Systems Pathology I Material Flashcards

1
Q

What is the general term for a developmental anomaly of bone?

A

dysostosis

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2
Q

what causes dysostosis?

A

Abnormal mesenchymal migration during embryogenesis

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3
Q

what is the result of dysostosis?

A

Defective ossification of fetal cartilage

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4
Q

What is Klippel-Feil Syndrome?

A

Congenital fusion of any 2 cervical vertebrae

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5
Q

What is the most common congenital malformation?

A

Syndactyly

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6
Q

What condition represents malformation and dysostosis of the scapula?

A

Sprengel’s Deformity

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7
Q

What term is used to describe absent or incomplete development of tissue?

A

aplasia

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8
Q

What is dysplasia?

A

When mutations interfere with growth or homeostasis (dwarfism)

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9
Q

Multiple fractures, extremity bowing, scoliosis, ligamentous laxity (dislocations), and hearing loss are associated with what musculoskeletal congenital disorder?

A

Osteogenesis imperfecta

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10
Q

The zebra stripe sign is associated with what musculoskeletal congenital disorder?

A

Osteogenesis imperfecta

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11
Q

Is achondroplasia usually sporadic or inherited?

A

sporadic (75%)

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12
Q

What musculoskeletal congenital condition is accompanied by short proximal extremities, lower extremity bowing, frontal bossing, and mid face hypoplasia (low nasal bridge)?

A

Achondroplasia

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13
Q

What percentage of achondroplasia diagnoses are inherited?

A

25%

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14
Q

What type of dwarfism is primarily endocrine oriented and is more rare than achondroplasia?

A

Hypopituitary dwarfism

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15
Q

Spinal issues including hyperlordosis and hyperkyphosis at birth, bullet vertebrae, scoliosis, and spinal stenosis are common among which musculoskeletal genetic disorder?

A

achondroplasia

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16
Q

What two specific types of spinal stenosis are common among achondroplasia patients?

A

Foramen magnum stenosis and spinal canal stenosis

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17
Q

What is another name for osteogenesis imperfecta?

A

brittle bone disease

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18
Q

What type of dwarfism is completely fatal?

A

Thanatophoric dwarfism

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19
Q

Extremely small thorax with shortened long bones leading to perinatal respiratory failure occurs with which musculoskeletal congenital disorder?

A

Thanatophoric dwarfism

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20
Q

What rare genetic disorder is a result of decreased osteoclast-mediated bone resorption?

A

Osteopetrosis

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21
Q

Why is deranged hematopoiesis common with osteopetrosis?

A

The medullary cavity fills with extra bone due to the decreased osteoclast activity

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22
Q

Why does hepatosplenomegaly occur with osteopetrosis?

A

The liver and spleen attempt to take over the function of the bone marrow that has been compressed and therefore hypertrophy

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23
Q

Erlenmeyer flask deformity is present among which musculoskeletal disorder?

A

osteopetrosis

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24
Q

What is the most important form of osteopenia?

A

osteoporosis

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25
Q

What is usually the cause of localized osteoporosis?

A

disuse/immobilization

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26
Q

What is the most common type of osteoporosis?

A

primary

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27
Q

When does primary osteoporosis occur?

A

post-menopausal/ senile

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28
Q

Neoplasia, hyperparathyroidism, nutrient deficiency, and drug exposure are causes of what which type of osteoporosis?

A

secondary

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29
Q

What is mutated in osteogenesis imperfecta?

A

Type 1 collagen (alpha 1 or alpha 2 chains)

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30
Q

Osteoporosis is more likely to affect what kind of bone?

A

Trabecular (cancellous/spongy) bone

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31
Q

What are specific common locations of fractures as a result of osteoporosis due to these areas possessing increased amounts of trabecular bone?

A

Vertebral bodies, femoral neck, calcaneus

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32
Q

What type of bone cell is less impacted by aging and therefore retains normal activity?

