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Clin Med - NEURO > disease of peripheral nerves - waldron > Flashcards

disease of peripheral nerves - waldron Flashcards

1
Q

what is peripheral neuropathy

A

refers to many conditions that involve damage to the peripheral nervous system
symptoms depend on the type of nerve fibers affected and the type and severity of damage

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2
Q

how is nerve signaling in neuropathy disrupted

A

loss of signals normally sent (broken wiring)
inappropriate signaling when there shouldn’t be any (like static)
error that distort the messages being sent (like a wavy television picture)

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3
Q

what is motor nerve damage

A

usually associated with muscle weakness - other symptoms include painful cramps, fasciculations, muscle atrophy

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4
Q

what is sensory nerve damage

A

various symptom patterns
loss of vibratory sense, stocking glove sensation, loss of reflexes, loss of position sense, loss of temperature sensation, pain

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5
Q

what is autonomic nerve damage

A

aka small-fiber neuropathies
common symptoms: excess sweating, heat intolerance, inability to expand and contract small blood vessels regulating blood pressure, gastrointestinal symptoms

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6
Q

what is a mononeuropathy

A

only one nerve affected

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7
Q

what is it called when several discrete nerves are effected

A

multiple mononeuropathy or mononeuritis multiplex

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8
Q

what is polyneuropathy

A

multiple nerves diffusely affected

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9
Q

what is a plexus effect called

A

plexopathy

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10
Q

what is a radiculopathy

A

when a nerve root is affected

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11
Q

what are symptomatic acquires causes of peripheral neuropathy

A

Physical injury (trauma)
Diabetes
vascular and blood problems
systemic autoimmune diseases
triggered autoimmune
kidney and liver disorders
nutritional/vitamin imbalances, alcoholism, exposure to toxins
cancer/benign tumors
chemotherapy/radiation
infections

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12
Q

what is the most common cause of single nerve injury

A

physical injury (trauma)

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13
Q

what is the leading cause of polyneuropathy in the US

A

diabetes - 60-70% of patients

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14
Q

what is a genetic cause of peripheral neuropathy

A

Charcot-Marie- Tooth - rare but one of the most common INHERITED neurological disorder
no cure - supportive - permanent damage to nerves

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15
Q

what is the most common INHERITED neurological disorder

A

Charcot-Marie-Tooth

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16
Q

how is peripheral neuropathy evaluation

A

medical history
physical with full neurological exam
body fluid tests: blood work/CSF
genetic tests

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17
Q

what are important history events for peripheral neuropathy examination

A

work, travel, toxin exposure, ETOH, history if infection

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18
Q

what are unilateral defects typically caused by

A

mononeuropathies or plexopathies such as focal disorders

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19
Q

what are physiologic tests of nerve functions

A

nerve conduction velocity (NCV) and EMG

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20
Q

what is NCV assessing

A

nerve conduction velocity - signal strength and speed

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21
Q

what is an EMG assessing

A

electrical activity rest/contraction

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22
Q

what are neuropathology tests of Nerve appearance

A

Neurodiagnostic skin biopsy and nerve biopsy

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23
Q

what is the gold standard for diagnosing small fiber neuropathies

A

Neurodiagnostic skin biopsy

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24
Q

what is Autonomic testing for peripheral Neuropathy

A

QSART (small fiber neuropathies) - sweat ability, ACH

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25
Q

what are radiology imaging tests used for peripheral neuropathies

A

MRI, CT and muscle and nerve ultrasound

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26
Q

what is MRI looking for when evaluating for peripheral neuropathies

A

looking for compression/mass/soft tissue structural

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27
Q

What is a CT looking for when evaluating for peripheral neuropathies

A

looking for mass/solid tissue structural I.e. spinal stenosis

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28
Q

how is peripheral neuropathies managed?

A

Addressing neuropathies cause
specific symptoms can usually be improved
TENS
medications
surgery

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29
Q

How are you able to address the cause of neuropathy

A

glucose control, smoking cessation, diet, exercise
plasmapheresis, immunoglobulins for inflammatory/autoimmune source

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30
Q

how are specific symptoms managed for peripheral neuropathies

A

motor -orthotic, mechanical aids
autonomic - acupuncture, massage, CBT/psychotherapy, herbal remedies
Sensory - behavioral strategies

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31
Q

what is the best treatment for peripheral neuropathy

A

the best treatment is prevention - unnecessary procedures should be avoided

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32
Q

what is the prognosis of peripheral neuropathy

A

long term prognosis and complications vary; depending on underling cause, its control and which nerves have been damaged
some symptoms improve with time
some may have permanent damage or may have symptoms progressively worsen with time
rarely fatal

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33
Q

What is CRPS

A

complex regional pain syndrome
chronic >6 months) pain condition that most often affects one limb usually after an injury

