Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

1111 NEUROBIOLOGY > Anterior Horn and NMJ > Flashcards

Anterior Horn and NMJ Flashcards

1
Q

Anterior horn cell disorders (4)

A

ALS
SMA - spinal muscular atrophy
Polio
West nile virus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Radiculopathies and plexopathies (4)

A

cervical spine
lumbar spine
brachial plexus
lumbosacral plexus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Mononeuropathies (3)

A

carpal tunnel
ulnar palsy
peroneal palsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Polyneuropathies (5 causes)

A 
genetic (CMT)
Diabetes
systemic illness
vitamin deficiency 
toxic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

NMJ disorders (4)

A

myasthenia
LEMS
Botulism
Organophosphate poisoning

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Myopathies (4)

A

dystrophies
myositis
metabolic
toxic and endocrine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

signs and symptoms characterizing UMN disorders

A

spastic tone
increased reflexes
emotional lability

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

signs and symptoms characterizing LMN disorders

A

decreased tone
decreased reflexes
atrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

ALS

Average onset

A

54

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

ALS

males vs females

A

males 60%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what percent of ALS is inherited

A

5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Onset presentation of ALS

A

Leg>arm/bulbar; multiple sites

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

ALS may spread how?

A

along neuraxis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

mortality in ALS

A

median 3.5 years

worse with bulbar / respiratory onset and elderly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Treatment ALS

A

Riluzole - slows progression / extends life 3-6 months

Comp/Alt medicine

Symptom - improve QOL / assistive devices

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

ALS treatment of cramps/spasticity

consideration?

A

hydration/avoid overexertion

benzos

phenytoin

quinine

lioresal

dantrolene

gabapentin

consideration: helps jaw quivering/clenching but can increase weakness and decrease FVC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Treatment of pseudobulbar affect in ALS

A

Ask about it - they don’t volunteer information

Tricyclic and SSRI

Dextromethorphan + quinidine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Treatment of drooling/laryngospasm in ALS

A

To decrease saliva: glycopyrrolate, amitriptyline, diphenhydramine, oxybutinin, scopalamine, artane, salvary gland irradiation

for thick saliva: metoprolol, propanolol

nasal congestion: decongestants, antihistamines

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

ALS treatment of dysphagia and dysarthria

A

Tips to reduce choking: chin tuck, multiple swallows, celaring cough

PEG indications =
>1hr to eat meal
weight loss
silent aspiration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Treatment of sleep disturbance in ALS

A

avoid short acting sedatives

test for desaturation and consider nocturnal NIPPV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Causes of sleep disturbance in ALS?

A 
Could be multiple
depression 
anxiety 
apnea
periodic movements 
GERD
pain
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Charcot Marie Tooth Disease

Characterization -

A

hereditary neuropathies
hands and feet are weak
demyelination (few myelin profiles; digestion chambers)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

CMT1A/HNPP

What causes cmt1a

A

A 1.4 Mb duplication on chr 17 (PMP gene) causes CMT1A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What characterizes CMT1A

A

Distal weakness, sensory loss, and deformities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What percent of CMT1A mutations are de novo?

A

10

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Genetic cause of CMT1A

A

Repeats on either side of gene (CMT1A) are hot spot for recombination

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What results from deletion of CMT1A gene?

A

HNPP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What characterizes HNPP?

A

Focal weakness and sensory loss

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Multidisciplinary treatment approach to CMT

A

Physical therapy - exercise and range of motion

Occupational therapy - orthotics and assistive devices - cane, walker, easy to use utensils

Orthopedic surgery - correct deformities - ankle fusion, tendon transplants

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What characterizes sensory or sensorimotor distal polyneuropathies?

A

Stocking glove numbness and burning pain

foot drop and weak hand muscles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What characterizes autonomic diabetic neuropathy

A

hypotension, diarrhea, impotence, urinary retention

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What nerves can be impacted by diabetic mononeuropathy?

A

cranial nerve (3,6,7) or peripheral (median, ulnar, peroneal)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What characterizes lumbosacral plexopathy?

A

pelvic girdle pain,

asymmetric hip flexor weakness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What nerve causes carpal tunnel?

A

median nerve

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Treatment of diabetic polyneuropathy

A

Good metabolic control of diabetes

foot hygiene to avoid ulceration / infection

pain management - anticonvulsants, antidepressants, analgesics

autonomic symptoms
diarrhea - codeine, diphenoxylate
hypotension - fluorocortisone, midodrine
urinary retention - regular voiding, suprapubic pressure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Myasthenia gravis

Ocular finding?

A

ptosis

ophthalmoparesis - weakness of one or more of the external ocular muscles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Are jaw and neck weakness characteristic of MG?

A

Yes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

MG treatment?