A

osteoclasts

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33
Q

How is type I collagen affected by osteogenesis imperfecta?

A

Premature breakdown of collagen

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34
Q

Beginning in mid-20s, bone loss occurs at what rate?

A

.5% per year

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35
Q

What percentage of postmenopausal women experience an osteoporosis related fracture?

A

50%

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36
Q

Osteogenesis imperfecta follows what type of genetic pattern?

A

Autosomal dominant

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37
Q

What percentage of males (of the same age as postmenopausal women) experience an osteoporosis related fracture?

A

3%

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38
Q

Reduction in what hormone accelerates bone loss as seen in postmenopausal women with osteoporosis?

A

estrogens

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39
Q

What is the age of maximum bone density?

A

mid-adulthood, 25

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40
Q

How can osteoporosis be prevented?

A

diet and exercise

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41
Q

Calcium and vitamin D deficiency are most common among what gender?

A

females

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42
Q

Osteoporosis is usually asymptomatic, true or false?

A

true

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43
Q

What is the appropriate way of determining osteoporosis?

A

DEXA scan

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44
Q

What are the specific areas affected by osteogenesis imperfecta?

A

Eyes, inner ear bones, skin, joints, teeth

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45
Q

What are the two extreme issues with type II osteogenesis imperfecta?

A

Cerebral hemorrhage and respiratory failure

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46
Q

What is the unique effect of osteogenesis imperfecta on the eye?

A

Abnormal collagen of the sclera (choroid) giving a bluish appearance instead of white

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47
Q

With what type of osteogenesis imperfecta does abnormal collagen of the sclera occur?

A

type 1

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48
Q

What is the treatment used for children with osteogenesis imperfecta?

A

Bisphosphonates

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49
Q

What is the most common form of dwarfism?

A

achondroplasia

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50
Q

What are the results of achondroplasia?

A

Decreased cartilage synthesis and decreased growth plate expansion

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51
Q

What location is specifically affected by the decreased cartilage synthesis with achondroplasia?

A

growth plate

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52
Q

What is the genetic mutation with achondroplasia?

A

Fibroblast growth factor receptor (FGFR3) mutation

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53
Q

What is the genetic pattern of achondroplasia?

A

autosomal dominant

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54
Q

What is usually the death of those with achondroplasia?

A

brain stem compression

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55
Q

What is trident hand that is associated with achondroplasia?

A

Underdevelopment of the bones in the hands

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56
Q

What type of bone cell is inhibited in osteopetrosis & what is the result of that?

A

Osteoclasts; osteoblasts therefore overwork and build up extreme bone density leading to increased fractures

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57
Q

Why are cranial nerve palsies associated with osteopetrosis?

A

Foraminal stenosis occurs narrowing the bony canals for the nerves

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58
Q

What is the treatment for osteopetrosis?

A

stem cell replacement

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59
Q

Enlargement of what organ is a cause of secondary osteoporosis?

A

parathyroid

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60
Q

What types of drugs can be a cause of secondary osteoporosis?

A

Alcohol, smoking, long term corticosteroids

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61
Q

How is the cortex of the bone affected by osteoporosis?

A

Thinned (not completely destroyed)

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62
Q

What type of osteogenesis imperfecta results in a normal lifespan nonetheless?

A

type 1

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63
Q

What is the exaggerated forward hunching of those with osteoporosis called that is due to multiple compression fractures?

A

Dowager’s hump

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64
Q

What type of bone cells is affected by the aging cause of osteoporosis?

A

Osteoblasts (decreased synthesis)

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65
Q

What is another feature important to consider with the aging cause of osteoporosis besides the reduction in osteoblast synthesis?

A

general sedentary lifestyle

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66
Q

What is the main location affected by osteogenesis imperfecta?

A

Extracellular matrix (ECM)

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67
Q

What is the result with brain stem compression with achondroplasia?

