Strohm content Flashcards

1
Q

Encephalopathy: [definition]

A

Encephalopathy: dysfunction of cerebrum

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2
Q

Myelopathy: [definition]

A

Myelopathy: dysfunction of spinal cord

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3
Q

Radiculopathy: [definition]

A

Radiculopathy: dysfunction of nerve root

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4
Q

Plexopathy: [defintion]

A

Plexopathy: dysfunction of plexus

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5
Q

Polyneuropathy: [defintion]

A

Polyneuropathy: dysfunction of more than one nerve

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6
Q

Mononeuropathy: [definition]

A

Mononeuropathy: dysfunction of one nerve
* Ex: median mononeuropathy = carpal tunnel syndrome

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7
Q

Myopathy: [definition]

A

Myopathy: dysfunction of muscle

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8
Q

Examples of bulbar weakness:

A

Bulbar weakness involves a bilateral LMN lesion of CN IX, X, XI, XII
* Dysarthria: speech poorly articulated
* Dysphagia: difficulty swallowing
* Dysphonia: voice weak

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9
Q

Biceps and brachioradialis reflex involves [spinal level]

A

Biceps and brachioradialis reflex involves C5, C6
* C5, C6 pick up sticks

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10
Q

Triceps reflex involves [spinal level]

A

Triceps reflex involves C6,C7
* C7 not 8 lay them straight

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11
Q

Cremasteric reflex involves [spinal levels]

A

Cremasteric reflex involves L1, L2

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12
Q

Patellar reflex involves [spinal levels]

A

Patellar reflex involves L2-L4
* L2-L4 kick the door

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13
Q

Achilles reflex involves [spinal levels]

A

Achilles reflex involves S1, S2
* S1, S2 buckle my shoe

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14
Q

Anal wink reflex involves [spinal levels]

A

Anal wink reflex involves S3, S4

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15
Q

Increased muscle stretch is detected by muscle spindle receptors via [type axons]

A

Increased muscle stretch is detected by muscle spindle receptors via type Ia and II sensory axons

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16
Q

Muscle spindle afferent fibers travel to the spinal cord via _ and synapse with _ and _

A

Muscle spindle afferent fibers travel to the spinal cord via DRG and synapse with alpha motor neuron and inhibitory interneuron

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17
Q

Increased muscle stretch will send signals to the spinal cord that result in simultaneous _ and _

A

Increased muscle stretch will send signals to the spinal cord that result in simultaneous inhibition of antagonist muscle (prevent overstretching) and activation of agonist muscle (contraction)

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18
Q

Increased muscle force is detected by golgi tendon organ via [type axons]

A

Increased muscle force is detected by golgi tendon organ via type Ib sensory axons

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19
Q

Explain the golgi tendon pathway:

A
  1. Type Ib fibers sense increased muscle tension
  2. Fiber travel to spinal cord via DRG
  3. Activation of inhibitory interneuron
  4. Inhibition of agonist muscle (to reduce tension)
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20
Q

_ is an immune-mediated or inflammatory PNS demyelinating disease

A

Guillain-Barre syndrome is an immune-mediated or inflammatory PNS demyelinating disease

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21
Q

_ is a hereditary PNS demyelinating disease

A

Charcot Marie Tooth disease is a hereditary PNS demyelinating disease

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22
Q

Most cases of Guillain-Barre are _ , meaning they are monophasic and acute; cases that are > 8 weeks are called _

A

Most cases of Guillain-Barre are acute inflammatory demyelinating polyneuropathy (AIDP) , meaning they are monophasic and acute; cases that are > 8 weeks are called CIDP

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23
Q

Miller-fisher syndrome is a type of Guillain-Barre in which we see _ deficits due to antibodies against _

A

Miller-fisher syndrome is a type of Guillain-Barre in which we see oculomotor deficits due to antibodies against GQ1B

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24
Q

The most common pattern of multiple sclerosis is _

A

The most common pattern of multiple sclerosis is relapsing remitting

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25
Q

_ is a common presentation of multiple sclerosis that involves painful unilateral vision loss and Marcus Gunn pupil

A

Acute optic neuritis is a common presentation of multiple sclerosis that involves painful unilateral vision loss and Marcus Gunn pupil

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26
Q

Pyramidal tract demyelination in M.S. may present as [symptoms]