A

Tensilon (acetylcholinesterase inhibitor)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Thymus in MG?

A

Abnormal hyperplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What is MG associated with?

A 
other autoimmune disorders:
Thyroid disease
RA
SLE
Pernicioius anemia
nephrotic syndrome
thrombotic thrombocytopenic purpura
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Treatment of MG in addition to tensilon?

A 
pyridostigmine
steroids
azathioprine
plasmapheresis targeting the ab
thymectomy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

complications of MG steroid use

A 
cataracts
infection
hypertension
obesity 
osteoporosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

DMD - muscle biopsy findings

A

fibrosis
degeneration
opaque fibers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

DMD - mutant?

A

dystrophin gene and protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Genetic therapeutic strategies for DMD

A

Viral gene introduction

Exon skipping

Read through

upregulate utrophin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Symptoms of DMD

A

Progressive contractures
progressive scoliosis
restrictive breathing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

____ diseases produce spastic tone, hyperactive tendon reflexes, and pathologic reflexes, emotional lability

A

UMN

48
Q

____ disease produce muscle atrophy, fasciculations, diminished tone

A

LMN

49
Q

_____ all motor neuron, neuromuscular junction, and muscle diseases have no sensory changes accompanying the weakness. Coexisting sensory complaines suggests ____

A

a nerve root, plexus, or peripheral nerve disorder

50
Q

rapidly developing weakiness is characteristic of

A

NMJ disorders such as MG, botulism, and organophosphhate poisoning

51
Q

organophosphate poisoning -

A

inhibition of acetylcholinesterase leads to buildup of acetylcholine - like in nerve gas

52
Q

acute demyelination (GB), electrolyte disturbance, and toxic myopathies can all produce_

A

acute weakness

53
Q

Neuromuscular disorders are characterized by

A

insidious, slow progressive weakness

54
Q

proximal weakness is most characterisitc of

A

muscle disorders

55
Q

distal weakness is most characteristic of

A

neuropathies

56
Q

____ weakness affects extraocular, facial, and oropharyngeal muscles and are often seen in MG

A

Cranial weakness

57
Q

limb disuse can yield atrophy but with neuromuscular disorders, msucle atrophy is accompanied by…

A

striking weakness

58
Q

in addition to muscle weakness what other signs and symptoms are there of neuromuscular disorders

A 
pain (myalgia) 
tenderness 
cramps
siffness
scoliosis 
contractures 
decreased tone
59
Q

contracture

A

loss of range of motion at a join

60
Q

Enzyme studies

A

muscle necrosis results in elevated levels of CK… muscular dystrophies and inflammatory myopathies have moderate elevations

61
Q

Electrodiagnostic studies

A

NCS
EMG
repetitive nerve stimulation

62
Q

NCS distinguish between

A

primary demyelinating (Very slow NCS) and axonal neuropathies

follow course of disease

63
Q

Needle EMG differentiates

A

myopathic from neuropathic

64
Q

How does the pattern of motor units at rest and with exertion allow for distinction between neuropathic disorders and myopathic?

A

neuropathic (anterior horn cell and peripheral nerve) results in loss of whole motor units while
myopathic disorders produce drop out fibers within a motor unit

65
Q

classification by anatomical localization

anterior horn

A

ALS
Spinal muscular atrophy
Poliomyelitis and WN virus

66
Q

classifiation by anatomical localization

radiculopathies and plexopathies

A

cervical and lumbosacral nerve root compression

brachial and lumbosacral plexopathies

67
Q

classification by anatomical localization

peripheral neuropathies

A

mononeuropathies
mononeuropathy multiplex
polyneuropathy

68
Q

mononeuropathies (3)

A

carpal tunnel - entrapment of median nerve

ulnar nerve palsy - entrapment at elbow

peroneal palsy - entrapment at the head of the fibula

69
Q 
polyneuropathies
hereditary 
systemic 
vitamin deficient
exogenous toxins
A

hereditary - CMT
Systemic - diabetes / immune
Vitamin - B12 deficiency
exogenous - alcohol / chemo

70
Q 
myasthenia gravis
lambert eaton
botulism 
organophosphate poisoning 
all are examples of ???
A

NMJ disorders

71
Q

myopathies

muscular dystrophies

A

Duchenne / Becker
limb girdle
FSH

72
Q

Myotonic disorders -

A

myotonia congenital

myotonic dystrophy

73
Q

polymyositis
dermatomyositis
inclusio body myositis
all are?

A

inflammatory myopathies

74
Q

glycogen storage
lipid myopathy
mitochondrial myopathy
all are?

A

metabolic myopathies

75
Q

what is a channelopathy myopathy example?