A

Cardiovascular abnormalities (may be lethal)

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68
Q

What are some issues that occur when the medullary cavity fills with excess bone in osteopetrosis?

A

Hepatosplenomegaly, recurrent infections, anemia

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69
Q

Which type of osteogenesis imperfecta is lethal in utero?

A

type 2

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70
Q

Why is cerebral hemorrhage common with type II osteogenesis imperfecta?

A

weak skull

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71
Q

At what point does osteopenia become the severe form of osteoporosis?

A

T score of 2.5 standard deviation (threshold from the norm)

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72
Q

Activity of what substances are decreased in senile osteoporosis?

A

growth factors and osteoblasts

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73
Q

What are some concerns following surgery for a femoral neck (hip) fracture?

A

Pneumonia, deep vein thrombosis, pulmonary embolism (result of disablement)

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74
Q

What is osteopenia?

A

reduced bone mineralization

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75
Q

What is a positive note on the current findings of Paget’s Disease diagnoses?

A

its becoming less common and less severe

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76
Q

What is ivory vertebra sign?

A

Increased density of one specific vertebral level

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77
Q

What named conditions result from vitamin D deficiency?

A

Rickets (in children) or osteomalacia (in adults)

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78
Q

What is the name of the perpendicular fractures seen on X-ray with Paget’s Disease?

A

chalk stick fractures

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79
Q

What is secreted from the parathyroid glands?

A

Parathyroid hormone (PTH)

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80
Q

In what condition can a “brown tumor” result?

A

Hyperparathyroidism

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81
Q

What is a “brown tumor”?

A

Collection of loose connective tissue filling a space, giving off a bloody appearance (not actually a tumor but a lesion)

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82
Q

Renal stones, peptic ulcers, bowing of long bones, and pathological fractures are present with what condition?

A

hyperparathyroidism

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83
Q

The salt & pepper skull is indicative of what condition?

A

hyperparathyroidism

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84
Q

Is hyperparathyroidism possibly reversible?

A

Yes, with normalization of PTH levels

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85
Q

Most cases of Paget’s Disease are asymptomatic: true or false?

A

true, up to 90%

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86
Q

The appearance of Paget’s Disease of the pelvis on X-ray is what?

A

fuzzy/shaggy looking

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87
Q

What is the appearance of the bone cells with Paget’s Disease?

A

Mosaic, jigsaw puzzle appearance

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88
Q

What population is more like to develop Paget’s Disease?

A

caucasians

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89
Q

What is the older term for Paget’s Disease?

A

Osteitis Deformans

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90
Q

Why are there increased levels of serum alkaline phosphatase in those with Paget’s Disease?

A

It is a byproduct of osteoblast activity

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91
Q

When ivory vertebra sign is present, what are likely causes?

A

Paget’s Disease, metastatic cancer (usually from prostate cancer), lymphoma

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92
Q

If a patient comes in saying they’ve noticed their hats don’t quite fit anymore, it could be indicative of what condition?

A

Paget’s Disease

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93
Q

What is the average age of diagnosis of Paget’s Disease?

A

70 years old

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94
Q

What gender is at a greater risk for Paget’s Disease?

A

males (2X)

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95
Q

What is the treatment for Paget’s Disease?

A

Bisphosphonates

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96
Q

What is the intention with treating Paget’s Disease with bisphosphonates?

A

Slowing of the osteolytic (first) phase

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97
Q

What are ways vitamin D deficiency can occur?

A

Malnutrition, deficient UV Rays, abnormal metabolism

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98
Q

Osteomalacia is similar to what condition?

A

osteoporosis

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99
Q

What is the main function of the parathyroid glands?

A

Maintains serum calcium (in blood)

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100
Q

What type of hyperparathyroidism involves autonomous PTH production?

A

primary

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101
Q

What is the most common cause of excessive PTH?

A

From an adenoma (80%)

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102
Q

Excessive PTH has what kind of effect on bone cells?