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34
Q

how is CRPS characterized

A

by prolonged or excessive pain and changes in skin color, temperature, and/or swelling in affected area

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35
Q

what are the types of CRPS

A

CRSP-1
CRSP - 2

36
Q

what is CRPS-1

A

WITHOUT confirmed nerve injury (previously known as reflex sympathetic dystrophy syndrome)

37
Q

what is CRPS-2

A

with an associated, CONFIRMED nerve injury (previously known as causalgia)

38
Q

what is the epidemiology of CRPS

A

W>M
any age with peak at 40yo
(rarely in elderly, very few children <10yo, almost no children <5yo)

39
Q

what population is CRPS more common in

A

patients with other inflammatory and autoimmune conditions such as asthma

40
Q

what is the pathophysiology of CRPS

A

> 90% triggered by clear history of trauma or injury
vascular effects
immune system effects
may be influenced by genetics

41
Q

what are the most common triggers of CRPS

A

fractures/sprains/strains/limb immobilization
soft tissue injuries
surgery/minor medical procedures

42
Q

what is the immune systems effects causing CRPS

A

high levels of cytokines=redness, swelling and warmth

43
Q

what vascular effects cause CRPS

A

possible ischemia of deeper tissues causing muscle and pain/damage

44
Q

what are the typical ‘insults’ leading to CRPS

A

infection
blood vessel inflammation
entrapment of nerve causing internal injury

45
Q

what is the key symptom of CRPS

A

prolonged sever pain, may be constant
often described as “burning”, “pins and needles”, or “squeezing” sensation of affected limb
pain may spread to entire arm or leg, even though injury might have only involved a finger/toe
often increased sensitivity in affected area

46
Q

what are possible presentation of CRPS

A

may experience changes in skin temperature, skin color or swelling of affected limb
may feel warmer/cooler compared to opposite limb
may change color, becoming blotchy, blue, purple, pale or red

47
Q

what is the evaluation of CRPS

A

no specific test that can confirm CRPS
clinical diagnosis
distinguishing feature of CRPS is that of an injury to affected area
MRI or triple-phase bone scan may help confirm diagnosis

48
Q

what is CRPS often associated with that can be seen on testing

A

excess bone resorption but may be observed in other illnesses as well

49
Q

what is the treatment of CRPS

A

Rehabilitation and PT
psychotherapy
sympathetic nerve block
surgical sympathectomy
intrathecal drug pumps
spinal cord stimulation
neural stimulation (transcranial magnetic stimulation, or rTMS)
Medications

50
Q

what medications are often used in the treatment of CRPS

A

bisphosphonates
NSAIDs
Corticosteroids
neuropathic pain meds
botox
opioids
NMDA receptor antagonists
topical anesthetics

51
Q

what is the prognosis of CRPS

A

outcome is highly variable
younger adults, children and teens have better outcomes
some patients experience severe pain and disability despite treatment

52
Q

what is vitamin B 12

A

a water soluble vitamin important in cellular metabolism and maintenance of the integrity of the nervous system
important in synthesis of DNA and cell division

53
Q

what is deficiency in Vitamin B12 associated with

A

impaired erythropoiesis and nervous system demyelination, which account for most of its clinical manifestations

54
Q

what is the most common etiology of vitamin B12 deficiency

A

lack of intrinsic factor (pernicious anemia), a common finding in the elderly

55
Q

what are other etiologies of B12 deficiency/neuropathy

A

autoimmune
malabsorption
dietary insufficiency

56
Q

what is the epidemiology of B12 Neuropathy

A

common in elderly regardless of cause
patients with anemia
patient with macrocytosis
pernicious anemia
overall incidence is rare

57
Q

what is the pathophysiology of B12 neuropathy

A

dietary B12 binds to R-factor protein in saliva
in small intestines - b12 cleared from R-factor by pancreatic enzymes
b12 then binds to intrinsic factors secreted by gastric parietal cells
complex binds to receptor in ileum which allows for absorption
once absorbed b12 integrated into neurologic and hematologic pathways

58
Q

what is seen on the H&P for B12 neuropathy

A

pallor, fatigue - consistent with microcytic anemia
dementia, mental status, peripheral neuropathy, ataxia/loss of proprioception on neuro exam

59
Q

what bloodwork is run with vitamin B12 neuropathies

A

CBC with peripheral smear
serum B12 folate
MMA and Hemoysteine

60
Q

if the GI or dietary source is negative on evaluation of B12 neuropathy what needs to be considered

