BRS- Neuro Flashcards
(99 cards)
1
Q
Contrast hypotonia and weakness
A
- hypotonia: decreased resistance of movement during passive stretch
- weakness: less force for active contraction
2
Q
Cause of central vs peripheral hypotonia
A
- central: upper motor neuron dysfxn
- peripheral: LMN dysfxn
3
Q
Antenatal/neonatal signs of hypotonia
A
antenatal: decreased fetal movement, breech presentation (peripheral)
neonatal: seizures (central)
4
Q
PE findings in both central and peripheral hypotonia
A
decreased movement
frogleg posture
muscle contractures
5
Q
Central hypotonia workup
A
- CT
- serum electrolytes
- chromosome studies
6
Q
Peripheral hypotonia workup
A
- CK levels
- DNA tests
- EMG
- muscle biopsy
7
Q
Four possible areas lesioned in peripheral hypotonia
A
1) spinal cord
2) peripheral nerves
3) NMJ
4) muscle
8
Q
Three disorders affecting the NMJ/ causing hypotonia
A
- botulism
- myasthenia gravis
- mag tox
9
Q
Disorder affecting the spinal cord causing hypotonia?
A
spinal muscular atrophy
10
Q
Disorder affecting the peripheral nerves causing hypotonia
A
familial dysautonomia
11
Q
Two most common neuromuscular disorders?
A
#1: DMD #2: SMA
12
Q
SMA:
- affects what cells?
- chromosome
- gene
- inheritance
A
- anterior horn cells
- chromosome 5
- SMN1 (survival motor neuron)
- AR
13
Q
Three types of SMA:
A
1: infantile/ Werdnig Hoffman (less than 6 mos)
2: intermediate 6-12 mos
3: juvenile, less than 3 years
14
Q
3 PE findings in SMA
A
- tongue fasiculations
- bell shaped chest
- normal sensation
15
Q
Gold standard for diagnosis of SMA
A
DNA testing
16
Q
Prognosis for SMA:
A
type 1: rare to live beyond 1st year
II-II: survive to adolescent/adult hood
17
Q
Botulism:
- weakness type
- mechanism of weakness
- time from ingestion to onset
A
- bulbar weakness, symmetric and descending
- prevents presynaptic release of Ach
- 12-48 hours after ingestion of spores
18
Q
Treatment botulism
How long until recovery?
A
IVIG
abx contraindicated
full recovery in weeks –> months
19
Q
Congenital myotonic dystrophy:
- inheritance pattern
- parent affected
- time of onset
A
-AD
-mother
-myotonia onset at age 5
(but may have neonatal feeding/ respiratory trouble)
20
Q
Myotonic dystrophy:
- typical facial appearance
- typical IQ
- three other features
A
- temporalis/ masseter atrophy
- IQ 50-65
- cataracts, arrhythmias, infertility
21
Q
Three types of hydrocephalus:
A
- noncommunicating: obstruction
- communicating (^ production/ low reabsorption)
- hydrocephalus ex vacuo (atrophy)
22
Q
Describe Chiari II malformation
A
- lumbosacral myelomeningocele
- medullary/cerebellar displacement through foramen magnum
23
Q
Describe Dandy Walker malformation
A
-absent cerebellar vermis + dilation of the fourth ventricle
24
Q
Congenital aqueductal stenosis:
- inheritance pattern
- other assc abnormalities
A
- X linked
- thumb abnormalities
- spina bifida
25
Cause of sunset sign in hydrocephalus
-eyes down because third ventricle enlargement causes pressure on the upward gaze center of the midbrain
26
Nerve palsy suggestive of hydrocephalus
-unilateral CN6 palsy
27
First step in hydrocephalus workup
-urgent head CT
28
Spina Bifida is failure of fusion in what part of the vertebral column?
posterior midline
29
Three types of neural tube defects
- meningocele
- myelomeningocele
- SB occulta
30
What areas have the highest and lowest incidences of neural tube defects?
highest: Ireland
lowest: Japan
31
Meningocele PE finding
mass that transluminates
32
90% of lumbosacral myelomeningoceles are assc with ______.
Chiari II & hydrocephalus
33
PE findings with myelomeningocele:
- above L3
- below S3
above L3: paraplegia
| below S3: incontinence
34
How are NTDs diagnosed prenatally?
80% diagnosed by increased AFP in weeks 16-18
35
Myelomeningoceles must be surgically repaired within _____.
24 hours of birth
36
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
37
Drugs that may be assc with coma:
-alcohol, opiates, benzos, TCAs, atropine, lead/ mercury etc
38
Key PE components in case of coma:
- head and neck
| - scalp injuries, breath odor, nuchal rigidity, CSF/ Blood leakage
39
Describe decerebrate posturing
- extension of arms and legs
| - subcortical injury
40
Describe decorticate posturing
- bilateral cortical injury
| - flexion of arms and legs
41
Coma with no movement suggests _____
severe spine or brainstem injury
42
Coma with hypoventilation =
opiates, sedatives
43
Coma with hyperventilation=
metabolic acidosis
neurogenic pulmonary edema
midbrain injury
44
Cheyene Strokes Breathing
- pattern
- cause
- pattern: alternating apnea and hyperpnea
| - bilateral cortical injury
45
Apneustic Breathing
- pattern
- cause
- pausing at full inspiration
| - pontine injury
46
Ataxic/agonal breathing: cause
medullary injury/ impending brain death
47
Cause of unilateral dilated nonreactive pupil?
| bilateral?
uni: uncal herniation
bilateral: brainstem injury, postictal/dilating meds
48
Cause of bilateral pupillary constriction?
