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Clinical Med - Neurology > Neuropathies / Demyelinating Disorders > Flashcards

Neuropathies / Demyelinating Disorders Flashcards

1
Q

Is Guillain Barre Syndrome (GBS) is an immune-mediate, demyelinating polyneuropathy, of the peripheral or central nervous system?

A

Peripheral Nervous System

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2
Q

What TWO symptoms/exam findings characterize GBS?

A

Muscle Weakness

Areflexia

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3
Q

Does GBS present as ascending or descending paralysis?

Where does it typically start?

A

Ascending paralysis

Typically starts in the hands/feet and spreads to the trunk

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4
Q

Which variant of GBS presents with flaccid paralysis?

What organism is associated with this condition?

What anti-bodies are present in this condition?

A

Acute Motor Axonal Neuropathy (AMAN)

C. jejuni

IgG Anti-GM
IgG anti-GD

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5
Q

Which variant of GBS presents with ascending paralysis and motor weakness?

A

Acute inflammatory demyelinating polyneuropathy (AIDP)

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6
Q

Ventilation is typically required in which variant of GBS?

A

Acute motor sensory axonal neuropathy (AMSAN)

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7
Q

The GBS variant, Miller-Fisher Syndrome, is associated with what symptoms?

What antibody is present?

A

Ataxia
Areflexia
Ophthalmoplegia

IgG Anti-GQ

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8
Q

Which GBS variant is the most common?

A

Acute inflammatory demyelinating polyneuropathy (AIDP)

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9
Q

AIDP is an auto-immune response against cells responsible for myelination.

What is the name of these cells?

A

Schwann Cells

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10
Q

AMAN attacks what portion of the nerve?

A

Nodes of Ranvier

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11
Q

T/F: Miller-Fischer Syndrome presents as descending paralysis

A

True

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12
Q

Which variant of GBS often is associated with a Babinski’s Sign?

A

Bickerstaff’s Brainstem Encephalitis

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13
Q

In GBS immune responses are directed against what TWO part of a nerve?

A

Myelin

Axon

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14
Q

Other than C. jejuni, what TWO infections were seen in GBs patients?

A

CMV

Epstein Barr

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15
Q

What cranial nerve may sometime be effected in GBS?

A

CN VII

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16
Q

What is typically the first symptom/presentation of GBS?

A

“Rubbery Legs”

Progressing to ascending paralysis

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17
Q

T/F: Immunizations can trigger GBS

A

True

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18
Q

Other than immunizations, what can trigger GBS?

A

Trauma
Surgery
Bone marrow transplant

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19
Q

What CA is associated with GBS?

A

Hodgkin Lymphoma

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20
Q

Are eye movement abnormalities common in GBS?

A

No

Only in Miller Fisher Syndrome

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21
Q

Will GBS patients often complain of pain?

A

Yes

Described as a dull aching pain in weakened muscles

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22
Q

How is GBS diagnosed initially?

A

Clinically

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23
Q

What might a LP show in a GBS patient?

A

Elevated protein

Normal WBCs

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24
Q

What study is useful in detecting active axonal damage in GBS?

A

Electromyography (EMG)

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25
Q

Abnormal conduction on EMG would confirm a diagnosis of ____________ polyneuropathy.

A

Demyelinating

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26
Q

What are the TWO mainstays for GBS treatment?

A 
Plasma Exchange (Plasmaphoresis) 
IV IgG Therapy
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27
Q

What medications can be used to treat neuropathic pain in GBS?

A

Gabapentin
NSAIDs
Narcotics

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28
Q

T/F: Most GBS patients will not require some form of rehab

A

False

Many will

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29
Q

What is the most common disorder of neuromuscular transmission?

A

Myasthenia Gravis

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30
Q

T/F: Myasthenia Gravis is not an autoimmune disorder

A

False

It is

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31
Q

What are TWO characteristics of Myasthenia Gravis?

A

Distal-to-Proximal weakness

Fatigability of muscles

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32
Q

What Sx of the EYE may a myasthenia gravis patient have?

A

Ptosis

Diplopia

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33
Q

What FACIAL manifestation may a myasthenia gravis have?

A

Weak Chewing

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34
Q

Would a Myasthenia Gravis patient’s weakness be worse in the morning or throughout the day?

A

Throughout the day - Symptoms typically worsening with daily actives or exercise

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35
Q

What is the most serious Sx of Myasthenia Gravis?

A

Respiratory Distress

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36
Q

T/F: Thyroid disorders are found in 10% of Myasthenia Gravis patients

A

True

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37
Q

What antibody is found in 40% of MG patients who do not test positive for acetylcholine receptor antibodies?

A

Muscle-Specific Kinase (MuSk)

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38
Q

If a MG patient does not test positive for AchR or MuSK antibodies then they are considered what?

A

Seronegative MG

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39
Q

What is the name of the bedside test used to diagnosis Myasthenia Gravis?

A

Tensilon Test

Give Edrophonium
If symptoms improve = MG

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40
Q

Why is a CT or MRI ordered in MG evaluation?

