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Flashcards in BRS- Neuro Deck (99)
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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