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Pathoma LAH > Neuro 17.2: Spinal Cord Lesions > Flashcards

Neuro 17.2: Spinal Cord Lesions Flashcards

1
Q

What is the mutation in Friedreich Ataxia?

A

unstable trinucleotide repeat (GAA) in the frataxin gene

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2
Q

What is Friedreich Ataxia?

A
  • a degenerative disorder of the cerebellum and spinal cord tracts
  • *** “Friedreich is Fratastic (frataxin): he’s your favorite frat brother, always staggering and falling but has a sweet, big heart.”
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3
Q

Where does sensory info for pain/temperature ascend?

A

in the spinothalamic tract

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4
Q

Why does dysfunction of the frataxin gene cause Friedreich Ataxia to occur?

A
  • normal = essential for mitochondrial iron regulation
  • damage = increased risk of free radicals via the Fenton reaction –> free radical damage of neurons
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5
Q

What is Riluzole? What is it for? How does it work?

A
  • tx for ALS/Lou Gehrig disease
  • decreases glutamate excitotoxicity (via unknown mech.)
  • *** “For Lou Gehrig disease, give rilouzole”
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6
Q

Dx?

  • unstable trinucleotide repeat (GAA) in the frataxin gene
A

Friedreich Ataxia

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7
Q

Where in the spinal cord is the the hypothalamic spinal tract found?

A

in the lateral horns

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8
Q

What does syringomyelia arise in assoc. with?

A
  • trauma
  • Arnold-Chiari malformation
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9
Q

Who classically gets Amyotrophic Lateral Sclerosis (ALS)?

A

middle-aged adults

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10
Q

Where does sensory info for pain/temperature decussate?

A

at the anterior white commissure

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11
Q

This is the name for the cystic degeneration of the spinal cord at the anterior white commissure.

A

syringomyelia

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12
Q

Dx?

  • a degenerative disorder of upper AND lower motor neurons (the corticospinal tract)
A

Amyotrophic Lateral Sclerosis (ALS)

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13
Q

How is polio transmitted?

A

fecal oral

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14
Q

Which spinal cord lesion presents with a “cape-like” distribution of symptoms?

A

syringomyelia

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15
Q

O2- + superoxide dismutase = _______

A

H2O2

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16
Q

What does a negative Babinkski sign look like?

A

downgoing toes

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17
Q

What are the initial s/s of a poliovirus infection?

A
  • fever
  • sore throat
  • ab pain
  • nausea/vomiting
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18
Q

How can Amyotrophic Lateral Sclerosis (ALS) be distinguished from syringomyelia?

A

there is NO sensory impairment in Amyotrophic Lateral Sclerosis (ALS)

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19
Q

At what level of the spinal cord does syringomyelia classically occur?

A

C8-T1

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20
Q

If a syringomyelia lesion expanded, it would knock out the anterior horns and then the ______ of the spinal cord.

A

lateral horns

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21
Q

On which chromosome is the frataxin gene located?

A

chromosome 9

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22
Q

What are the 3 classic findings of Horners syndrome?

A

ptosis, myosis, and anhydrosis

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23
Q

Where does sensory info for pain/temperature synapse?

A

in the posterior horn of the spinal cord

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24
Q

What is the prognosis for Werdnig-Hoffman disease?

A

death w/i a few years after birth (median = 7 mos)

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25
Q

What disease presents like poliovirus?

A

Werdnig-Hoffman disease

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26
Q

What is Amyotrophic Lateral Sclerosis (ALS)?

A

a degenerative disorder of upper AND lower motor neurons (the corticospinal tract)

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27
Q

Where in the body does poliovirus initially infect?

A

oropharynx and small bowel

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28
Q

Dx?

  • a degenerative disorder of the cerebellum and spinal cord tracts
  • ataxia
  • loss of vibration sense
  • loss of proprioception
  • muscle weakness in lower extremities
  • loss of DTRs
A

Friedreich Ataxia

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29
Q

How does poliomyelitis present?

A
  • lower motor neuron signs:
    • muscle weakness and atrophy
    • decreased tone
    • impaired reflexes
    • flaccid paralysis
    • negative Babinksi sign (downgoing toes)
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30
Q

What info. does the dorsal column of the spinal cord carry?

A

fine touch/position sense

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31
Q

If a syringomyelia lesion expanded, it would first knock out the _____ of the spinal cord.

A

anterior horns

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32
Q

What runs thru the lateral horn of the spinal cord?

A

the hypothalamal spinal tract- sympathetic input to the face

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33
Q

Upper motor neurons start in the cortex and first synapse in the anterior horn of the spinal cord. Then, the lower motor neuron continues on to the _______.

34
Q

What are the upper motor neuron (UMN) dysfunction signs?

A
  • spastic paralysis
  • hyperreflexia
  • increased muscle tone
  • positive Babinski sign (= upward going toes)
35
Q

How is Werdnig-Hoffman disease transmitted?

A

auto. recessive inheritance

36
Q

What are the clinical s/s associated with loss of the hypothalamal spinal tract at T1?

A
  • ptosis, myosis, and anhydrosis (Horner’s syndrome)
37
Q

What are the sensory findings in syringomyelia?

A
  • loss of pain and temp. sensation in the upper extremities
  • ***fine touch, position sense in tact
38
Q

How does one get Friedreich Ataxia?

A

it’s auto. recessive

39
Q

What is poliomyelitis?

A

damage to the anterior motor horn due to poliovirus infection

40
Q

Upper or lower motor neuron defect?

