Peripheral neuropathies and ALS Flashcards

1
Q

mononeuropathy or focal involvement of a single nerve is often due to

A
  • trauma
  • compression
  • entrapment
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2
Q

Meralgia paresthetica

A
  • tingling, numbness and burning pain in your outer thigh
  • lateral femoral cutaneous nerve
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3
Q

define mononeuropathy multiplex

A
  • damage to one or more peripheral nerves
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4
Q

pattern of mononeuropathy multiplex early on is

A

asymmetric

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5
Q

polyneuropathy predominantly presents as what type of deficit

A
  • several peripheral nerves affected at the same time
  • presents as a distal and symmetric deficit
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6
Q

peripheral neuropathies are categorized into

A
  • axon (axonal or neuronal neuropathies)
  • myelin (demyelinating)
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7
Q

in axonal neuropathies, what is the target

A
  • axon
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8
Q

most polyneuropathies falls into what category

A
  • axonal neuropathies
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9
Q

what is the most common type of polyneuropathy

A
  • diabetic polyneuropathy
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10
Q

in axonal neuropathies, may see both motor and sensory deficits but which usually precedes the other

A
  • sensory usually preceeds motor symptoms
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11
Q

neuronal neuropathies affect what

A
  • nerve cell bodies in the anterior horn of the spinal cord or dorsal root ganglion
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12
Q

List two examples of neuronal neuropathies

A
  • type 2 charcot marie tooth hereditary neuropathy
  • vitamin B6 toxicity
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13
Q

demyelinating neuropathies involve what structure

A
  • myelin sheath surrounding the axon
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14
Q

demyelinating neuropathies are often caused by?

A
  • autoimmune or inherited
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15
Q

guillain barre syndrome is an example of

A

demyelinating neuropathies

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16
Q

guillain-barre syndrome is an idiopathic inflammatory neuropathy that is associated with what infection

A
  • campylobacter jejuni
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17
Q

clinical presentation

  • ascending weakness (symmetric), usually beginning in legs
  • sensory complaints frequent
  • DTRs frequently absent
  • may have autonomic dysfunction
A
  • guillain-barre syndrome
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18
Q

describe chronic inflammatory demyelinating polyneuropathy

A
  • idiopathic inflammatory neuropathy
  • slowly progressive relapsing polyneuropathy
  • diffuse hyporeflexia or areflexia
  • diffuse weakness
  • generalized sensory loss
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19
Q

List the three types of charcot-marie-tooth hereditary motor and sensory neuropathies and what type they are

A
  • CMT-1: demyelinating
  • CMT-2: neuronal
  • CMT-3: demyelination
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20
Q

clinical presentation

  • weakness
  • wasting of distal muscles in the limbs (with or without sensory loss)
  • pes cavus
  • reduced or absent DTR
A
  • charcot-marie-tooth
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21
Q

differentiate between acute, subacute, and chronic peripheral neuropathies

A
  • acute: up to 4 weeks
  • subacute: 4 - 8 weeks
  • chronic: > 8 weeks
22
Q

peripheral nerves are composed of what elements

A
  • sensory
  • motor
  • autonomic
23
Q

size of proprioception and vibratory sensory fibers

A

large

24
Q

size of pain and temperature fibers

A

small

25
Q

what reflexes are usually the first to be lost in patients with polyneuropathies

A
  • ankle reflexes
26
Q

autonomic symptoms associated with peripheral neuropathies

A
  • postural hypotension
  • tachycardia
  • cold extremities
  • impaired thermoregulatory sweating
  • bowel and bladder dysfunction
  • impotence
27
Q

In chronic axonal polyneuropathies, such as diabetes, injury tends to be related to

A
  • axon length
    • sx begin in the lower extremities
28
Q

in chronic axonal polyneuropathies, does sensory or motor sx come first

A
  • sensory precedes motor
29
Q

stocking and glove distribution of sensory loss is associated with

A
  • chronic axonal polyneuropathies
30
Q

in acute demyelinating polyneuropathies, such as guillain barre, what structures are primarily affected

A
  • motor nerve fibers
31
Q

what is the earliest sign in acute demyelinating polyneuropathies

A
  • weakness rather than sensory loss
    • distal muscles are predominantly affected
32
Q

in acute demyelinating polyneuropathies, large myelinated fibers are the most damaged. What should you see on physical exam

A
  • abnormalities of vibratory testing and proprioception - out of proportion to loss of pin prick or temperature sensation
33
Q

myopathies usually present with whereas neuropathies present with

A
  1. myopathies: proximal motor weakness
  2. neuropathies: distal motor weakness
34
Q

Regardless if a patient presents with motor weakness, if there is any sensory loss and/or loss of relfexes - think

A
  • neuropathy
35
Q

define radiculopathy

A
  • nerve root lesions
36
Q

how can radiculopathy be differentiated from peripheral neuropathies

A
  • in radiculopathy, nerve root involvement produces impaired cutaneous sensation in a segmental pattern
37
Q

clinical presentation

  • neuropathy is symmetrical
  • affects legs more than arms
  • begins with paresthesias and ataxia associated with loss of vibration and position sense
  • can progress to severe weakness, spasticitiy, clonus, paraplegia, and fecal/urinary incontinence
A
  • B-12 deficiency
38
Q

what condition typically produces symptoms of upper and/or lower motor neuron dysfunction without sensory symptoms

A
  • motor neuron disease
39
Q

nerve conduction study has what function

A
  • permits conduction velocity tp be measured in motor and sensory fibers
40
Q

electromyography (EMG) has what function

A
  • may reveal evidence of denervation in affected muscles
41
Q

what type of neuropathy typically reveals little or no evidence of denervation, but does show conduction block or marked slowing of maximal conduction velocity in affected nerves

A

demyelinating neuropathy

42
Q

what type of neuropathy typically reveals denervation changes but maximal nerve conduction velocity is normal or only slightly slowed

A
  • axonal neuropathies
43
Q

what medication should not be given in tx of guillan-barre syndrome

A

steroids

44
Q

medication options for neuropathies

A
  • gabapentin/pregabalin
  • tricyclic antidepressants
45
Q

what causes amyotrophic lateral sclerosis (ALS)

A
  • 90% sporadic
  • caused by degeneration of both upper and lower motor neurons
    • leads to skeletal muscle atrophy, weakness, disability, and eventually death
46
Q

clinical presentation

  • progressive symptoms consistent with upper and lower motor neuron dysfunction
  • present in one of four body segments (cranial, cervical, thoracic, and lumbosacral)
    • spread to other segments in time
A
  • amyotrophic lateral sclerosis (ALS)
47
Q

sensory exam in patients with amyotrophic lateral sclerosis (ALS) is usually

A
  • normal
48
Q

signs of lower motor neuron disease

A
  • weakness
  • atrophy
  • muscle cramps
  • hyporeflexia
  • fasciculations
49
Q

signs of upper motor neuron disease

A
  • increased tone
  • incoordination
  • slowness of movement
  • hyper-reflexia
    • babinski
  • spasticity
  • clonus
50
Q

how is amyotrophic lateral sclerosis (ALS) diagnosed

A
  • clinically
    • EMG often reveals evidence of diffuse, ongoing, chronic denervation
51
Q

LMN + UMN deficits in 3 regions is consistent with

A
  • definite ALS