A

Increased osteoclast activity

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103
Q

What is absorbed more heavily from renal tubules in a state of excessive amounts of PTH?

A

vitamin D and calcium

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104
Q

What can result from secondary hyperparathyroidism?

A

Mild renal failure

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105
Q

How is hyperparathyroidism diagnosed?

A

Hypercalcemia, parathyroid immunoassay

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106
Q

What is the most common nonmalignant cause of hypercalcemia?

A

Hyperparathyroidism

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107
Q

In which group is hyperparathyroidism most common?

A

Post menopausal women

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108
Q

Hyperparathyroidism results in resorption of what type of bone?

A

BOTH cortical and trabecular bone

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109
Q

When cortical and trabecular bone are resorbed in hyperparathyroidism, what replaces that bone?

A

Loose connective tissue

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110
Q

What is a condition that produces holes in the skull that can appear similar to the salt and pepper skull seen on X-rays of those with hyperparathyroidism?

A

multiple myeloma

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111
Q

Rugger-jersey spine is seen on X-rays of those with what condition?

A

Hyperparathyroidism

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112
Q

Bone pain, fractures, deformation, kidney stones, nausea, anorexia, fatigue, and decreased cognition are features of what condition?

A

hyperparathyroidism

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113
Q

Subperiosteal resorption of the radial side of the middle phalanges is unique to what condition?

A

hyperparathyroidism

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114
Q

What are the three phases of Paget’s Disease?

A

Osteolytic phase, mixed osteolytic and osteoblastic phase, osteosclerotic phase

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115
Q

What are the general symptoms of Paget’s Disease when they’re present?

A

Bone pain, multiple fractures, nerve compression

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116
Q

In what percentage of cases is hyperparathyroidism asymptomatic?

A

> 50%

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117
Q

What is the most common joint disorder?

A

Osteoarthritis

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118
Q

What is affected with osteoarthritis?

A

Degeneration of articular cartilage occurs (collagen)

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119
Q

What substance is decreased in OA and what is the result of that reduction?

A

Decreased proteoglycans leading to matrix breakdown

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120
Q

Is OA largely inflammatory?

A

not necessarily

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121
Q

What causes OA?

A

Both mechanical wear and tear from aging along with a genetic influence

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122
Q

Which type of OA occurs without trauma and is common among adults?

A

primary

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123
Q

Which type of OA is insidious in onset and is oligocarticular in nature?

A

primary

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124
Q

Which type of OA is associated with trauma or obesity, deformity, or a systemic disease?

A

secondary

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125
Q

Which type of OA involves a predisposing injury or deformity?

A

secondary

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126
Q

What are the common specific locations for OA?

A

Spine (cervical, lumbar), DIP joints, 1st metacarpal joint, 1st tarsal metatarsal joint

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127
Q

What is the most common location for OA in females?

A

knees and hands

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128
Q

What is the most common location for OA in males?

A

hips

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129
Q

What occurs to the intervertebral discs and is seen on X-ray during OA?

A

Dehydration of discs

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130
Q

When can inflammation in OA develop?

A

After full blown degeneration

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131
Q

What are the functions of articular cartilage?

A

Decrease friction and shock allow load absorption

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132
Q

OA disrupts the function of what cells?

A

chondrocytes

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133
Q

What are risks for OA?

A

Mechanical stress, age 50s-60s, genetics, increased bone density, increased circulating estrogen

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134
Q

Insidious onset, deep/achy pain, crepitus, decreased range of motion, and worse pain the morning are all associated with what joint condition?

A

OA

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135
Q

What affect can osteophytes in OA have on nerves?

A

possible impingement on nerve rootlets

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136
Q

What is the treatment for OA?

A

Palliative: ice, heat, NSAIDs, adjust, joint replacement

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137
Q

What is ankylosis?