A

Pyramidal tract demyelination in M.S. may present as weakness, spasticity

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27
Q

M.S. patients may experience spinal cord syndromes such as _

A

M.S. patients may experience spinal cord syndromes such as electric shock-like sensation along cervical spine on neck flexion
* Also neurogenic bladder, paraparesis, etc

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28
Q

M.S. symptoms may be aggrevated by _ temperatures

A

M.S. symptoms may be aggrevated by hot temperatures
* Ex: exercise, hot bath

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29
Q

The classic demographic for MS is _

A

The classic demographic for MS is 20-30 yo female

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30
Q

[Imaging] showing _ is diagnostic for MS

A

MRI showing multiple lesions, separated by time and space is diagnostic for MS

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31
Q
A

Multiple sclerosis

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32
Q
A

Osmotic demyelination syndrome

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33
Q
A

Progressive multifocal leukoencephalopathy (PML)

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34
Q

_ is a disorder of beta-oxidation due to a mutation in ABCD1 gene that causes buildup of VLCFA in the adrenal glands and white matter of the brain

A

Adrenoleukodystrophy is a disorder of beta-oxidation due to a mutation in ABCD1 gene that causes buildup of VLCFA in the adrenal glands and white matter of the brain
* X-linked recessive
* Progressive disease that leads to adrenal gland crisis, coma, death

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35
Q

Another name for osmotic demyelination syndrome is _

A

Another name for osmotic demyelination syndrome is central pontine myelinolysis

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36
Q

Osmotic demyelination syndrome is massive axonal demyelination in the _ secondary to rapid osmotic change

A

Osmotic demyelination syndrome is massive axonal demyelination in the pontine white matter secondary to rapid osmotic change

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37
Q

The most common cause of osmotic demyelination syndrome is _

A

The most common cause of osmotic demyelination syndrome is iatrogenic- correction of hyponatremia

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38
Q

Signs of osmotic demyelination syndrome include _

A

Signs of osmotic demyelination syndrome include acute paralysis, dysarthria, dysphagia, diplopia, loss of consciousness
* Can cause “locked in syndrome”

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39
Q

“Locked in syndrome” can be caused by _

A

“Locked in syndrome” can be caused by osmotic demyelination syndrome

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40
Q

Correcting sodium too fast:
“From high to low, _ “
“From low to high _ “

A

Correcting sodium too fast:
“From high to low, your brains will blow “ - cerebral edema/ herniation
“From low to high your pons will die “ - osmotic demyelination syndrome

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41
Q

_ is the most common subtype of Guillain-Barre syndrome

A

Acute inflammatory demyelinating polyradiculopathy is the most common subtype of Guillain-Barre syndrome

42
Q

AIDP is an autoimmune condition (subtype of Guillain-Barre) that destroys _

A

AIDP is an autoimmune condition (subtype of Guillain-Barre) that destroys Schwann cells
* Damage occurs via inflammation
* Demyelination of motor fibers, sensory fibers, peripheral nerves

43
Q

Guillain Barre does not have known definitive causal link to any pathogen though assocition with _ is most common

A

Guillain Barre does not have known definitive causal link to any pathogen though assocition with Campylobacter jejuni is most common
* Damage is mediated by inflammation
* Likely facilitated by molecular mimicry and triggered by stress

44
Q

Guillain-Barre results in [symmetric/ asymmetric] and [ascending/ descending] paralysis

A

Guillain-Barre results in symmetric and ascending paralysis

45
Q

Gullain-Barre will often present with _ DTR

A

Gullain-Barre will often present with depressed or absent DTR
* Starting in the lower extremities
* Can progress to facial paralysis and respiratory failure

46
Q

Guillain-Barre is associated with _ , an increase in CSF protein with normal cell count

A

Guillain-Barre is associated with albuminocytologic dissociation , an increase in CSF protein with normal cell count

47
Q

Guillain Barre is treated via:

A

Guillain Barre is treated via:
* Respiratory support
* Plasmapheresis or IVIG

48
Q

Acute disseminated encephalomyelitis is _

A

Acute disseminated encephalomyelitis is multifocal inflammation and demyelination after infection
* Can also occur after vaccination
* Presents with rapidly progressive multifocal neurologic symptoms, altered mental status

49
Q

Charcot-Marie-Tooth disease is also called _

A

Charcot-Marie-Tooth disease is also called hereditary motor and sensory neuropathy

50
Q

Charcot-Marie-Tooth is a “dysmyelinating” disease, meaning _

A

Charcot-Marie-Tooth is a “dysmyelinating” disease, meaning there is defective production of proteins involved in structure and function of peripheral nerves/ myelin sheath

