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Flashcards in Neuropathy and myopathy Deck (75)
1

Diseases in the anterior horn lead to LMN or UMN disease?

LMN

2

What is the classic disease of the anterior horn?

Polio

3

UMN and LMN s/sx = what disease?

ALS

4

Which has hyperactive, and which hypoactive reflexes: UMN and LMN lesions

UMN = hyperactive
LMN = hypoactive

5

What is the MOA of botulinum toxin?

Cleavage of SNARE proteins prevents the release of vesicles containing ACh

6

What is bulbar weakness?

The bulb is part of the lower pons, so dysarthria and dysphagia

7

Which has greater proximal and which has greater distal weakness: myopathy vs neuropathy?

Neuropathy = greater distal weakness

Myopathy = greater proximal weakness

8

Stocking glove distribution of sensation loss is usually a LMN or UMN lesion?

LMN

9

What defines acute vs chronic weakness of neuromuscular disorders?

Acute = days-weeks
Chronic = months to years

10

Anterior horn cell damage = what type of deficit?

Motor only--no sensory

11

How do you differentiate between demyelination and cell body nerve problems?

Demyelination can have relapses, while cell body lesions do not.

12

UMN lesions what is the diagnostic modality that is especially helpful?

MRIs

13

LMN lesions what is the diagnostic modality that is especially helpful?

Nerve conduction test

14

Fasciculations is a hallmark of UMN or LMN lesions?

LMN

15

What is the amyotrophic part ALS?

symptoms of weakness, atrophy, and fasciculations

16

What is the lateral sclerosis part of ALS?

UMN in the DC/ML pathway, producing s/sx of hyperactive DTRs, clonus, and babinski signs

17

Which has a better prognosis: the lateral sclerosis or the amyotrophic form of ALS?

Lateral sclerosis

18

What is the mean age of onset of ALS?

58 years

19

What is the incidence of ALS?

1/100,000

20

What is the prognosis with ALS?

Variable--Respiratory involvement is much worse than limb involvement

21

Bulbar involvement with ALS leads to what?

Flaccid dysarthria, tongue atrophy, fasciculations

22

True or false: for the most part, ALS spares cognition, EOMs, and sphincters

True

23

How does ALS usually present initially?

Single limb issue

24

What are fasciculations, generally?

"Worm-like" movement beneath the skin of a patient that does not move a limb

25

How do you diagnose ALS?

-Clinical diagnosis is correct more than 95% of the time

26

What are the antibodies that can be tested for in multi-focal motor neuropathy?

Anti-GM1 antibodies

27

Which usually has eye findings: MG or ALS?

MG

28

How often are fasciculations in people in general (without ALS)? How do you differentiate this with ALS?

70%

No weakness/atrophy

29

What is the diagnostic criteria for ALS?

UMN and LMN signs with sensattion spared in:
-bulbar region and 2 spinal regions
OR
-three spinal regions

30

What is the cause of ALS?

Unknown--can be familial, but usually sporadic

31

What is the symptomatic treatment for ALS?

PT/OT
Orthotics

32

What is the MOA of Riluzole in treating ALS?

Glutamate antagonist

33

What are the usual causes of mononeuropathies?

Single nerve damaged, leading to deficits in a single nerve distribution

34

What is mononeuritis multiplex?

When two or more (typically just a few, but sometimes many) separate nerves in disparate areas of the body are affected it is called "mononeuritis multiplex,"

35

What is allodynia?

Neuropathic pain following nonpainful stimuli

36

What is dysesthesia?

Unpleasant sensation that is either spontaneous or evoked by pressure, movement, or touching

37

What is hyperalgesia?

heightened pain sensation provoked by painful stimulus

38

What are paresthesias?

Irritating spontaneous sensation

39

What are the motor signs of peripheral neuropathies?

Distal weakness and foot drop

40

What are the sensory findings with peripheral neuropathies?

Loss of vibratory, position, and/or pin

41

What are reflexes like with peripheral neuropathies?

Hypoactive to absent

42

What is the role of EMG in diagnosing peripheral neuropathies?

Helpful in distinguishing axonal vs demyelinating neuropathy

43

What is the most common type of neuropathy?

DM neuropathy

44

What is toxic neuropathies?

Uremia induced neuropathy, or extrinsic toxins of neuropathy

45

What are the two major extrinsic toxins that can cause neuropathies?

EtOH
Chemotherapy

46

What are the two major immune mediated neuropathies?

AIDP-(acute inflammatory demyelinating polyneuropathy) = GB

CIDP (Chronic Inflammatory Demyelinating Polyneuropathy)

47

What is Charcot-Marie-Tooth disease (hereditary motor sensory neuropathy)?

AD disease caused by mutations that cause defects in neuronal proteins involved in the production and function of peripheral nerves

48

What are the classic foot findings of CMT disease?

Pes cavus and hammer toe

49

What is the expressivity of CMT?

Variable

50

What is the frequency of CMT?

1/2500

51

What is the most common inherited neurological disorder?

HSMN (CMT)

52

What is the mode of inheritance of CMT?

AD

53

What is the usual presentation of CMT?

Slow, progressive distal weakness; difficulty walking

54

What is a steppage gait?

Bring the knees high to clear the anterior tibialis

55

What are the two types of CMT? What are the EMG findings of each?

Demyelinating (slow NCV)
Axonal (normal NCV)

56

How do you definitely diagnose CMT?

Genetic testing

57

GB is a motor or sensory neuropathy?

Motor--sensation is intact

58

What is the classic CSF finding of GB?

Albuminocytological dissociation (no WBC increase, but marked protein)

59

What are the two viruses that can cause GB?

CMV
EBV

60

What are the two bacteria that predispose to GB?

-Campylobacter jejuni
-Mycoplasma pneumoniae

61

What are the usual s/sx of GB?

Difficulty walking

62

What fraction of GB syndrome patients develop respiratory issues?

1/3

63

What is the Fisher syndrome variant of GB syndrome?

Starts with ophthalmoplegia, ataxia, and loss of DTRs

64

What is the time course of GB disease?

50% reach nadir in 2 weeks, with slow recovery over months

65

What percent of patients who develop GB have residual symptoms?

15-20%

66

Suspected GB syndrome, but with marked pleocytosis = ?

HIV

67

Presence of fibrillations with GB implies a shorter or longer course?

Longer

68

What is the treatment for GB syndrome?

Plasma exchange or IVIG

69

Which usually has more pronounced atrophy: LMN lesions or UMN lesions?

LMN

70

The cell body of a LMN is located where?

ventral horn of the spine cord

71

What is the MOA of tetanus toxin?

Cleaves synaptobrevin, leading to blocked release of GABA and glycince across the synaptic cleft

72

True or false: the tongue is never affected with ALS

False--can have hypoglossal nerve involvement, leading to atrophy of the tongue

73

Which nerves are more myelinated:DC/ML nerves, or spinothalamic tract nerves?

DC/ML

74

Inverted champagne bottle appearance of the leg = which disease?

CMT

75

What is the difference between GB and CIDP?

progression over 2 months suggests CIDP?