A

Abnormal fusion of joints

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138
Q

Is ankylosis present in OA?

A

no

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139
Q

Is ankylosis present in rheumatoid arthritis?

A

yes

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140
Q

OA is more common among which gender?

A

females

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141
Q

What is the name for the enlargement of the DIP joints?

A

Heberden’s nodes

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142
Q

What is the name for the visual enlargement of the PIP joints?

A

Bouchard’s nodes

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143
Q

Which joint disease is a systemic, chronic autoimmune disease?

A

RA

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144
Q

RA results in what effect on the joints?

A

Increased collagenase and osteoclast activity

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145
Q

The biomarker, HLA-DRB1, is a risk factor for what joint issue?

A

RA

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146
Q

What two things are commonly tested for when checking for RA?

A

Anti-CCP antibodies and rheumatoid factor (RF)

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147
Q

In 70% of cases, RA patients test positive for what?

A

anti-CCP antibodies

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148
Q

What is another cause of testing positive for anti-CCP antibodies?

A

inflammation due to smoking or infection

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149
Q

80% of people, with RA will test positive for what test?

A

rheumatoid factor (RF)

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150
Q

What are the most common specific locations for RA?

A

MCP and PIP joints (Bouchard’s nodes)

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151
Q

What is the name for the visual swelling in the fingers seen with RA?

A

Fusiform swelling

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152
Q

What is significant about the patients with RA and their C1/C2 region?

A

30% demonstrate atlanto-axial instability

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153
Q

Pannus is associated with what joint condition?

A

RA

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154
Q

Swan neck deformity and ulnar deviation are associated with what joint condition?

A

RA

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155
Q

Which joint condition has relatively symmetric defects?

A

RA

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156
Q

What is a condition that is similar to RA but shows up negative for RF and positive for HLA-B27 (seronegative)?

A

Seronegative spondyloarthropathies

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157
Q

What is the unique location for the inflammation seen with seronegative spondyloarthropathies?

A

Sacroiliitis, spinal ligaments (syndesmophytes)

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158
Q

What are the categories of seronegative arthropathies?

A

Psoriatic arthritis (psoriasis), enteropathic arthritis (IBD), ankylosing spondylitis, reactive arthritis

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159
Q

What is the most common diagnosis when a negative test for RF is seen?

A

Juvenile rheumatoid arthritis

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160
Q

What gender is more likely to develop juvenile rheumatoid arthritis?

A

females

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161
Q

What is the most common age for juvenile rheumatoid arthritis to be causing pain?

A

1-6

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162
Q

What is Still’s disease?

A

Spiking fever, rash, pericarditis

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163
Q

In what location does inflammation typically occur with juvenile rheumatoid arthritis?

A

Large joints like the knee and hip leading to dysfunction

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164
Q

What is the other name for ankylosing spondylitis (AS)?

A

Marie-Strumpell disease

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165
Q

What part of the skeleton is affected by ankylosing spondylitis?

A

Axial skeleton

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166
Q

Where do you see referred pain with ankylosing spondylitis?

A

gluteal region

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167
Q

What is the actual pain in ankylosing spondylitis?

A

Sacro iliac joint

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168
Q

Bamboo spine is associated with what joint condition?

A

Ankylosing spondylitis

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169
Q

What is the typical onset age for ankylosing spondylitis?

A

before age 40

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170
Q

Characteristic morning stiffness after thirty minutes of being awake is associated with what joint condition?

A

Ankylosing spondylitis

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171
Q

Nocturnal low back pain that is unrelieved when lying down is associated with what joint condition?

A

Ankylosing spondylitis

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172
Q

95% of ankylosing spondylitis cases test positive for what test?

A

HLA-B27

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173
Q

Where is a secondary location for ankylosis in patients with ankylosing spondylitis?

A

rib joints

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174
Q

Syndesmophytes are indicative of what general joint condition?

