Gen Path Exam 2 - CNS Disease Flashcards

1
Q

What category of disease?

Hydrocephalus

A

Primary disease

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2
Q

What category of disease?

Epidermal hematoma

A

Vascular/traumatic

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3
Q

What category of disease?

Intracerebral hemorrhage

A

Vascular/traumatic

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4
Q

What category of disease?

Subarachnoid hemorrhage

A

Vascular/traumatic

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5
Q

What category of disease?

Subdural hematoma

A

Vascular/traumatic

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6
Q

What category of disease?

CVA/stroke

A

Vascular/traumatic

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7
Q

What category of disease?

MS

A

Acquired

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8
Q

What category of disease?

Alzheimer’s

A

Acquired

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9
Q

What category of disease?

Parkinson’s

A

Acquired

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10
Q

What category of disease?

Huntington’s

A

Acquired

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11
Q

What category of disease?

ALS

A

Acquired

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12
Q

What category of disease?

Brain abscess

A

Infectious

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13
Q

What category of disease?

Viral meningitis

A

Infectious

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14
Q

What category of disease?

Bacterial meningitis

A

Infectious

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15
Q

What category of disease?

Pilocytic astrocytoma

A

Neoplastic

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16
Q

What category of disease?

Glioblastoma multiforme

A

Neoplastic

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17
Q

What category of disease?

Meningioma

A

Neoplastic

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18
Q

What category of disease?

Medulloblastoma

A

Neoplastic

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19
Q

What category of disease?

Metastatic tumors

A

Neoplastic

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20
Q

Which disease?

Symptomatic increase in the volume of CSF within the cerebral ventricles of the brain

A

Hydrocephalus

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21
Q

In hydrocephalus, what does the increase in volume of CSF in the cerebral ventricles result in?

A

Expansion of ventricles due to increased CSF pressure

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22
Q

What are the causes of hydrocephalus?

A
  1. Obstructive
  2. Impaired absorption of CSF at arachnoid villi (rare)
  3. Overproduction of CSF by choroid plexus neoplasms (very rare)
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23
Q

What is the MOST COMMON form of hydrocephalus?

A

Obstructive

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24
Q

Obstructive hydrocephalus can be ___________ or __________

A

congenital; acquired

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25
Q

What can happen if hydrocephalus is left untreated?

A

Permanent brain damage
Mental impairment
Death

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26
Q

CNS trauma often directly disrupts vessel walls, leading to what?

A

Hemorrhage

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27
Q

Depending on the affected vessel, the hemorrhage and hematoma formation may be one or a combination of what areas?

A

Epidural
Subdural

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28
Q

In epidural hematomas, what vessels are vulnerable to traumatic injury?

A

Dural vessels (especially middle meningeal artery)

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29
Q

In adults with epidural hematoma, what do tears involving dural vessels almost always stem from?

A

Skull fractures

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30
Q

T/F: In infants with epidural hematoma, displacement of the easily deformable skull may tear a vessel, even in the ABSENCE of a skull fracture

A

True!

(epidural hematomas in adults usually always stem from a skull fracture tho)

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31
Q

Once a vessel is torn, blood accumulating under arterial pressure can dissect the tightly applied dura away from the inner skull surface producing, a ___________ that compresses the brain surface

A

hematoma

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32
Q

How does a patient with an epidural hematoma appear clinically?

A

Lucid for hours btwn moment of trauma and development of neurologic signs

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33
Q

An epidural hematoma may expand rapidly and constitutes a neurosurgical ____________ necessitating prompt __________ and repair to prevent _______

A

emergency; drainage; death

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34
Q

What disease?

Caused by tearing of the middle meningeal artery

A

Epidural hematoma

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35
Q

What disease?

Rapid movement of the brain during trauma can tear the bridging veins that extend from the cerebral hemispheres
through the subarachnoid and subdural space to the dural sinuses

A

Subdural hematoma

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36
Q

In subdural hematoma, the disruption of bridging veins that extend from cerebral hemispheres causes what to occur?

A

Bleeding into subdural space

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37
Q

Which types of people have a higher rate/are more susceptible of subdural hematomas?

A

Elderly with brain atrophy
Infants

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38
Q

Why are elderly people with brain atrophy more susceptible to subdural hematomas?

A

Bridging veins are stretched out - brain has additional space to move

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39
Q

Why are infants more susceptible to subdural hematomas?

A

Their bridging veins are thin-walled

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40
Q

When do subdural hematomas become clinically evident?

A

Within 48 hrs after injury

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41
Q

Where are subdural hematomas most commonly found?

A

Over lateral aspects of cerebral hemispheres (may be bilateral)

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42
Q

In subdural hematoma, neurologic signs are attributable to what?

