inheritance pattern
- parent affected
- time of onset
-AD
-mother
-myotonia onset at age 5
(but may have neonatal feeding/ respiratory trouble)
Myotonic dystrophy:
- typical facial appearance
- typical IQ
- three other features
- temporalis/ masseter atrophy
- IQ 50-65
- cataracts, arrhythmias, infertility
Three types of hydrocephalus:
- noncommunicating: obstruction
- communicating (^ production/ low reabsorption)
- hydrocephalus ex vacuo (atrophy)
Describe Chiari II malformation
- lumbosacral myelomeningocele
- medullary/cerebellar displacement through foramen magnum
Describe Dandy Walker malformation
-absent cerebellar vermis + dilation of the fourth ventricle
Congenital aqueductal stenosis:
- inheritance pattern
- other assc abnormalities
- X linked
- thumb abnormalities
- spina bifida
Cause of sunset sign in hydrocephalus
-eyes down because third ventricle enlargement causes pressure on the upward gaze center of the midbrain
Nerve palsy suggestive of hydrocephalus
-unilateral CN6 palsy
First step in hydrocephalus workup
-urgent head CT
Spina Bifida is failure of fusion in what part of the vertebral column?
posterior midline
Three types of neural tube defects
- meningocele
- myelomeningocele
- SB occulta
What areas have the highest and lowest incidences of neural tube defects?
highest: Ireland
lowest: Japan
Meningocele PE finding
mass that transluminates
90% of lumbosacral myelomeningoceles are assc with ______.
Chiari II & hydrocephalus
PE findings with myelomeningocele:
- above L3
- below S3
above L3: paraplegia
below S3: incontinence
How are NTDs diagnosed prenatally?
80% diagnosed by increased AFP in weeks 16-18
Myelomeningoceles must be surgically repaired within _____.
24 hours of birth
Two most common causes of coma in children under 5?
older than 5?
- young: drowning and nonaccidental trauma
- older: accidental head injury and drug OD
Drugs that may be assc with coma:
-alcohol, opiates, benzos, TCAs, atropine, lead/ mercury etc
Key PE components in case of coma:
- head and neck
- scalp injuries, breath odor, nuchal rigidity, CSF/ Blood leakage
Describe decerebrate posturing
- extension of arms and legs
- subcortical injury
Describe decorticate posturing
- bilateral cortical injury
- flexion of arms and legs
Coma with no movement suggests _____
severe spine or brainstem injury
Coma with hypoventilation =
opiates, sedatives
Coma with hyperventilation=
metabolic acidosis
neurogenic pulmonary edema
midbrain injury
Cheyene Strokes Breathing
- pattern
- cause
- pattern: alternating apnea and hyperpnea
- bilateral cortical injury
Apneustic Breathing
- pattern
- cause
- pausing at full inspiration
- pontine injury
Ataxic/agonal breathing: cause
medullary injury/ impending brain death
Cause of unilateral dilated nonreactive pupil?
bilateral?
uni: uncal herniation
bilateral: brainstem injury, postictal/dilating meds
Cause of bilateral pupillary constriction?
- opiates
- pontine injury
Loss of oculocephalic reflex: cause
injury to brainstem
STAT tests in comatose patients
- CT
- LP if CT is negative
- gluc, UDS, BMP
- EEG
Define epilepsy
Define status
2+ seizures without cause
30+ minutes
Afebrile seizures:
generalized vs partial
generalized: starts in both hemispheres
partial: starts in one hemisphere
6 types of generalized seizures
- tonic
- clonic
- tonic clonic
- myoclonic
- absence
- atonic
Two types of partial seizures
simple vs complex (conscious vs unconscious)
Describe absence seizures
staring less than 15 seconds with no postictal states
Workup for first time afebrile seizure
none
electrolytes and imaging with prior history
Workup needed in febrile seizures
- LP
- Cultures
- CBC
Treatment of status
- short acting benzo (lorazepam/diazepam)
- loading dose of phenobarbital or phenytoin
Generalized epilepsy treatment:
- valproic acid
- phenobarb
Partial epilepsy treatment
- carbamazepine
- phenytoin
“Alternate” seizure treatment
ketogenic diet
vagal nerve stimulator
Age range for febrile seizures
6 months –> 6 years
Contrast simple and complex febrile seizures
- simple less than 15 mins
- comples longer than 15 minutes
Abortive treatment for febrile seizure
rectal diazepam
Infantile Spasms/ West Syndrome:
- most common cause
- EEG pattern
- treatment of choice
- TS
- hypsarrythmia
- ACTH
Describe appearance of infantile spasms:
-jack knife seizures
arm extension, head flexion
Absence seizures:
- inheritance pattern
- sex
- EEG finding
- AD
- predominantly female
- 3 Hz generalized spike and wave pattern
DOC absence seizures
ethosuximide
Most common childhood partial epilepsy + inheritance pattern & sex
- Benign rolandic epilepsy– AD
- predominantly male
First line drug for benign temporal epilepsy + EEG pattern
- spike and wave pattern in temporal region on EEG
- valproate= DOC
Systemic causes of anemia in kiddos
- anemia
- hypoglycemia
- depression
- HTN
Most common cause of headaches in children and adolescents. More common in boys or girls? Inheritance pattern?
migraine
AD
Common in boys before puberty, girls after
Hormones/ Chemicals assc with migraine
Vasoactive intestinal Peptide
5HT
substance P
Most common form of migraine in kiddos?
migraine without aura
DOC for migraine px and abortion in kiddos
sumatriptan, propranolol
Two symptoms assc with cluster headache
- lacrimation
- conjunctival erythema
Most common cause of ataxia in childhood
acute cerebellar ataxia (autoimmune, postinfectious)
usually in kiddos under 10
Common infections preceding ACA
- varicella
- flu
- EBV
- mycoplasma
Workup of acute ataxia in kids
- neuroimaging
- normal in ACA
Guillian Barre:
- bug
- weakness type
- campy jejuni
- ascending weakness
Describe demyelination in GBS
-demyelination of the peripheral myelin nerves/ schwann cells via cell mediated immunity
Describe the miller fischer variant of GBS
- ataxia
- areflxia
- ophthalmoplegia
CSF findings in GBS
Treatment
albuminocytologic dissociation
IVIG
Sydenham chorea is assc with ______ and affects _____.
Rheumatic fever ; basal ganglia
PE findings in Sydenham chorea
- milkmaids grip
- choreic hand
Diagnosis of Syd C:
- ASO/ADB titers
- increased signal density at caudate and putamen
Treatment of syd C
Haldol
valproate
phenobarb
How long does it take sydenhams chorea to resolve
months- 2 years
When must tics be diagnosed in tourettes?
How long must they be present?
before 18
at least one year
Drug of choice in tourettes
pimozide
Duchenne and Becker Muscular Dystrophies:
-Inheritance Patterns
0assc gene
- X linked
- dystrophin gene
Appearance of muscle in DMD
damaged muscle –> replaced by lipid deposits/ fibroblasts
When do children lose the ability to walk in DMD/BMD
DMD: 10
BMD: 20
Three PE findings in DMD
- Gowers sign
- Pseudohypertrophy of calves
- cardiac involvement
Diagnosis of DMD
- CK levels
- Muscle biopsy
- absent dystrophin
- EMG= weak potentials
Drug that increases strength in DMD
steroids
Myasthenia Gravis:
cause
sex most commonly affected
- AChR Abs
- girls
MG:
- most common presenting sign
- classic test
- bilateral ptosis
- edrophonium/ tensilon test
Treatment of choice for MG
-pyridostigmine (cholinesterase inhibitor)
+/- thymectomy