A

Seronegative spondyloarthropathies

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175
Q

Trolley track sign and dagger sign on X-ray is indicative of what condition?

A

Ankylosing spondylitis

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176
Q

What are the effects of ankylosing spondylitis?

A

Decreased flexion and lordosis leaded to a hunched appearance

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177
Q

What condition is an abnormal purine metabolism leading to uric acid accumulation?

A

gout

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178
Q

Red meat and alcohol can be risk factors for what condition due to purine emphasis?

A

gout

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179
Q

When uric acid accumulates in gout, what do they become?

A

Monosodium urate crystals (tophus/tophi)

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180
Q

What occurs with gout?

A

Recurrent acute joint inflammation leading to destruction and fibrosis

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181
Q

Over 90% of the cases of gout is what kind?

A

Primary - hyperuricemia

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182
Q

What is the hypothesized cause of primary gout?

A

enzyme defects

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183
Q

With primary gout, what accumulates in the blood?

A

uric acid

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184
Q

What is secondary gout?

A

Gout that is secondary to another disease or from drug exposure

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185
Q

What are examples of some causes of secondary gout?

A

Renal disease leukemia, multiple myeloma, diuretics

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186
Q

What is the cause of the inflammation of gout?

A

failed crystal phagocytosis

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187
Q

What is the usual age of those with gout?

A

over 30

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188
Q

Gout usually occurs among which gender?

A

males

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189
Q

What is the most common location for gout inflammation?

A

Hallux (MTP) aka podagra

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190
Q

About what percent of the population is affected by gout?

A

1%

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191
Q

The historical name, “Kings Disease”, is associated with what condition?

A

gout

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192
Q

Monoarticular inflammation is characteristic of what condition?

A

gout

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193
Q

What is the first stage of gout?

A

Asymptomatic hyperuricemia

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194
Q

What is the second stage of gout?

A

Acute gouty arthritis causing erythema

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195
Q

What is the third stage of gout?

A

Repeated chronic tophaceous arthritis that fails to resolve itself between cycles

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196
Q

20% of those with gout die from what issue?

A

renal failure

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197
Q

What are other names for pseudogout?

A

Chondrocalcinosis or Calcium Pyrophosphate Deposition Disease (CPDD)

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198
Q

What causes pseudogout?

A

Altered pyrophosphate metabolism

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199
Q

Are most cases of pseudogout inherited?

A

no most commonly sporadic

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200
Q

What is the usual location for pyrophosphate crystal deposition of pseudogout?

A

Knee, wrist, shoulder, hip, elbow, ankle

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201
Q

Only about how many cases of pseudogout lead to destruction of joints from inflammation?

A

50%

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202
Q

After what age is pseudogout seen among half of the population?

A

over the age of 85

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203
Q

What is the usual onset age for pseudogout?

A

over 50

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204
Q

What is the treatment for pseudogout?

A

Palliative medicine (NSAIDs or corticosteroids)

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205
Q

What specific location in the wrist is common for pseudogout to occur?

A

Triangular fibrocartilage complex

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206
Q

What is hydroxyapatite deposition disease usually called?

A

calcific tendonitis

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207
Q

What is the sign of calcific tendinitis on X-ray?

A

Soft tissue opacities from ossification

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208
Q

What is the most common location for calcific tendinitis?

A

rotator cuff muscles

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209
Q

What is the age of onset for calcific tendinitis?

A

40-70

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210
Q

What is the most common muscle affected by calcific tendinitis?

A

supraspinatus

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211
Q

Adhesive capsulitis (frozen shoulder) and increased age are risk factors for what condition?

A

calcific tendonitis

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212
Q

What is the other name for diffuse idiopathic skeletal hyperostosis?

A

Forestier disease

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213
Q

What ligament is affected in the cervical and thoracic spine in DISH?

A

anterior longitudinal ligament (ALL)

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214
Q

What is the usual spinal location for DISH?