A

Pressure exerted on adjacent brain

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43
Q

What are the symptoms of a subdural hematoma?

A

Headache
Confusion
Slowly progressive neurologic deterioration

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44
Q

T/F: Symptoms of subdural hematoma are most often localized

A

FALSE, they are more often non-localized

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45
Q

How are symptomatic subdural hematomas treated?

A

Surgical removal of blood and associated reactive tissue

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46
Q

What commonly occurs during the healing process of a subdural hematoma? What does this result in?

A

Rebleeding

Results in chronic subdural hematoma

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47
Q

An acute subdural hematoma appears as a collection of ________ _________ blood apposed to the contour of the brain surface, ___________ extension into the depths of ________.

A

freshly clotted; without; sulci

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48
Q

In subdural hematoma, what does the underlying brain look like? What does the subarachnoid space look like?

A

Underlying brain = flat
Subarachnoid space = clear

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49
Q

In subdural hematoma, venous bleeding is ____________ with breakdown and organization of the hematoma taking place over time

A

self-limited

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50
Q

Describe the process of breakdown and organization of subdural hematoma over time

A
  1. Hematoma organizes by lysis of clot in 1 week
  2. Granulation tissue grows from dural surface into hematoma in 2 weeks
  3. Fibrosis occurs in 1-3 months
  4. Fibrosing lesions retract, leaving a thin layer of CT (“subdural membranes”)
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51
Q

What disease?

Characterized by injury to the brain as a consequence of altered blood flow

A

Cerebrovascular disease (CVD)

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52
Q

What disease?

Clinical designation that applies to all the conditions of cerebrovascular disease, particularly when symptoms begin acutely

A

Stroke

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53
Q

What are the 2 mechanisms of the pathophysiology of cerebrovascular disease?

A

Hypoxia/ischemia/infarction
Hemorrhage

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54
Q

Which pathophysiologic mechanism of CVD?

Results from impairment of blood supply and oxygenation of CNS tissue

A

Hypoxia/ischemia/infarction

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55
Q

Which pathophysiologic mechanism of CVD?

Results from rupture of CNS vessels

A

Hemorrhage

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56
Q

What are the common causes of hemorrhage in CVD?

A

Hypertension
Vascular anomalies like malformation and aneurysm

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57
Q

What is the common cause of hypoxia/ischemia/infarction in CVD?

A

Embolism

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58
Q

T/F: In the brain, embolism is a more common etiology of hypxia/ischemia/infarction than thrombosis

A

True

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59
Q

What are the 2 most common causes of cerebral infarctions?

A

Atherosclerotic thrombi
Emboli of cardiac origin

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60
Q

What is the cause of atherosclerotic thrombi, leading to cerebral infarction?

A

Carotid artery atherosclerotic disease (plaque rupture) at carotid bifurcation

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61
Q

What are the causes of emboli of cardiac origin, leading to cerebral infarction?

A

Valvular pathology (mitral)
MI/congestive cardiomyopathy
Cardiac arrhythmia (afib)

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62
Q

What is the extent of cerebral infarction determined by?

A

Site of occlusion
Size of occluded vessel
Duration of occlusion
Collateral circulation

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63
Q

What do neurologic abnormalities from cerebral infarction depend on?

A

Artery involved + area of supply

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64
Q

Gross appearance of an ischemic cerebral infarct varies with ________

A

time

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65
Q

What is the gross appearance of an ischemic cerebral infarct during the first 6 hrs?

A

Little change

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66
Q

What is the gross appearance of an ischemic cerebral infarct during the first 48 hrs?

A

Tissue = pale, soft, swollen
Corticomedullary junction = indistinct

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67
Q

What is the gross appearance of an ischemic cerebral infarct during days 2-10?

A

Brain = gelatinous, friable
Boundary btwn normal and infarcted tissue is more distinct
Edema resolves in viable adjacent tissue

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68
Q

What is the gross appearance of an ischemic cerebral infarct during days 10-3 weeks?

A

Liquefactive necrosis
Fluid-filled cavity that expands until all dead tissue is gone

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69
Q

What risk factor is most commonly associated with clinically significant deep brain intracerebral hemorrhages?

A

Hypertension

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70
Q

Hypertension can cause vessel wall ___________, making them weaker and more vulnerable to rupture. Name 3.

A

abnormalities

  1. Accelerated atherosclerosis (large arteries)
  2. Hyaline arteriosclerosis (small arteries)
  3. Frank necrosis of arterioles
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71
Q

Where does hypertensive intracerebral hemorrhage originate in? (4)

A

Putamen (MOST COMMON)
Thalamus
Pons
Cerebellar hemispheres

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72
Q

In intracerebral hemorrhage, rupture of an artery within brain tissue leads to ____________ of blood, which displaces ________ tissue and causes increased intracranial volume until the resulting tissue compression halts the
bleeding

A

extravasation; brain

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73
Q

What is the MOST COMMON cause of clinically significant subarachnoid hemorrhage?