  • muscle weakness and atrophy
  • decreased tone
  • impaired reflexes
  • flaccid paralysis
  • negative Babinksi sign
A

lower motor neuron

41
Q

What will the CSF findings be in polio?

A
  • increased WBCs
  • slightly increased protein
  • normal glucose
42
Q

What is the function of the frataxin gene?

A

it’s essential for mitochondrial iron regulation

43
Q

O2- –> H2O2 via?

A

superoxide dismutase (SOD)

44
Q

What are the signs of lower motor neuron dysfunction?

A
  • muscle weakness and atrophy
  • decreased tone
  • impaired reflexes
  • flaccid paralysis
  • negative Babinksi sign = downgoing toes
45
Q

Where does sensory info for pain/temperature synapse in the brain?

A

the thalamus –> cortex

46
Q

Does polio present with symmetric or asymmetric weakness? What about Werdnig-Hoffmann disease?

A
  • polio = asymmetric
  • Werdnig-Hoffmann = symmetric
47
Q

What familial mutation is known to cause Amyotrophic Lateral Sclerosis (ALS)?

A

zinc-copper superoxide dismutase mutation

48
Q

In the hypothalamic spinal tract, a nerve arising in the ______ synapses on the lateral horn at T1.

A

hypothalamus

49
Q

In the hypothalamic spinal tract, a nerve arising in the hypothalamus synapses on the _______ at T1.

A

lateral horn

50
Q

Do ALS pts have bowel/bladder deficits?

51
Q

Where is the cell body located for pain/temperature?

A

in the sensory ganglion outside the spinal cord

52
Q

How many different nerves are used to transmit pain/temperature info from the periphery to the brain?

53
Q

This gene is essential for mitochondrial iron regulation.

A

the frataxin gene

54
Q

How is Amyotrophic Lateral Sclerosis (ALS) transmitted?

A
  • most = sporadic genetic mutations
  • some = familial zinc-copper superoxide dismutase mutation
55
Q

What other pathology is Friedreich Ataxia associated with?

A
  • hypertrophic cardiomyopathy
  • diabetes mellitus
  • hammer toes
56
Q

Upper motor neurons start in the cortex and first synapse in the _______.

A

anterior horn of the spinal cord

57
Q

Ataxia + multiple spinal cord tract dysfunction = ?

A

Friedreich Ataxia

58
Q

What is the function of superoxide dismutase (SOD)?

A

removal of free radicals

59
Q

How does poliovirus get to the CNS?

A

hematogenous spread

60
Q

What occurs in the posterior horn of the spinal cord?

A

pain/temperature sensory info synapses here

61
Q

What info. is transmitted in the anterior horn of the spinal cord?

A

motor neurons

62
Q

Dx?

  • an inherited degeneration of the anterior motor horn
A

Werdnig-Hoffman disease

63
Q

Why is syringomyelia?

A

a cystic degeneration of the spinal cord at the anterior white commissure

64
Q

In the hypothalamal spinal tract, a nerve arising in the hypothalamus synapses on the lateral horn at _____.

65
Q

Is there loss of pain/temp sensation in ALS or syringomyelia?

A

syringomyelia

66
Q

Where in the spinal cord does syringomyelia classically occur?

A

in the anterior white commissure

67
Q

How does Werdnig-Hoffman disease present?

A
  • a floppy baby
  • tongue fasciculations
  • s/s similar to polio but will have SYMMETRIC WEAKNESS
68
Q

Upper or Lower Motor Neuron dysfunction signs?

  • spastic paralysis
  • hyperretlexia
  • increased muscle tone
  • positive Babinski sign
A

Upper motor neuron

69
Q

Dx?

  • damage to the anterior motor horn due to poliovirus infection
  • lower motor neuron signs
A

poliomyelitis

70
Q

What is the result if the anterior white commissure of the spinal cord is lesioned?

A

bilateral loss of pain and temperature sensation

71
Q

What neuro. disease is hypertrophic cardiomyopathy assoc. with?

A

Friedreich Ataxia

72
Q

When does Friedreich Ataxia present? How?

A
  • in early childhood
  • kyphoscoliosis
73
Q

What info does the hypothalamic spinal tract carry?

A

sympathetic input to the face

74
Q

How does Friedreich Ataxia present?

A
  • ataxia
  • loss of vibration sense
  • loss of proprioception
  • muscle weakness in lower extremities
  • loss of DTRs
75
Q

Does a syringomyelia usually affect the upper or lower limbs? Why?

A
  • upper
  • because they usu. occur at C8-T1
76
Q

What is an early sign of Amyotrophic Lateral Sclerosis (ALS)?

A
  • atrophy
  • hand weakness
77
Q

What occurs in the at the anterior white commissure of the spinal cord?

A

sensory info for pain/temperature decussates

78
Q

What does a zinc-copper superoxide dismutase mutation cause?

A

Amyotrophic Lateral Sclerosis (ALS)

79
Q

Does Amyotrophic Lateral Sclerosis (ALS) present with upper or lower motor neuron dysfunction signs? Why?

A
  • Both
  • Anterior motor horn involvement = LMN signs
  • Lateral corticospinal tract involvement = UMN signs
80
Q

What is the tx for ALS? How does it work?

A
  • riluzole
  • decreases glutamate excitotoxicity (via unclear mech.)
  • *** “For Lou Gehrig disease, give rilouzole”

Pathoma LAH (77 decks)

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