A

T7-T11

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215
Q

What is the most common age for DISH?

A

50-70

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216
Q

What are risks for DISH?

A

Diabetes and HLA-B27

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217
Q

What is the most common cause of infectious arthritis?

A

bacteria

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218
Q

What occurs with infectious arthritis?

A

Rapid joint destructions within days

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219
Q

What is the treatment for infectious arthritis?

A

Based on microbial agent: IV antibiotics and analgesics

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220
Q

What is the name of the pus forming infectious arthritis?

A

Suppurative arthritis

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221
Q

What is the most common location for suppurative arthritis?

A

knee

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222
Q

90% of suppurative arthritis involve how many joints?

A

one

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223
Q

Which microbe is the most common agent involved with suppurative arthritis among children under 2 years of age?

A

Haemophilus influenzae

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224
Q

Which microbe is the most common agent involved in suppurative arthritis among children older than 2 years of age and adults?

A

Staphylococcus aureus

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225
Q

Which microbe is the most common agent involved in adolescents and young adults?

A

Neisseria gonorrhoeae

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226
Q

Who is at a higher risk for neisseria gonorrhoeae causing infectious arthritis?

A

sexually active females

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227
Q

What is the most common microbe at fault for infectious arthritis among those with sickle cell anemia?

A

salmonella

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228
Q

Are primary joint neoplasms rare or common?

A

rare and most commonly benign

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229
Q

Which is more common: tumor like lesions or neoplasia in joints?

A

tumor like lesions

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230
Q

What makes up a ganglion?

A

degenerative CT

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231
Q

What is the name of a synovial cyst in the popliteal region?

A

bakers cyst

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232
Q

What is the difference between a ganglion and a synovial cyst?

A

Communication with the synovium is present in synovial cysts only

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233
Q

What is the typical age for tenosynovial giant cell tumors?

A

20-50 years old

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234
Q

What joint condition involves an aggressive benign tumor?

A

Tenosynovial giant cell tumor (TGCT)

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235
Q

What is the type of TGCT that is larger?

A

Diffuse TGCT (pigmented villonodular synovitis)

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236
Q

Which type of TGCT is smaller and involved with the tendon sheaths?

A

Localized TGCT

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237
Q

What is the common location for diffuse TGCT?

A

knee

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238
Q

What is the common location for localized TGCT?

A

hand

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239
Q

What is the most common soft tissue tumor of the hand?

A

localized TGCT

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240
Q

Tumors of adipose, fibrous, muscle, vessels, or the PNS are what kind of tumors?

A

Soft tissue tumors

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241
Q

Are soft tissue tumors usually benign or malignant?

A

benign (100X)

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242
Q

If a soft tissue tumor is malignant, what is it called?

A

soft tissue sarcoma

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243
Q

What is the most common area for a soft tissue tumor or sarcoma?

A

thigh

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244
Q

What is the exception for a location where soft tissue tumors are more likely to be malignant than benign?

A

skeletal muscle

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245
Q

What is a malignant tumor of adipocytes?

A

liposarcoma

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246
Q

What is the most common type of soft tissue tumor of adults?

A

lipoma

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247
Q

What type of soft tissue tumor is likely to be encapsulated?

A

lipoma

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248
Q

What is the usual age range for liposarcomas?

A

40-50

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249
Q

What is the location for possible metastasis for a liposarcoma?

A

lungs

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250
Q

What is the usual location for liposarcomas?

A

Retroperitoneum, thigh

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251
Q

What is the genetic factor of liposarcomas?

A

Degraded p53 resulting from a translocation of chromosomes 12 and 16

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252
Q

What is the term for a reactive fibroblastic mass?

A

Nodular fasciitis

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253
Q

What is the usual location for a nodular fasciitis?

A

Volar (Palm-side) of arm, chest, back

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254
Q

Nodular fasciitis tends to result from what kind of event?