A

Rupture of saccular aneurysm in cerebral artery

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74
Q

What is the MOST COMMON type of intracranial aneurysm?

A

Saccular aneurysm

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75
Q

Which vessels are most often affected by saccular aneurysms?

A

Circle of Willis vessels

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76
Q

What disease?

May result from:
Extension of a traumatic hematoma,
Rupture of a hypertensive intracerebral hemorrhage into the
ventricular system
Vascular malformation
Hematologic disturbances
CNS tumors

A

Subarachnoid hemorrhage

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77
Q

Which meningitis is the most serious form?

A

Bacterial meningitis

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78
Q

What disease?

This is infection of the leptomeninges (pia and arachnoid mater), and the CSF, which diffusely affects the whole
meninges and subarachnoid space

A

Bacterial meningitis

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79
Q

What are the most common organisms that cause bacterial meningitis?

A

Neisseria meningitidis
Strep pneumoniae

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80
Q

How quickly do symptoms of bacterial meningitis develop?

A

24 hrs to 7 days

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81
Q

What are the following classic symptoms associated with?

Headache
Stiff neck
Fever/chills
Vomiting
Photophobia
Confusion
Seizures
Recent URI

A

Bacterial meningitis

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82
Q

Meningitis caused by ___________ may progress to a meningococcemia with multiple organ involvement, and may be associated with maculopapular, petechial, or purpuric _______ ______

A

N. meningitidis; skin rash

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83
Q

Meningococcemia is sometimes complicated by which syndrome?

A

Waterhouse-Friderichsen syndrome

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84
Q

What disease?

Complications include ventriculitis, intracerebral abscess, cerebral infarction, subdural empyema (pus in subdural space)

A

Meningitis

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85
Q

How is bacterial meningitis diagnosed?

A

Cloudy CSF due to increased neutrophils
Increase in CSF proteins
Decrease in glucose

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86
Q

What is the tx for bacterial meningitis?

A

Vigorous IV antibiotics

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87
Q

How is bacterial meningitis caused by N. meningitidis prevented?

A

Vaccine

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88
Q

What disease?

Mortality ranges from 3% for H. influenzae to 60% for Strep pneumoniae; highest in very young and elderly

A

Bacterial meningitis

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89
Q

What disease?

Serious complications occasionally are
observed including:
Nerve deafness
Cortical blindness
Paralysis
Muscular hypertonia
Ataxia
Complex seizure disorders
Learning disabilities
Obstructive hydrocephalus
Cerebral atrophy

A

Bacterial meningitis

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90
Q

What is the most common cause of meningitis?

A

Viral meningitis

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91
Q

What type of meningitis?

Benign and self-limiting, usually less severe

A

Viral meningitis

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92
Q

Viral meningitis may occur as a complication of what?

A

A viral infection (ex: mumps, measles)

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93
Q

What are the common causative organisms of viral meningitis?

A

Enteroviruses
Mumps virus

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94
Q

What disease?

Clinically presents with acute onset of headache, fever, nuchal rigidity; irritability, and rapid development of
meningeal irritation

A

Viral meningitis

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95
Q

How is viral meningitis diagnosed?

A

CSF = clear/colorless
Increased lymphocytes and protein
Normal glucose

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96
Q

How is viral meningitis treated?

A

Control symptoms (palliative)

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97
Q

What disease?

Severe focal infection of the brain and is typically 1 to 2 cm across

A

Brain abscess

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98
Q

What disease?

Starts as an area of cerebritis and develops into a pus-filled cavity walled off by gliosis and surrounded by cerebral edema

A

Brain abscess

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99
Q

What does a brain abscess often result in?

A

Increased intracranial pressure

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100
Q

What are the 6 causes of brain abscesses?

A

Middle ear infection (60%)
Frontal sinusitis infection (20%)
Bacteremia/septicemia (10%)
Penetrating skull trauma
Meningitis
Odontogenic infection

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101
Q

Which cause of brain abscess?

Due to temporal lobe and cerebellar abscesses

A

Middle ear infection

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102
Q

Which cause of brain abscess?

Due to frontal lobe abscess

A

Frontal sinusitis infection
Bacteremia/septicemia

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103
Q

What are the common causative organisms of brain abscesses?

A

Strep viridans
Staph aureus
Klebsiella
Fungus

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104
Q

What disease?

Clinical presentation is similar to that of acute bacterial meningitis but focal neurological signs, epilepsy, and fever are common manifestations

A

Brain abscess

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105
Q

How are brain abscesses treated?