A

15% of MG patients have a thymoma

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41
Q

What medications are used EARLY in MG?

A

Anticholinesterase:

Pyriogostigmine
Neostigmine
Ambenonium

This is because there are still acetylcholine receptors left

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42
Q

Other than anticholinesterase agents…..

What are additional pharmacological MG treatment options?

A
  1. Glucocorticoids
  2. Immunosuppression (Cyclosporine)
  3. Plasmaphoresis, IV IgG
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43
Q

What surgery may be preformed to treat MG?

A

Thymectomy

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44
Q

Does multiple sclerosis effect the peripheral or central nervous system?

A

Central Nervous System

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45
Q

Does MS more commonly effect men or women?

A

Women (Onsets around 30 y.o.)

46
Q

What are the FOUR major pathological mechanisms of MS?

A
  1. Inflammation
  2. BBB Disruption
  3. Demyelination
  4. Axon Degeneration
47
Q

MS involves a loss of what cells in the CNS responsible for myelination of axons?

A

Oligodendrocytes

48
Q

Are MS plaques more common in the white or grey matter of the brain?

A

White Matter

49
Q

Can MS plaques be found on the spinal cord?

A

Yes

50
Q

Patients complaining of an electric shock down their spine with neck flexion wold be concerning for MS.

What is the name of the sign described above?

A

Lhermitte’s Sign

51
Q

What cranial nerve is often involved in MS?

What disease of this nerve may be present?

A

CN II

Unilateral Optic Neuritis

52
Q

Are sensory or motor symptoms more common in MS?

Does this affect the ascending or descending pathways in the spinal cord?

A

Ascending

53
Q

T/F: Urinary urgency is a common complaint in MS

A

True

54
Q

A 35 yo femal presents to your clinic complaining of diplopia and trouble seeing as well as she used to, feeling like she can’t walk well, and decreaesed sensation in her right arm that has gradually onset over the last few months.

On examination, he is found to be hyperreflexic, has horizontal nystagmus, and impaired sensation in her right arm.

What is you most concerning diagnosis?

What is the imaging study of choice in this patient and what might it show to confirm your Dx?

A

Multiple Sclerosis

MRI of the Brain: Irregular, Spotty Plaque formation (“Dawson’s Fingers”)

55
Q

T/F: Almost 90% of MS patients are characterized as Progressive relapsing

A

False

90% of MS are characterized as Relapsing-Remitting

56
Q

How are MS exacerbations treated?

A

High dose corticosteroids (Methylprednisone)

Plasmaphoresis may also be useful

57
Q

What are examples of disease-modifying treatments of MS?

A 
Beta-Interferons
Glatiramar Acetate
Methotrexate
Mitoxantrone
Cyclophosphamide
58
Q

Does Amyotrophic Lateral Sclerosis (ALS) cause reversible or irreversible nerve damage?

A

Irreversible

59
Q

Is ALS characterized by upper or lower motor neuron degeneration and death?

A

Upper Motor Neuron

Lower motor neuron deficits will develop late in the disease

60
Q

Which spinal tract does ALS effect?

A

Corticospinal tract

61
Q

What may you expect an ALS patient’s muscles to look like?

A

Atrophic

62
Q

What is the pharmacological option for ALS treatment?

A

Riluzole

63
Q

Typically ALS management involves treating the symptoms…..

What may need to be place if a patient develops severe dysphonia?

What may need to happen is a patient develops respiratory distress of compromised?

A

Dysphonia: PEG Tube

Respiratory: Tracheostomy, Ventilation

64
Q

Are axonal or demyelinating neuropathies more common?

A

Axonal

65
Q

How do mononeuropathies differ from polyneuropathies?

A

Mononeuropathies only effect a single nerve while polyneuropathies effect multiple nerves

66
Q

What is more common acute, subacute, or chronic polyneuropathies?

A

Chronic Polyneuropathies (ie: Diabetic Neuropathy)

67
Q

T/F: Bell’s Palsy is a polyneuropathy

A

False

It is a mononeuropathy

68
Q

_______ is defined as increased pain from a stimulus that normally provokes pain

A

Hyperalgesia

69
Q

________ is defined as diminished pain in response to a normally painful stimulus

A

Hypoalgesia

70
Q

________ is defined as pain due to a stimulus that does not normally provoke pain

A

Allodynia

71
Q

Neuronal degredation involves what part of the nerve?

Wallerian Degredation?

Segmental demyelination?

A

Neuronal: Nerve Bodies

Wallerian: Axon below the cell body

Segmental: Myelin Sheath

72
Q

What is the most common hereditary neuropathy?

A

Charcot-Marie-Tooth Disease

73
Q

What co-morbid condition is often closely associated with chronic axonal polyneuropathies?

A

Diabetes

74
Q

T/F: Lyme Disease can result in axonal polyneuropathies

A

True

75
Q

What percent of polyneuropathies are idiopathic?

A

25%

76
Q

What is the “characteristic” presentation of polyneuropathies?