51
Q

Charcot-Marie-Tooth disease has [inheritance pattern]

A

Charcot-Marie-Tooth disease has autosomal dominant inheritance

52
Q

Presenting symptoms of Charcot-Marie Tooth disease include:

A

Presenting symptoms of Charcot-Marie Tooth disease include:
* Foot deformities (pes cavus, hammer toe)
* Lower extremity weakness (foot drop)
* Sensory deficits

53
Q

The most common type of Charcot marie tooth, CMT1A is caused by [mutation]

A

The most common type of Charcot marie tooth, CMT1A is caused by PMP22 gene duplication

54
Q

Progressive multifocal leukoencephalopathy is a demylination disease of the (CNS/PNS)

A

Progressive multifocal leukoencephalopathy is a demylination disease of the CNS
* Involves destruction of oligodendrocytes

55
Q

PML occurs secondary to _

A

PML occurs secondary to reactivation of a latent JC virus infection
* Occurs primarily in AIDS patients
* Rapidly progressive, often fatal

56
Q

_ motor neuron produces muscle movement on contraction

A

Alpha motor neuron produces muscle movement on contraction

57
Q

_ motor neuron regulates the sensitivity to stretch and contributes to fine motor control

A

Gamma motor neuron regulates the sensitivity to stretch and contributes to fine motor control

58
Q

Name some chronic, axonal polyneuropathies

A

Chronic, axonal polyneuropathies:
* Diabetic neuropathy
* B12 deficiency
* Critical illness myopathy/neuropathy

59
Q

Arsenic and lead poisoning cause acute (axonal/demyelinating) polyneuropathy

A

Arsenic and lead poisoning cause acute axonal polyneuropathy

60
Q

Delayed (blocked) conduction velocity on nerve conduction study may be an indication of [type pathology]

A

Delayed (blocked) conduction velocity on nerve conduction study may be an indication of demyelinating neuropathy
* E.g. Guillain Barre

61
Q

Reduced signal amplitude on nerve conduction study may be an indication of underlying [type pathology]

A

Reduced signal amplitude on nerve conduction study may be an indication of underlying axonal neuropathy
* E.g diabetic neuropathy

62
Q

What disease is seen in navy?

A
63
Q

UMN lesion will cause _ bladder

A

UMN lesion will cause spastic bladder
* Urgency, incontinence, increased bladder tone
* E.g. stroke, MS, spinal cord lesion

64
Q

LMN lesion will cause _ bladder

A

LMN lesion will cause flaccid bladder
* Incontinence, retention, no anal reflex, decreased bladder tone
* E.g. cauda equina

65
Q

Spinal muscular atrophy is a congenital degeneration of _ that results in _

A

Spinal muscular atrophy is a congenital degeneration of anterior horns of spinal cord that results in LMN symptoms, hypotonia

66
Q

A “floppy baby” with marked hypotonia and tongue fasiculations is suggestive of _

A

A “floppy baby” with marked hypotonia and tongue fasiculations is suggestive of spinal muscular atrophy
* aka Werdnig-Hoffmann disease

67
Q

Werdnig-Hoffmann disease is a [inheritance pattern] mutation in _ gene

A

Werdnig-Hoffmann disease is an autosomal recessive mutation in SMN1
* Causes defective snRNP

68
Q

ALS is associated with _ bladder deficits

A

ALS is associated with no bladder deficits

69
Q

Amyotrophic lateral sclerosis involves _ type degeneration

A

Amyotrophic lateral sclerosis involves combined UMN and LMN degeneration
* UMN: corticobulbar/corticospinal
* LMN: medullary and spinal cord degeneration

70
Q

ALS can be caused by a defect in _

A

ALS can be caused by a defect in superoxide dismutase 1

71
Q

Complete occlusion of the anterior spinal artery spares the _ and _

A

Complete occlusion of the anterior spinal artery spares the dorsal column and Lissauer tract

72
Q

The _ regions of the anterior spinal artery is a watershed area

A

The mid-thoracic (T3-T8) regions of the anterior spinal artery is a watershed area
* Below T8 the artery of Adamkiewicz supplies ASA

73
Q

Complete occlusion of the ASA presents with _ deficit below the lesion

A

Complete occlusion of the ASA presents with UMN deficit below the lesion
* Corticospinal tract