A

Aggressive antibiotics
Surgical aspiration or excision

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106
Q

What is the overall mortality of a brain abscess?

A

10%

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107
Q

What are the 4 complications of brain abscess?

A

Meningitis
Intracranial herniation
Focal neurological deficit
Epilepsy

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108
Q

What disease?

Characterized by destruction of myelin with relative preservation of axons

A

Demyelinating diseases

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109
Q

What disease is by far the MOST COMMON of the demyelinating diseases?

A

MS

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110
Q

When does MS begin? Is it more common in males or females?

A

20-30 yrs old; females

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111
Q

What disease?

Characterized by relapsing and remitting episodes of immunologically mediated demyelination within the CNS

A

MS

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112
Q

What disease?

Recovery from each episode of demyelination is usually
incomplete, leading to progressive deterioration

A

MS

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113
Q

What is MS caused by?

A

Unknown
Could be immune/viral/environmental/genetic

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114
Q

What disease?

Characterized by irregularly scattered, focal areas of demyelination in the brain and spinal cord

A

MS

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115
Q

What are the 3 favored sites for demyelination in MS?

A

Optic nerve
Brain stem
Periventricular areas

116
Q

MS is typically characterized by ___________ with long asymptomatic __________ and often a progressive course, leading to invalidism w/ mental deterioration

A

exacerbations; remissions

117
Q

What are these 5 early manifestations associated with?

Weakness of legs
Visual disturbances
Retrobulbar pain
Sensory disturbances
Loss of bladder control

A

MS

118
Q

What is the classic manifestation of MS that is significant for diagnosis?

A

Charcot triad

119
Q

Nystagmus, intention tremor, and dysarthria

A

Charcot triad (MS)

120
Q

Heat-induced exacerbation of MS symptoms
(especially optic neuritis). This lead the development of the ‘hot bath test’ in the 1950’s.

A

Uhthoff’s phenomenon (MS)

121
Q

Remained the principal diagnostic tool used to determine if a patient had MS for 30+ years, with the practice ending in the 1980’s when more accurate and safer testing methods became available

A

Uhthoff’s phenomenon (MS)

122
Q

During an acute MS exacerbation, a patient’s face may develop ___________, and muscles of ___________ ___________ (especially the periorbital) can undulate in a wave-like manner

A

paresthesia; facial expression

123
Q

______ manifestations of MS are reported to occur in 2–3% of those affected and may serve as the _____ presenting symptoms of MS

A

Oral; first

124
Q

Unusual muscle movement that resembles
movement of a “bag of worms”

A

Myokymia (MS)

125
Q

If this symptom is observed, referral to a
physician is advised if MS has not been diagnosed

A

Myokymia

126
Q

What is 400x more likely among individuals with MS?

A

Trigeminal neurolgia

127
Q

What are the 4 degenerative diseases?

A

Alzheimer’s
Parkinson’s
Huntington’s
ALS

128
Q

Which disease is the prototype of cortical degenerative diseases?

A

Alzheimer’s

129
Q

Which disease accounts for the majority (50 – 75%) of all cases of dementia?

A

Alzheimer’s

130
Q

Currently, ~ 5.7 million Americans have this disease

A

Alzheimer’s

131
Q

The risk of which disease doubles every 5 yrs after the age of 65 up until age 90?

A

Alzheimer’s

132
Q

Which disease has a female predilicition (~2:1)?

A

Alzheimer’s

133
Q

The prevalence of Alzheimer’s is expected to substantially increase in this century because it preferentially affects the _________, who constitute the fastest growing age group in the U.S

A

elderly

134
Q

The number of persons affected by Alzheimer’s in the U.S. is projected to nearly ________ by the year 2050

A

triple

135
Q

T/F: Most cases of Alzheimer’s are from familial cause (aka early-onset)

A

FALSE; most cases are sporadic (aka late-onset)

136
Q

Alzheimer’s disease affects the 3 processes that keep neurons healthy. Name them.

A

Communication
Metabolism
Repair

137
Q

Which disease?

Certain nerve cells in the brain stop working, lose connections with other nerve cells, and finally die

A

Alzheimer’s

138
Q

In Alzheimer’s, what causes memory failure, personality changes, and problems with daily activities?

A

Destruction and death of nerve cells

139
Q

What are the 2 major factors for pathogenesis of Alzheimer’s?

A

Beta-amyloid senile plaques
Neurofibrillary tangles

140
Q

Which disease?

Other factors like neuroinflammation, oxidative stress, cholinergic insufficiency, mitochondrial dysfunction, and autophagy dysfunction also play major role in the disease progression

A

Alzheimer’s

141
Q

The fundamental abnormality in Alzheimer’s is the accumulation of which 2 proteins in the brain?