A

periodic paralysis

76
Q

ALS

A

characterized by progressive weakness and wasting resulting from degeneration of brainstem and spinal cord LMNs
Coexisting spasticity and hyperreflexia caused by degeneration of UMNs

77
Q

ALS

etiology?

A

most sporadic

5-10% familal

78
Q

ALS initial symptoms

A

asymmetric limb weakness

fasiculuations

79
Q

ALS sensory exam?

A

normal

80
Q

degeneration of brainstem and spinal cord LMNs?

A

ALS

81
Q

ALS rate of spread

what is often spared?

A

unpredictable

extraocular and facial muscles are often spared

82
Q

CMT with slow nerve conduction velocity and pathological evidence of a hypertrophic demyelinating neuropathy

A

CMT type 1

83
Q

CMT with relatively normal nerve conduction velocity and axonal degeneration

A

CMT type 2

84
Q

genetics of CMT 1 and 2

A

Autosomal dominant

85
Q

are CMT disorders motor or sensory

A

motor and sensory neuropathies

86
Q

What causes CMT1A

A

Duplication of the DNA containing the peripheral myelin protein gene PMP22

87
Q

What is caused by a deletion of PMP22

A

Hereditary neuropathy with liability to pressure palsy HNPP

88
Q

Most common CMT phenotype

A

onset of walking is normal

distal hand and feet weakness and sensory loss develops slowly in the first two decades of life

89
Q

Second most common CMT phenotype

A

Impaired as infants and experience delayed walking - confined to WC later in life

90
Q

Third CMT phenotype

A

adult onset may not appear until age 40

91
Q

Treatment of CMT

A

Currently no meds reverse

Treat symptoms and improve QOL

92
Q

What % of diabetics have DN?

A

50-60

93
Q

What is the most common cause of neuropathy in the western world

A

diabetes

94
Q

what kind of neuropathy is found in diabetes

A

all types

95
Q

what is the most common DN

A

Sensorimotor polyneuropathy

96
Q

Sensorimotor polyneuropathy presenting symptoms in DN

A

Patients intially complain of numbess and buring dysesthsias in tehir feet which over time spreads up the legs and eventually into the hands
Weakness of dorsiflexor and slapping foot drop gait
Pin sensation loss in a stocking glvoe disbtribution / loss of position, vibration, and light touch as well as decreased reflexes is prominent in large fiber patterns

97
Q

NCV in SMPN in DN

A

often near normal if only small fibers involved

98
Q

does autonomic dysfunction occur in DN

A

It can with or without evidence for a neuropathy - postural hypotension, diarrhea, impotence, urinary retention and increased sweating are typical

99
Q

DN

what characterizes lumbosacral plexopathy

A

acute onset of asymmetrical proximal weakness and pain of the legs

100
Q

DN mononeuropathies

which nerves?

A

can affect almost any

101
Q

Examples given of disorder of the NMJ

A

MG

102
Q

What is MG?

A

autoimmune disorder with a post-junctional defect of the ACHR

103
Q

Thymic involvement of MG

A

85% of patients of thymic enlargemnet and 10% have a thymoma

104
Q

MG

Sex?

A

more common in women in earlier decades and men later in life

105
Q

Characteristic presentation of MG

A

Fluctuating weakness and fatigue in cranial, limb, or trunk musculature
ocular symptoms of ptosis / diplopia / and blurred vision are present in 50% initially and eventually 90%

106
Q

MG

cabeza?

A

facial muscles are weak and speech may become slurred, nasal and hoarse as the patient continues to speak - progressive trouble chewing and swallowing can lead to choking

107
Q

MG

breathing

A

weakness of respiratory muscles can cause SOB

108
Q

MG repetitive nerve stimulation

A

may produce characteristic decremental response in the train of muscle twitches

109
Q

MG intravenous injection of edrophonium

A

temproraily impoves strength bc it is a ACHterase inhibito

110
Q

MG treatmetn

A

pyridostimine - oral cholinesterase inhibitor

prednisone

plasma IV exchange

Thymectomy

111
Q

DMD and BMD

Genetics

A

X linked dystrophin gene

112
Q 
clumbsy waddling gait from the time they first walk
protuberant abdomen 
lumbar lordosis 
pseudohypertrophy of calves 
toe walking 
Gower's maneuver
common subnormal intelligence
A

DMD

113
Q

What is unaffected in DMD

A

Swallowing and eye movemnets and sensations

114
Q

DMD COD

A

The combination fo weak respiratory muscles and kyphoscoliosis drastically reduces pulmonary reserve so that they succumb from respiratory and hear failure by their late teens or 20s

115
Q

what aids in dx of DMD

A

markedly elevated serum CK

116
Q

DMD

muscle biopsy and EMG

A

Characteristic myopathic features

1111 NEUROBIOLOGY (6 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions