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Flashcards in BRS- Neuro Deck (99):
1

Contrast hypotonia and weakness

-hypotonia: decreased resistance of movement during passive stretch
-weakness: less force for active contraction

2

Cause of central vs peripheral hypotonia

-central: upper motor neuron dysfxn
-peripheral: LMN dysfxn

3

Antenatal/neonatal signs of hypotonia

antenatal: decreased fetal movement, breech presentation (peripheral)
neonatal: seizures (central)

4

PE findings in both central and peripheral hypotonia

decreased movement
frogleg posture
muscle contractures

5

Central hypotonia workup

-CT
-serum electrolytes
-chromosome studies

6

Peripheral hypotonia workup

-CK levels
-DNA tests
-EMG
-muscle biopsy

7

Four possible areas lesioned in peripheral hypotonia

1) spinal cord
2) peripheral nerves
3) NMJ
4) muscle

8

Three disorders affecting the NMJ/ causing hypotonia

-botulism
-myasthenia gravis
-mag tox

9

Disorder affecting the spinal cord causing hypotonia?

spinal muscular atrophy

10

Disorder affecting the peripheral nerves causing hypotonia

familial dysautonomia

11

Two most common neuromuscular disorders?

#1: DMD
#2: SMA

12

SMA:
-affects what cells?
-chromosome
-gene
-inheritance

-anterior horn cells
-chromosome 5
-SMN1 (survival motor neuron)
-AR

13

Three types of SMA:

1: infantile/ Werdnig Hoffman (less than 6 mos)
2: intermediate 6-12 mos
3: juvenile, less than 3 years

14

3 PE findings in SMA

-tongue fasiculations
-bell shaped chest
-normal sensation

15

Gold standard for diagnosis of SMA

DNA testing

16

Prognosis for SMA:

type 1: rare to live beyond 1st year
II-II: survive to adolescent/adult hood

17

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

18

Treatment botulism
How long until recovery?

IVIG
abx contraindicated
full recovery in weeks --> months

19

Congenital myotonic dystrophy:
-inheritance pattern
-parent affected
-time of onset

-AD
-mother
-myotonia onset at age 5
(but may have neonatal feeding/ respiratory trouble)

20

Myotonic dystrophy:
-typical facial appearance
-typical IQ
-three other features

-temporalis/ masseter atrophy
-IQ 50-65
-cataracts, arrhythmias, infertility

21

Three types of hydrocephalus:

-noncommunicating: obstruction
-communicating (^ production/ low reabsorption)
-hydrocephalus ex vacuo (atrophy)

22

Describe Chiari II malformation

-lumbosacral myelomeningocele
-medullary/cerebellar displacement through foramen magnum

23

Describe Dandy Walker malformation

-absent cerebellar vermis + dilation of the fourth ventricle

24

Congenital aqueductal stenosis:
-inheritance pattern
-other assc abnormalities

-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