A

Beta-amyloid
Tau

142
Q

What protein forms senile plaques in Alzheimer’s?

A

Beta-amyloid

143
Q

What protein forms neurofibrillary tangles in Alzheimer’s?

A

Tau

144
Q

What is the primary critical initiating event for the development of Alzheimer’s and precedes the clinical (symptomatic) onset of Alzheimer’s?

A

Accumulation of senile plaques

145
Q

Besides accumulation of senile plaques, what 3 things accompany the progression of cognitive decline in Alzheimer’s?

A

Neurofibrillary tangles
Neuronal dysfunction/death
Inflammatory rxns

146
Q

What is formed from the transmembrane protein amyloid precursor protein (APP)?

A

Beta-amyloid

147
Q

What readily aggregates and eventually forms into large aggregates and fibrils in the brain called senile plaques?

A

Beta-amyloid

148
Q

What can be directly neurotoxic, meaning even small aggregates can result in neuronal synaptic dysfunction?

A

Beta-amyloid

149
Q

What is an example of neuronal synaptic dysfunction due to beta-amyloid?

A

Blocking of long-term potentiation

150
Q

What elicits an inflammatory response from microglia and astrocytes in the brain?

A

Senile plaques

151
Q

What stimulates the secretion of mediators from microglia and astrocytes that cause damage to neurons?

A

Senile plaques

152
Q

What are neurofibrillary tangles composed of?

A

Neuronal filaments of hyperphosphorylated tau protein

153
Q

What does tau protein normally do?

A

Bind/stabilize microtubules
Support axonal transport of organelles, glycoproteins, neurotransmitters

154
Q

What does tau do once it is hyperphosphorylated (abnormal)?

A

Can’t bind to microtubules
Forms neurofibrillary tangles -> accumulate in pyramidal neurons

155
Q

What are the 2 suggested mechanisms of neurofibrillary tangle injury to neurons?

A
  1. Tau protein aggregates elicit stress response -> neuronal death
  2. Tau protein can’t stabilize microtubules -> neuronal toxicity & death
156
Q

The brain content of the enzyme ______ and its product, ____________, is decreased in patients with AD, in the cerebral cortex, hippocampus and especially the ________ ___________

A

ChAT; acetylcholine; basal forebrain

157
Q

What does ChAT stand for?

A

Choline acetyltransferase

158
Q

What is located in the basal forebrain and is severely lost in Alzheimer’s disease?

A

Cholinergic neurons

159
Q

What contributes to memory and attention deficits seen in Alzheimer’s pts?

A

Loss of cholinergic neurons

160
Q

What disease?

Slow, progressive intellectual deterioration during the course of several years

A

Alzheimer’s

161
Q

Who usually notes insidious memory impairment in pts with Alzheimer’s?

A

Spouse/family member

162
Q

What do pts with Alzheimer’s have difficulty with?

A

Learning/retaining new info
Handling complex tasks
Impaired reasoning, judgment, spatial ability, orientation

163
Q

Behavioral changes such as what may accompany memory impairment in Alzheimer’s?

A

Mood changes
Apathy

164
Q

What may pts develop in later stages of Alzheimer’s?

A

Agitation
Psychosis

165
Q

In Alzheimer’s, the brain shows a variable degree of ________ __________ marked by widening of the cerebral sulci that is most
pronounced in the frontal, temporal, and parietal lobes

A

cortical atrophy

166
Q

With significant atrophy in Alzheimer’s, there is compensatory __________ enlargement (hydrocephalus ex vacuo) secondary to loss of parenchyma and reduced ______ volume

A

ventricular; brain

167
Q

In Alzheimer’s, structures of the medial temporal lobe, including hippocampus, entorhinal cortex and amygdala, are involved
early in the course and are usually severely _________ in the later stages

A

atrophied

168
Q

What are the characteristics of cerebral atrophy in Alzheimer’s?

A

Narrowed gyri
Widened sulci

169
Q

Hydrocephalus ex vacuo

A

Compensatory dilation of ventricles (Alzheimer’s)

170
Q

Composed of an extracellular core of beta-amyloid protein surrounded by microglial cells and reactive astrocytes

A

Senile plaques

171
Q

Occur most frequently in the hippocampus, amygdala and neocortex

A

Senile plaques

172
Q

Abnormal tangles of insoluble cytoskeletal-like hyperphosphorylated tau (paired helical) filaments that form within the cytoplasm of cortical neurons, especially the pyramidal cells of the hippocampus, the amygdala, the basal forebrain.

