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Systems Pathology Exam Two > Chapter 22 - CNS Part 3 > Flashcards

Chapter 22 - CNS Part 3 Flashcards

1
Q

What is the result of cerebellar degeneration?

A

Ataxia

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2
Q

What is the result of motor neuron degeneration?

A

Weakness

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3
Q

What is the result of degeneration of the cortex?

A

Memory, language, insight/planning issues

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4
Q

What is dementia?

A

Impaired memory and cognition along with personality changes accompanied with decreased functioning

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5
Q

What is the most common cause of dementia?

A

Alzheimer’s disease

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6
Q

After the age of 85, what is the frequency for Alzheimer’s disease?

A

50%

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7
Q

What kind of plaques are associated with Alzheimer’s disease?

A

Beta-amyloid

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8
Q

What is the resulting substance from the neuronal degeneration of Alzheimer’s disease?

A

Tau proteins

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9
Q

What happens to the brain in Alzheimer’s disease?

A

Neurotoxicity, decreased neurotransmission, cortical atrophy

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10
Q

How long does it usually take to be diagnosed with Alzheimer’s disease?

A

5-10 years

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11
Q

What chromosome abnormality based condition is associated with the 10% genetic predisposition of Alzheimer’s disease?

A

Down Syndrome

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12
Q

Is Alzheimer’s disease widely genetic?

A

No (only 10%)

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13
Q

What is the culprit for CNS degeneration?

A

Abnormal protein accumulation

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14
Q

What is the most common cause of Parkinsonism?

A

Parkinson’s disease

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15
Q

What is Parkinsonism?

A

Abnormal motor function, tremor, rigidity, bradykinesia, instability

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16
Q

What neurons are damaged in Parkinsonism?

A

Dopaminergic neurons

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17
Q

What specific CNS areas are affected by Parkinsonism?

A

Substantia nigra, cortex, medulla, pons

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18
Q

What CNS neural activity is altered in Parkinsonism?

A

Synaptic transmission (most commonly involving dopamine)

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19
Q

Is Parkinsonism always caused by Parkinson’s disease?

A

No (just most commonly)

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20
Q

A Lewy body is a cluster of what protein?

A

Alpha-synuclein

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21
Q

What type of inclusion is associated with Parkinson’s disease?

A

Lewy bodies

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22
Q

Pallor of the substantia nigra is associated with what neurological disease?

A

Parkinson’s

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23
Q

What is the usual age of onset for Parkinson’s disease?

A

45-65 years

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24
Q

How long does it typically take for Parkinson’s to become immobilizing?

A

10-15 years

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25
Q

What type of gait is associated with Parkinson’s disease?

A

Festinating gait

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26
Q

Pill-rolling of the thumb and index fingers along with cogwheel rigidity is associated with what neurological disease?

A

Parkinson’s disease

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27
Q

What is usually the cause of death of those with Parkinson’s?

A

Frequent falls, infection (pneumonia)

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28
Q

What is the condition when dementia is present within one year of motor symptoms (Parkinson’s)?

A

Lewy body dementia

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29
Q

What is the treatment for Parkinson’s disease?

A

L-DOPA (increases dopamine), deep brain stimulator

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30
Q

What is the genetic pattern of Huntington disease?

A

Autosomal dominant

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31
Q

What is the genetic factor involved in Huntington disease?

A

Huntington gene, trinucleotide repeat: CAG (anticipation generation by generation)

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32
Q

What inclusion is involved with Huntington disease?

A

Huntingtin protein

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33
Q

Cerebral atrophy due to Huntington disease is prominent at what CNS locations?

A

Caudate and putamen nuclei

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34
Q

What is the typical age of delayed onset of Huntington disease?

A

30-40 year old (don’t forget it is still an inherited disease

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35
Q

Chorea of the entire body is associated with what condition?

A

Huntington disease

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36
Q

Huntington disease is most commonly lethal after how many years?

A

About 15

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37
Q

How do those with Huntington disease typically die?

A

Pneumonia, injuries, choking, suicide

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38
Q

What neurological condition is uniquely associated with both lower and upper motor neuron death?

A

Amyotrophic lateral sclerosis

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39
Q

What locations and tracts are affected with the lower motor neuron death seen in ALS?

A

Corticospinal and corticobulbar tracts, anterior horn cells, ventral nerve roots

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40
Q

What general locations of the CNS are affected with lower motor neuron death seen in ALS?

A

Spinal cord and brainstem

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41
Q

What specific cells are associated with upper motor neuron death seen in ALS and in what location?

A

Betz cells in the posterior frontal lobe

42
Q

What gender is more likely to get ALS?

A

Males

43
Q

What is the usual age range for ALS?

A

40-50 years

44
Q

What muscular effects are seen in ALS?

A

Fasciculations, asymmetric distal extremity weakness, progressive muscle atrophy and decreased strength

45
Q

What specific muscles are unaffected by ALS?

A

Extraocular muscles

46
Q

What is usually the cause of death with those who have ALS?

A

Respiratory paralysis (pneumonia)

47
Q

What is the genetic pattern of ALS?

A

10% autosomal dominant

48
Q

Are cases of ALS usually sporadic or genetically inherited?

A

Sporadic (90%)

49
Q

What is the frequency of cranial CNS tumors?

A

15 per 100,000

50
Q

What is the frequency of spinal CNS tumors?

A

2 per 100,000

51
Q

Which are more frequent: cranial or spinal CNS tumors?