74
Q

Complete occlusion of the ASA presents with _ deficit at the level of the lesion

A

Complete occlusion of the ASA presents with LMN deficit at the level of the lesion
* Ventral horn

75
Q

_ is an infectious cause of progressive sensory ataxia (impaired proprioception and coordination)

A

Tabes dorsalis is an infectious cause of progressive sensory ataxia (impaired proprioception and coordination)
* Associated with charcot joints, argyll robertson pupils, paroxysmal shooting pain

76
Q

In syringomyelia, the syrinx expands and damages the _ of the spinothalamic tract

A

In syringomyelia, the syrinx expands and damages the anterior white commissure of the spinothalamic tract

77
Q

Subacute combined degeneration involves demyelination of (3) tracts:

A

Subacute combined degeneration involves demyelination of (3) tracts: SCD
* Spinocerebellar tracts
* Lateral corticospinal tracts
* Dorsal columns

78
Q

Subacute combined degeneration will present with _ signs

A

Subacute combined degeneration will present with ataxic gait, parethesia, impaired position/vibration sense, UMN symptoms

79
Q

Cauda equina syndrome is associated with radicular pain, absent _ reflexes, loss of bladder and anal sphincter control, and _ anesthesia

A

Cauda equina syndrome is associated with radicular pain, absent knee and ankle reflexes, loss of bladder and anal sphincter control, and saddle anesthesia

80
Q
A

Tabes dorsalis

81
Q
A

ALS

82
Q
A

Syringomyelia

83
Q
A

SCD: vitamin B12 deficiency

84
Q

Non-infectious cause

A

Spinal muscular atrophy

85
Q
A

Complete occlusion ASA

86
Q

Syringomyelia most commonly occurs at [spinal levels]

A

Syringomyelia most commonly occurs at C8-T1
* Caused by trauma, tumor, chiari malformation

87
Q

Anterior spinal cord syndrome is associated with loss of pain and temperature _ level

A

Anterior spinal cord syndrome is associated with loss of pain and temperature below the lesion

88
Q

Hemisection of the spinal cord causes a contralateral loss of _ two levels below the lesion

A

Hemisection of the spinal cord causes a contralateral loss of pain and temperature two levels below the lesion
* Spinothalamic tract

89
Q

Hemisection of the spinal cord causes ipsilateral _ signs below the level of the lesion

A

Hemisection of the spinal cord causes ipsilateral UMN signs below the level of the lesion

90
Q

Hemisection of the spinal cord causes ipsilateral _ signs at the level of the lesion

A

Hemisection of the spinal cord causes ipsilateral LMN signs at the level of the lesion

91
Q

Hemisection of the spinal cord causes _ lateral loss of fine discrimination, vibration, proprioception below the level of lesion

A

Hemisection of the spinal cord causes ipsilateral loss of fine discrimination, vibration, proprioception below the level of lesion

92
Q

If brown-sequard syndrome occurs above _ level, ipsilateral Horner syndrome (ptosis, miosis, anhidrosis) is possible

A

If brown-sequard syndrome occurs above T1 level, ipsilateral Horner syndrome (ptosis, miosis, anhidrosis) is possible

93
Q

Friedreich ataxia is an autosomal recessive trinucleotide repeat GAA, involving [chromosome] and a gene that encodes _

A

Friedreich ataxia is an autosomal recessive trinucleotide repeat GAA, involving chromosome 9 and a gene that encodes frataxin (iron-binding protein)

94
Q

Friedreich ataxia involves degeneration of [tracts]

A

Friedreich ataxia involves degeneration of lateral corticospinal tract, spinocerebellar tract, dorsal columns

95
Q

The primary pathology in Friedreich ataxia is a loss of _ cells

A

The primary pathology in Friedreich ataxia is a loss of sensory ganglion cells

96
Q

Poliovirus is an RNA picornavirus that causes destruction of cells in the [spinal cord region]

A

Poliovirus is an RNA picornavirus that causes destruction of cells in the anterior horn
* Affects LMNs
* Asymmetric weakness

97
Q

Conus medullaris syndrome involves [spinal levels]

A

Conus medullaris syndrome involves T12-L2

98
Q

Conus medullaris syndrome presents with _ reflexic DTR

A

Conus medullaris syndrome presents with hyperreflexic DTR

99
Q

Sudden and symmetrical describes the deficits of (conus medullaris/cauda equina)

A

Sudden and symmetrical describes the deficits of conus medullaris
* Cauda equina is gradual and asymmetrical

100
Q
A