A

Neurofibrillary tangles

173
Q

Intraneuronal cytoplasmic granule-containing vacuoles occurring within the pyramidal cells of the hippocampus

A

Granulovacuolar degeneration (Alzheimer’s)

174
Q

Elongated, glassy, eosinophilic bodies consisting of paracrystalline arrays of beaded filaments, with actin as their major component

A

Hirano bodies (Alzheimer’s)

175
Q

Found most commonly within hippocampal pyramidal cells

A

Hirano bodies (Alzheimer’s)

176
Q

Distorted, twisted and dilated dendritic processes and axons of cerebral cortex found around beta-amyloid plaques

A

Neuropil threads (Alzheimer’s)

177
Q

What are the biological markers that have been correlated with the degree of dementia?

A

Amount of decreased ChAT
Beta-amyloid burden in brain

178
Q

What disease?

Slowly progressive, debilitating,
degenerative disorder of the basal ganglia

A

Parkinson’s

179
Q

What disease?

Characterized by resting tremor, bradykinesia, rigidity, postural changes, and
often mental changes

A

Parkinson’s

180
Q

What is another name for Parkinson’s?

A

Paralysis Agitans

181
Q

Are males or females more commonly affected by Parkinson’s?

A

Males

182
Q

What disease?

Predominately seen in persons between 50 and 65 years of age, with an average age of onset at 55 years

A

Parkinson’s

183
Q

What disease?

Can be primary (idiopathic) and accounts for ~75% of cases, or secondary (acquired) and accounts for ~ 25% of cases

A

Parkinson’s

184
Q

T/F: The specific etiology of primary Parkinson’s is unknown and is believed to be due to a combination of genetic and
environmental factors

A

True

185
Q

What are the 4 causes of secondary Parkinson’s?

A

Familial
Trauma
Drugs/toxins
Shy-Drager syndrome

186
Q

Describe the inheritance/familial pattern of secondary Parkinson’s

A

Autosomal dominant or recessive

187
Q

Which drug/toxin is correlated to secondary Parkinson’s?

A

MPTP (dopamine antagonist)

188
Q

Autonomic degeneration and orthostatic hypotension with Parkinson’s

A

Shy-Drager syndrome

189
Q

Parkinson’s shows degeneration of pigmented _____________ neurons of the __________ ________, the ________ __________,
and several other brainstem nuclei

A

dopaminergic; substantia nigra; locus ceruleus

190
Q

Degeneration of dopaminergic neurons and other brainstem nuclei causes Parkinson’s by reducing the amount of ___________ in the __________ ___________

A

dopamine; corpus striatum

191
Q

In Parkinson’s, what are surviving cells containing eospinophilic spherical inclusions in the substantia nigra called?

A

Lewy bodies

192
Q

Lewy bodies contain fine filaments composed of what?

A

a-synuclein

193
Q

Protein involved in synaptic transmission

A

a-synuclein

194
Q

When do clinical symptoms of Parkinson’s become evident?

A

Loss of 60% of dopaminergic neurons

195
Q

What disease has the following clinical presentation?

Resting tremor
Bradykinesia
Autonomic dysfunction
Sensory dysfunction
Personality changes
Dementia

A

Parkinson’s

196
Q

What symptom of Parkinson’s?

Seen in the hand as a “pill rolling” tremor (thumb and forefinger); can also involve the leg and lip

A

Resting tremor

197
Q

What does a resting tremor seen in Parkinson’s improve with?

A

Purposeful movement

198
Q

What symptom of Parkinson’s?

General slowing of movements; difficulty in initiating movements and freezing of motion

A

Bradykinesia

199
Q

What symptom of Parkinson’s?

Shuffling gait with short steps, lack of facial expression (mask-like facies), impaired swallowing causing drooling (sialorrhea)

A

Bradykinesia

200
Q

What symptom of Parkinson’s?

Orthostatic hypotension

A

Autonomic dysfunction

201
Q

What symptom of Parkinson’s?

Flushing and excessive sweating (“drenching sweats”)

A

Autonomic dysfunction

202
Q

What symptom of Parkinson’s?

Constipation

A

Autonomic dysfunction

203
Q

What symptom of Parkinson’s?

Paresthesias, pain, akathisia

A

Sensory dysfunction

204
Q

What symptom of Parkinson’s?

Apathy, lack of confidence, fearfulness, anxiety, emotional lability/inflexibility, social withdrawal, dependency

A

Personality changes

205
Q

What symptom of Parkinson’s?

Develops in 10-20% of patients

A

Dementia

206
Q

An autosomal dominant, fatal, progressive movement disorder associated with degeneration of the striatum (caudate/putamen)

A

Huntington’s

207
Q

Huntington’s especially affects which 2 neurons?