A

Cranial

52
Q

Are CNS tumors common overall?

A

Not really (17 per 100,000)

53
Q

Do CNS tumors usually metastasize outside of the CNS?

A

Not usually

54
Q

Are CNS tumors usually primary or secondary?

A

Primary

55
Q

What is significant about the stages of CNS tumors?

A

No premalignant (in situ) stages

56
Q

What effect do CNS tumors usually have on intracranial pressure?

A

Increase

57
Q

What is the term for a tumor derived from glial cells?

A

Glioma

58
Q

What are the three broad categories of gliomas?

A

Astrocytoma, oligodendroglioma, ependymoma

59
Q

Which categories of gliomas are known to be diffuse/infiltrative?

A

Astrocytoma and oligodendroglioma

60
Q

What kind of CNS tumor makes up 80% of adult gliomas?

A

Diffuse astrocytomas

61
Q

What is the usual age range of diffuse astrocytomas?

A

30-60

62
Q

Are diffuse astrocytomas benign or malignant?

A

Always malignant

63
Q

What is the prognosis for diffuse astrocytomas?

A

Poor: 1-5 years

64
Q

What is the primary location for diffuse astrocytomas?

A

Mostly frontal lobe

65
Q

What are the three grades of diffuse astrocytomas?

A

A: well-differentiated
B: anaplastic
C: glioblastoma

66
Q

What is the worst grade of a diffuse astrocytoma?

A

Glioblastoma (close to just a 1 year prognosis)

67
Q

What is the general location for diffuse astrocytomas?

A

Cerebral cortex (rarely brainstem or cerebellum)

68
Q

What kind of astrocytoma is benign, cystic, and commonly affects children or young adults?

A

Pilocytic

69
Q

What is the usual location of pilocytic astrocytomas?

A

Cerebellum and spinal cord (rarely cerebral cortex)

70
Q

Which is has a poorly circumscribed lesion: a diffuse or pilocytic astrocytoma?

A

Diffuse

71
Q

Which is has a well-circumscribed lesion: a diffuse or pilocytic astrocytoma?

A

Pilocytic

72
Q

Why does cerebral edema possibly occur with glioblastomas?

A

Leaky angiogenesis

73
Q

What is the age range of oligodendrogliomas?

A

30-50

74
Q

What type of tumor makes up about 5-15% of all gliomas?

A

Oligodendroglioma

75
Q

What is the usual location for oligodendrogliomas?

A

Frontal or temporal lobes of the cortex

76
Q

What is the prognosis for oligodendrogliomas?

A

5-20 years

77
Q

A history of neurological problems, especially years of seizures, is associated with what kind of CNS tumor?

A

Oligodendroglioma

78
Q

What CNS tumor is most commonly seen among pediatrics?

A

Ependymomas

79
Q

What is the most common location for ependymomas in pediatrics?

A

Periventricular regions

80
Q

What is the most common location for ependymomas in adults?

A

Spinal canal

81
Q

Which type of CNS tumor presents a microscopic fibrillary appearance with rosettes/canals?

A

Ependymoma

82
Q

Which CNS tumor presents a uniform, dark chromatin, and clear halo microscopic appearance?

A

Oligodendroglioma

83
Q

Embryonal (primitive) neoplasms are derived from what cells?

A

Neuroectodermal cells (small round cells)

84
Q

Is a medulloblastoma benign or malignant?

A

Malignant

85
Q

What is the location for a medulloblastoma?

A

Exclusively in the cerebellar region (specifically midline and infratentorial)

86
Q

What is the difference between a medulloblastoma and a primitive neuroectodermal tumor?

A

PNETs are located outside of the CNS

87
Q

Medulloblastoma are similar to what other kind of tumor?

A

Primitive neuroectodermal tumors

88
Q

What kind of tumor make up 20% of all pediatric tumors?

A

Medulloblastoma

89
Q

What type of malignant tumor is highly radiosensitive, well-circumscribed, and hyperchromatic nuclei?

A

Medulloblastoma

90
Q

Homer-Wright rosettes with a central neutrophil are commonly seen in which pediatric malignant tumor?

A

Medulloblastoma

91
Q

What is the most common CNS tumor in immunosuppressed patients, most uniquely with AIDS patients?

A

Primary CNS lymphoma

92
Q

What is a primary CNS lymphoma made of?

A

Diffuse large B cells

93
Q

Testing positive for what virus can lead to extensive CNS necrosis and possibly a primary CNS lymphoma?

A

EBV (which targets B cells possibly leading to their transformation)

94
Q

How does primary CNS lymphoma spread?

A

Via ventricles

95
Q

What is the age range for primary CNS lymphomas?

A

Any age but increases with age after the age of 60

96
Q

What is the usual location for primary CNS lymphoma?

A

Any brain tissue (multifocal)

97
Q

Are meningiomas benign or malignant?

A

Benign

98
Q

What are the transformed cells of meningiomas?

A

Transformed arachnoid cells

99
Q

Meningiomas most commonly affect what population?

A

Adult females

100
Q

Why don’t CNS tumors usually metastasize to other areas of the body?

A

Dural protection

101
Q

Tumors can metastasize to the CNS from what kinds of cancers?

A

Lung, breast, melanoma, kidney, GI

102
Q

What kind of tumor makes up 25-50% of intracranial tumors?

A

Tumors metastasizing to the CNS from other locations

Systems Pathology Exam Two (6 decks)

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