Neuropathy and myopathy Flashcards

1
Q

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

A

LMN

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2
Q

What is the classic disease of the anterior horn?

A

Polio

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3
Q

UMN and LMN s/sx = what disease?

A

ALS

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4
Q

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

A
UMN = hyperactive
LMN = hypoactive
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5
Q

What is the MOA of botulinum toxin?

A

Cleavage of SNARE proteins prevents the release of vesicles containing ACh

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6
Q

What is bulbar weakness?

A

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

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7
Q

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

A

Neuropathy = greater distal weakness

Myopathy = greater proximal weakness

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8
Q

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

A

LMN

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9
Q

What defines acute vs chronic weakness of neuromuscular disorders?

A
Acute = days-weeks
Chronic = months to years
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10
Q

Anterior horn cell damage = what type of deficit?

A

Motor only–no sensory

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11
Q

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

A

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

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12
Q

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

A

MRIs

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13
Q

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

A

Nerve conduction test

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14
Q

Fasciculations is a hallmark of UMN or LMN lesions?

A

LMN

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15
Q

What is the amyotrophic part ALS?

A

symptoms of weakness, atrophy, and fasciculations

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16
Q

What is the lateral sclerosis part of ALS?

A

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

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17
Q

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

A

Lateral sclerosis

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18
Q

What is the mean age of onset of ALS?

A

58 years

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19
Q

What is the incidence of ALS?

A

1/100,000

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20
Q

What is the prognosis with ALS?

A

Variable–Respiratory involvement is much worse than limb involvement

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21
Q

Bulbar involvement with ALS leads to what?

A

Flaccid dysarthria, tongue atrophy, fasciculations

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22
Q

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

A

True

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23
Q

How does ALS usually present initially?

A

Single limb issue

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24
Q

What are fasciculations, generally?

A

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

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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?