Of these symptoms is sensory or motor loss more common?

A
  1. Symmetric distal sensory loss
  2. Burning Sensation
  3. Pins and Needles
  4. Weakness

Sensory loss is most common

77
Q

Are the longer or shorter axons effect first in chronic axonal polyneuropathies?

A

Longer Axons

78
Q

What is the ‘classic’ distribution of sensory loss in chronic axonal polyneuropathy?

A

“Stocking and glove”

Hands and feet

79
Q

What is a unique chronic complication involving the foot in chronic axonal neuropathies, particularly in diabetic patients.

These result due to micro fractures in the foot that don’t heal well.

A

Charcot Arthropathy

“Rocker-Bottom Foot”

80
Q

Patients who present with a sudden onset of rapid sensory loss with or without pain after exposure to toxic chemicals would be considered to have a(n) __________ (acute/chronic) polyneuropathy.

A

Acute Polyneuropathy

81
Q

Would you expect a patient with polyneuropathy to be hyperreflexic or hyporeflexic?

A

Hyporeflexic

82
Q

How may you hear a patient’s legs described in more severe polyneuropathy resulting in muscle wasting?

A

“Inverted Champagne Bottle”

“Stork Leg Deformity”

83
Q

What is the best diagnostic and initial study when evaluating polyneuropathy?

A

Electromyography (EMG)

84
Q

What happens when you get a bladder infection?

A

…..Urine trouble

85
Q

Would axonal loss lead to higher or lower magnitudes on nerve conduction studies?

A

Lower Amplitude

86
Q

Would demyelination show an increase or decrease in conduction velocity?

A

Decrease in conduction velocity

87
Q

Nerve biopsy can be used when polyneuropathy diagnosis remains unclear, although it can be low yield….

What is the preferred nerve and site for this?

A

Sural Nerve (ankle)

88
Q

T/F: There are curative treatments for axonal polyneuropathy

A

False

There are no curative treatments, often you just treat the underling disease

89
Q

What medication is often used to treat pain in neuropathies?

A

Gabapentin

90
Q

Which area of the body do patients with diabetic neuropathies need to pay special attention to?

A

Their Feet

91
Q

What neuralgia is characterized by brief episodes of unilateral shock like pains, that can follow a cranial nerve distribution?

A

Trigeminal Neuralgia

92
Q

Trigeminal neuralgia is commonly due to idopathic neurovascular compression…..

However, it can also be associated with what virus?

A

Herpes Zoster

93
Q

T/F: Pain in trigeminal neuralgia can typically onset with light touch stimulation

A

True

94
Q

What are common triggers of trigeminal neuralgia?

A 
Chewing
Talking
Brushing Teeth 
Cold Air
Grimacing
95
Q

Although trigeminal neuralgia is mostly diagnosed clinically…..

When would neuroimaging be warranted?

A

If the patient had obvious causes of pain

Ex: Herpes Zoster, Trauma

96
Q

What medication is first line for trigeminal neuralgia treatment?

A

Carbamazepine

97
Q

What surgical procedures are available to treat trigeminal neuralgia?

A

Microvascular Decompression

Ablation

98
Q

Bell’s Palsy commonly affects what cranial nerve?

A

CN VII

99
Q

Which virus is most commonly associated with Bell’s Palsy?

A

Herpes Zoster

100
Q

What are classic presentations in Bell’s Palsy?

A 
Facial Droop
Unable to raise one eye brow 
Excessive Tearing
Drooling
Unable to close one eye
Lack of nasal labial fold
Nasal Flaring
101
Q

What is the ‘mainstay’ of Bell’s Palsy treatment?

What can be added to this pharmacotherapy?

A

Prednisone

Antivirals (Acyclovir)

102
Q

What ‘pain syndrome’ is described as pain that spreads beyond an area or trauma, injury, stroke, or MI?

A

Chronic Regional Pain Syndrome

103
Q

Which is typically worse, the initial injury/trauma or the CRPS that follows?

A

The CRPS that follows

104
Q

Is Type 1 or Type 2 CRPS more common?

A

Type 1

105
Q

What fractures are commonly associated with CRPS, particularly in the hand?

A

Intra-articular radial-ulnar fractures

Ulnar styloid fractures

106
Q

Is the upper extremity or the lower extremity more commonly effected by CRPS?

A

Upper Extremity

107
Q

What are the THREE stages of CRPS?

A
  1. Burning Pain, Temperature sensory loss, and local edema
  2. Worsing edema, atrophy, skin thickening
  3. Worsening to limited ROM
108
Q

T/F: Patients with CRPS often have allodynia

A

True

109
Q

What bone disorder is often found in patients with CRPS?

A

Osteoporosis

110
Q

CRPS is a diagnosis of _______.

A

Exclusion

111
Q

What is the most beneficial step in treatment of CRPS?

A

Early Mobilization

112
Q

What medication can be given to CRPS patients to prevent osteoporosis?

A

Vitamin C

Clinical Med - Neurology (7 decks)

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