A

Cholinergic and GABA-ergic

208
Q

Characterized by the delay of clinical abnormalities until age 30 to 40

A

Huntington’s

209
Q

Course extends 15 to 20 years, beginning with athetoid movements, followed by progressive deterioration leading to hypertonicity, fecal and urine incontinence, anorexia and weight loss, and eventually dementia and death

A

Huntington’s

210
Q

Huntington’s is characterized by increased copies of what within the HD gene?

A

CAG trinucelotide repeats

211
Q

Where is the HD gene located?

A

Short arm of chromosome 4

212
Q

What does the HD gene encode for?

A

Huntingtin protein

213
Q

Subject to ubiquitination and proteolysis yielding fragments that can form intranuclear aggregates

A

Huntingtin protein

214
Q

Huntingtin protein intranuclear aggregates are suspected to be what?

A

Toxic to neurons

215
Q

In Huntington’s, ___________ transmission results in an increased number of CAG repeats and respectively earlier onset of disease manifestations in successive generations

A

paternal

216
Q

In Huntington’s, the brain is small and shows atrophy of the ________ _________ and _________

A

caudate nucleus; putamen

217
Q

Which structure is atrophied secondarily in Huntington’s?

A

Globus pallidus

218
Q

What structures are dilated in Huntington’s?

A

Lateral + 3rd ventricles

219
Q

What is frequently seen in the frontal lobe, less often in the parietal lobe, and occasionally in the entire cortex in a pt with Huntington’s?

A

Atrophy

220
Q

Where is there severe loss of neurons and gliosis in Huntington’s?

A

Affected parts of corpus striatum

221
Q

In Huntington’s, what is present in the remaining striatal neurons and in the cortex? What do they contain?

A

Intranuclear inclusions

Contain aggregates of huntingtin protein

222
Q

What is a synonym of ALS?

A

Lou Gehrig’s

223
Q

Progressive, fatal motor neuron neurodegenerative disease of unknown etiology

A

ALS

224
Q

Characterized by the dysfunction and death of lower motor neurons in anterior horn of the spinal cord, and upper motor neurons (Betz cells) in the motor cortex

A

ALS

225
Q

What does the loss of lower motor neurons in anterior horn of spinal cord lead to in ALS?

A

Muscle atrophy

226
Q

T/F: Majority of cases of ALS are familial

A

FALSE, majority are sporadic, but ~10% are familial

227
Q

What disease?

If familial, caused by mutation of superoxide dismutase gene

A

ALS

228
Q

What is the age range for clinical onset of ALS?

A

50-70 yrs old

229
Q

Does ALS affect men or women more?

A

Men

230
Q

What are the 3 symptom groups of ALS?

A

Limb
Bulbar
Respiratory

231
Q

Which symptom group of ALS?

Painless muscle weakness
Clumsiness (e.g., dropping things, stumbling, falling)
Impaired fine motor skills (e.g., writing)
Limb stiffness
Gait disturbances
Wristdrop or footdrop

A

Limb

232
Q

Which symptom group of ALS?

Dysarthria or voice change
Dysphagia and/or weight loss
Drooling
Choking during swallowing

A

Bulbar

233
Q

Which symptom group of ALS?

Dyspnea on exertion or at rest
Orthopnea

A

Respiratory

234
Q

When does ALS typically lead to death after onset of symptoms?

A

1-6 yrs

235
Q

What factors are most important in the different mechanisms of ALS?

A

Genetic
Exogenous (toxins, viruses, metals)

236
Q

In ALS, the final mechanism of motor neuron death is likely to be via a process resembling what?

A

Apoptosis

237
Q

Most CNS tumors are _________; tumors of the spinal cord are much less frequent

A

intracranial

238
Q

What are the 2nd most common form of malignancy in children? (leukemia is 1st)

A

CNS tumors

239
Q

Primary malignant CNS tumors rarely do what?

A

Metastasize

240
Q

Metastatic tumors to the brain are found more frequently than what?

A

Primary intracranial neoplasms

241
Q

What are the most common primary intracranial tumors in adults?

A

Glioblastoma
Meningioma
Acoustic neuroma

242
Q

What are the most common primary intracranial tumors in children?

A

Medulloblastoma
Cerebellar astrocytomas

243
Q

What do the types of neurological symptoms that result from CNS tumor development depend on?

A

Site
Extent
Rate of growth

244
Q

T/F: Even benign intracranial tumors can result in devastating clinical consequences due to tumor compression of the brain parenchyma

A

True

245
Q

What disease?

Characterized by a history of:
-New-onset partial or generalized seizures
-Focal neurologic deficit (visual disturbances, cranial nerve palsy, motor impairment, hemiplegia, ataxia)
-Altered mental status
-Cognitive impairment

A

CNS tumor

246
Q

Tumors that arise from glial supportive tissue of brain

A

Gliomas

247
Q

They are the most common primary brain tumors, accounting for 50% - 60% of all newly diagnosed CNS tumors

A

Gliomas

248
Q

What type of tumor are astrocytomas?