- opiates
| - pontine injury
49
Loss of oculocephalic reflex: cause
injury to brainstem
50
STAT tests in comatose patients
- CT
- LP if CT is negative
- gluc, UDS, BMP
- EEG
51
Define epilepsy
| Define status
2+ seizures without cause
| 30+ minutes
52
Afebrile seizures:
| generalized vs partial
generalized: starts in both hemispheres
partial: starts in one hemisphere
53
6 types of generalized seizures
- tonic
- clonic
- tonic clonic
- myoclonic
- absence
- atonic
54
Two types of partial seizures
simple vs complex (conscious vs unconscious)
55
Describe absence seizures
staring less than 15 seconds with no postictal states
56
Workup for first time afebrile seizure
none
| electrolytes and imaging with prior history
57
Workup needed in febrile seizures
- LP
- Cultures
- CBC
58
Treatment of status
- short acting benzo (lorazepam/diazepam)
| - loading dose of phenobarbital or phenytoin
59
Generalized epilepsy treatment:
- valproic acid
| - phenobarb
60
Partial epilepsy treatment
- carbamazepine
| - phenytoin
61
"Alternate" seizure treatment
ketogenic diet
| vagal nerve stimulator
62
Age range for febrile seizures
6 months --> 6 years
63
Contrast simple and complex febrile seizures
- simple less than 15 mins
| - comples longer than 15 minutes
64
Abortive treatment for febrile seizure
rectal diazepam
65
Infantile Spasms/ West Syndrome:
- most common cause
- EEG pattern
- treatment of choice
- TS
- hypsarrythmia
- ACTH
66
Describe appearance of infantile spasms:
-jack knife seizures
| arm extension, head flexion
67
Absence seizures:
- inheritance pattern
- sex
- EEG finding
- AD
- predominantly female
- 3 Hz generalized spike and wave pattern
68
DOC absence seizures
ethosuximide
69
Most common childhood partial epilepsy + inheritance pattern & sex
- Benign rolandic epilepsy-- AD
| - predominantly male
70
First line drug for benign temporal epilepsy + EEG pattern
- spike and wave pattern in temporal region on EEG
| - valproate= DOC
71
Systemic causes of anemia in kiddos
- anemia
- hypoglycemia
- depression
- HTN
72
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
73
Hormones/ Chemicals assc with migraine
Vasoactive intestinal Peptide
5HT
substance P
74
Most common form of migraine in kiddos?
migraine without aura
75
DOC for migraine px and abortion in kiddos
sumatriptan, propranolol
76
Two symptoms assc with cluster headache
- lacrimation
| - conjunctival erythema
77
Most common cause of ataxia in childhood
acute cerebellar ataxia (autoimmune, postinfectious)
| usually in kiddos under 10
78
Common infections preceding ACA
- varicella
- flu
- EBV
- mycoplasma
79
Workup of acute ataxia in kids
- neuroimaging
| - normal in ACA
80
Guillian Barre:
- bug
- weakness type
- campy jejuni
| - ascending weakness
81
Describe demyelination in GBS
-demyelination of the peripheral myelin nerves/ schwann cells via cell mediated immunity
82
Describe the miller fischer variant of GBS
- ataxia
- areflxia
- ophthalmoplegia
83
CSF findings in GBS
| Treatment
albuminocytologic dissociation
| IVIG
84
Sydenham chorea is assc with ______ and affects _____.
Rheumatic fever ; basal ganglia
85
PE findings in Sydenham chorea
- milkmaids grip
| - choreic hand
86
Diagnosis of Syd C:
- ASO/ADB titers
| - increased signal density at caudate and putamen
87
Treatment of syd C
Haldol
valproate
phenobarb
88
How long does it take sydenhams chorea to resolve
months- 2 years
89
When must tics be diagnosed in tourettes?
| How long must they be present?
before 18
| at least one year
90
Drug of choice in tourettes
pimozide
91
Duchenne and Becker Muscular Dystrophies:
-Inheritance Patterns
0assc gene
- X linked
| - dystrophin gene
92
Appearance of muscle in DMD
damaged muscle --> replaced by lipid deposits/ fibroblasts
93
When do children lose the ability to walk in DMD/BMD
DMD: 10
BMD: 20
94
Three PE findings in DMD
- Gowers sign
- Pseudohypertrophy of calves
- cardiac involvement
95
Diagnosis of DMD
- CK levels
- Muscle biopsy
- absent dystrophin
- EMG= weak potentials
96
Drug that increases strength in DMD
steroids
97
Myasthenia Gravis:
cause
sex most commonly affected
- AChR Abs
| - girls
98
MG:
- most common presenting sign
- classic test
- bilateral ptosis
| - edrophonium/ tensilon test
99
Treatment of choice for MG
-pyridostigmine (cholinesterase inhibitor)
| +/- thymectomy