A

Autoimmune source - blood work for anti-intrinsic facrot antibodies

61
Q

if there is an autoimmune source with B12 neuropathy what will be present

A

pernicious anemia

62
Q

what is the management of B12 neuropathy

A

replace missing B12 pending etiology: duration and route vary

63
Q

what patient populations require routine monitoring with B12 supplimentation

A

Crohn’s disease and Celiac Disease

64
Q

what are complications of B12 neuropathy if undiagnosed

A

heart failure
severe disabling neurological deficits
increased risk for gastric cancer
increased risk of autoimmune disease

65
Q

what is Guillain-Barre syndrome

A

rare neurological disorder in which the body’s immune system mistakenly attacks PNS
severity can range from very mild with brief weakness to nearly devastating paralysis

66
Q

what is the epidemiology of Guillain-Barre syndrome

A

M=F
strike at any age (more frequent in adults and older people)
can be life-threatening - potentially interfering with breathing, blood pressure and/or heart rate

67
Q

what is the pathophysiology of Gillain-Barre Syndrome

A

exact cause unknown
most cases usually start a few days or weeks following a respiratory or gastrointestinal viral infection
occasionally surgery is a trigger

68
Q

what is the Molecular mimicry/innocent bystander therory

A

mechanism theories of guillian-barre syndrome
when preceded by viral or bacterial infection, infecting agent has changed chemical structure of some nerve and the nerves are now identified as foreign bodies and mistakenly attacked

virus itself makes immune system itself less discriminating and no longer able to recognize its own nerves.

69
Q

what is the campylobacter theory

A

mechanism theory of guillain-barre syndrome
antibodies to fight Campylobacter jejuni infection attack axon in motor nerves, causing acute motor axonal neuropathy

some campylobacter molecules resemble molecules in patients nerve axons, so their antibodies also attack the look-alike axons

70
Q

what is the presentation of Guillain-Barre syndrome presentation

A

symmetric weakness is the major symptom that prompts most patients to seek medical attention and is most characteristic of the syndrome
weakness may first appear as difficulty climbing the stairs or with walking - symptoms often affect arms, breathing muscles, or even the fact.

71
Q

where do symptoms typically start

A

the upper body and move down to the legs

72
Q

what is the progression of symptoms

A

they can increase in intensity over a period of hours, days, or weeks until certain muscles cannot be used at all
when sever, the person is almost totally paralyzed

73
Q

what are key diagnostic findings for Guillain-Barre syndrome

A

recent onset (days to at most 4 weeks of symmetric weakness)
abnormal sensation
absent or diminished deep tendon reflex
recent vital infections or diarrhea
elevated CSF protein without elevated cell count
abnormal nerve conduction velocity findings

74
Q

what are key points to presentation of Guillain-Barre syndrome

A

Symmetry and speed

75
Q

what are the types of Guillain-Barre syndrome

A

Acute inflammatory demyelinating polyneuropathy (AIDP)
acute motor axonal neuropathy (AMAN)/ Acute motor-sensory axonal neuropathy (AMSAN)
Miller-fisher syndrome
chronic inflammatory demyelinating polyneuropathy (CIDP)
Multifocal motor neuropathy

76
Q

what is acute inflammatory demyelinating polyneuropathy (AIDP)

A

most common type seen in the US
immune response damages the myelin coating and interferes with the transmission of nerve signals

77
Q

what is AMAN/AMSAN

A

Acute motor axonal neuropathy/acute motor-sensory axonal neuropathy
axon themselves are damaged by the immune response

78
Q

what is Miller-Fisher syndrome

A

rare, acquired nerve disease; most have a unique antibody
characterized by abnormal muscle coordination with poor balance and clumsy walking, weakness or paralysis of the eye muscles and absence of tendon reflexes. symptoms may follow viral illness
additional symptoms - generalized muscle weakness and respiratory failure

79
Q

what is CIDP

A

chronic inflammatory demyelinating polyneuropathy
weakness that can recur, repeatedly, over the course of years

80
Q

what is multifocal motor neuropathy

A

affects many different muscles in a small part of a limb/s
usually the symptoms are more severe on one side of the body

81
Q

what is the acute treatment of Guillain-Barre syndrome

A

plamsa exchange
High-dose immunoglobulin therapy (IVIg)

Both are equally effective if started within two weeks of onset of symptoms

82
Q

what are other acute treatments of Guillain-Barre

A

corticosteroids
supportive care
close monitoring - breathing, cardiac, swallowing

83
Q

what are the rehabilitation treatments for guillain-barre syndrome

A

PT - strength may not return uniformly
OT
Vocational therapy - evaluate need for implement assistive devices and other adaptive equipment and technology

84
Q

when assessing for GBS what is important to ask about

A

any recent viral infection or diarrhea

85
Q

why are patients with GBS admitted to the ICU

A

with concerns for respiratory failure

Clin Med - NEURO (13 decks)

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