A

Glioma

249
Q

What are astrocytomas derived from?

A

Astrocytes

250
Q

Where are astrocytomas found?

A

Cerebral hemispheres

251
Q

What CNS tumor?

Most frequent in the 4th-6th decades of life

A

Astrocytoma

252
Q

What CNS tumor?

Account for 80% of gliomas in adults

A

Astrocytoma

253
Q

What CNS tumor?

Regional effects are compression, invasion, destruction of parenchyma

A

Astrocytoma

254
Q

What are the 4 groups of astrocytomas based on?

A

Histology

255
Q

What are the 2 groups of astrocytomas we need to know for exam?

A

Grade I = Pilocytic (juvenile)
Grade IV = Glioblastoma multiforme

256
Q

Most common pediatric brain tumor

A

Pilocytic (juvenile) astrocytoma

257
Q

What CNS tumor?

Peak incidence is under age 20

A

Pilocytic (juvenile) astrocytoma

258
Q

What CNS tumor?

Early symptoms = headache, motor impairment, ataxia, visual disturbance

A

Pilocytic (juvenile) astrocytoma

259
Q

What CNS tumor?

Additional manifestations include features of hydrocephalus

A

Pilocytic (juvenile) astrocytoma

260
Q

What CNS tumor?

Gross tumor resection results in excellent prognosis

A

Pilocytic (juvenile) astrocytoma

261
Q

T/F: Radiotherapy and chemo are routinely used to treat Pilocytic (juvenile) astrocytoma

A

False!!

262
Q

What CNS tumor?

Most benign and treatable type of glioma; survival rate is 95% at 10 yrs

A

Pilocytic (juvenile) astrocytoma

263
Q

What CNS tumor?

Most malignant and pervasive subtype of glioma

A

Glioblastoma multiforme

264
Q

Most common primary tumor in adults

A

Glioblastoma multiforme

265
Q

What CNS tumor?

Peak incidence is 45-70 yrs old

A

Glioblastoma multiforme

266
Q

What CNS tumor?

Etiology largely unknown

A

Glioblastoma multiforme

267
Q

What CNS tumor?

Only well established causative factor is exposure to high doses of ionizing radiation

A

Glioblastoma multiforme

268
Q

What CNS tumor?

Clinical presentation:
Increased intracranial pressure/hypertension
Motor deficit
New onset seizures
Altered level of consciousness
Confusion
Visual deficit
Speech deficit

A

Glioblastoma multiforme

269
Q

What CNS tumor?

Median survival time ~15 months

A

Glioblastoma multiforme

270
Q

Tumor of primitive neuroepithelial cells that arises in the cerebellum in children

A

Medulloblastoma

271
Q

Most common malignant CNS tumor in children

A

Medulloblastoma

272
Q

What CNS tumor?

Highly malignant; rapid growth rate

A

Medulloblastoma

273
Q

What CNS tumor?

May cause CSF obstruction of 4th ventricle, resulting in hydrocephalus

A

Medulloblastoma

274
Q

What CNS tumor?

Characterized histologically by sheets of closely packed anaplastic “small blue” cells with scant cytoplasm arranged in a rosette pattern

A

Medulloblastoma

275
Q

2nd most common primary intracranial neoplasm

A

Meningioma

276
Q

What CNS tumor?

Benign, slowly growing tumor that produces symptoms by compression of brain tissue rather than by invasion

A

Meningioma

277
Q

What CNS tumor?

Occurs most often after age 30; more often in women

A

Meningioma

278
Q

What CNS tumor?

Originates in arachnoidal cells of meninges

A

Meningioma

279
Q

What CNS tumor?

Tumor is external to brain and can be successfully removed surgically

A

Meningioma

280
Q

What CNS tumor?

Most frequently in convexities of cerebral hemispheres and parasagittal region

A

Meningioma

281
Q

What CNS tumor?

Characterized by a whorled pattern of concentrically arranged spindle cells and laminated calcified psammoma bodies

A

Meningioma

282
Q

CNS is a common site for metastasis, and tumors are usually __________

A

multiple

283
Q

Metastatic tumors of the CNS may arise from what 2 ways?

A

Hematogenous
Direct spread

284
Q

What is the preferred site of CNS tumors?

A

Cerebellum

285
Q

Metastases often occur at what boundary in the brain?

A

Btwn gray and white matter

286
Q

What are the 5 most common malignant neoplasms to metastasize the CNS?

A

Breast carcinomas
Bronchus carcinomas
Renal carcinomas
Colon carcinomas
Malignant melanoma