A

trauma (10-15%)

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255
Q

What is the term for reactive metaplastic bone?

A

Myositis ossificans

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256
Q

What is the most common location for myositis ossificans?

A

proximal extremity muscles

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257
Q

A hard, painless mass is characteristic of what kind of fibrous tumor like mass?

A

Myositis ossificans

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258
Q

What condition should you attempt to rule out when suspicious of myositis ossificans due to their similarities in appearance?

A

osteosarcoma

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259
Q

Myositis ossificans is common among what group of people?

A

adolescent athletes

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260
Q

What type of fibrous like tumor mass is benign but locally invasive that tends to commonly recur?

A

fibromatosis

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261
Q

Dupuytren contracture and Peyronie disease are examples of what kind of fibromatosis?

A

superficial

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262
Q

What is the most common age for superficial fibromatosis?

A

40-70

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263
Q

What another term for deep fibromatosis?

A

desmoid tumor

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264
Q

Where are desmoid tumors or deep fibromatoses most commonly found?

A

abdominal area

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265
Q

Which type of fibromatosis is associated with Gardner Syndrome?

A

deep

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266
Q

What is the term for a slow growing, malignant fibrous like tumor mass?

A

fibrosarcoma

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267
Q

What are the usual locations for fibrosarcomas?

A

Thigh, retroperitoneum, knee

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268
Q

What is the most common age for fibrosarcomas?

A

35-55

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269
Q

Neoplastic fibroblasts and anaplastic spindle cells are associated with what kind of fibrous like tumor mass?

A

fibrosarcomas

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270
Q

25% of fibrosarcomas metastasize hematogenously to what location, most commonly,

A

lungs

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271
Q

Herringbone pattern is associated with what fibrous like mass tumor?

A

fibrosarcomas

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272
Q

What are skeletal muscle precursors?

A

Rhabdomyoblasts

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273
Q

What is the term for a malignant and aggressive skeletal muscle tumor?

A

Rhabdomyosarcoma

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274
Q

What is the most common location generally and specifically for rhabdomyosarcomas?

A

Generally in areas with little skeletal muscle. Specifically in the areas of the head and neck

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275
Q

What is the most common pediatric soft tissue sarcoma?

A

Rhabdomyosarcoma

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276
Q

What changes with the prognosis for adults with rhabdomyosarcomas?

A

Poor prognosis (2/3 of pediatrics will be 100% cured)

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277
Q

What is the term for a benign smooth muscle tumor?

A

leiomyoma

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278
Q

What is the term for a malignant smooth muscle tumor?

A

Leiomyosarcoma

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279
Q

What is the most common location for a leiomyoma?

A

uterus (often called fibromas)

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280
Q

What population is more at risk for leiomyosarcomas?

A

Adult females

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281
Q

What is a unique common area for leiomyosarcomas?

A

inferior vena cava

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282
Q

Spindle cells and cigar shaped nuclei are associated with what kind of smooth muscle tumor?

A

Leiomyosarcoma

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283
Q

What makes up 10% of all soft tissue sarcomas?

A

Synovial sarcomas

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284
Q

What is the most common age range for synovial sarcomas?

A

20-40

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285
Q

What is the unique pattern of growth associated with synovial sarcomas?

A

Cells grow in solid cords or aggregates

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286
Q

What is the most common location for a synovial sarcoma?

A

knee

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287
Q

What is the treatment for synovial sarcomas?

A

Limb-sparing surgery and chemotherapy

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288
Q

What is the usual survivability for those with synovial sarcomas?

A

10 year survivability of 10-30%

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289
Q

Can stress fractures be visible on X-rays?

A

no microfractures occur

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290
Q

What causes the bony necrosis with AVN?

A

ischemia

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291
Q

What is the most common cause of AVN?

A

vascular disruption via fracture

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292
Q

What is the most common form of osteomyelitis?

A

acute

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293
Q

What condition is known as “bone-marrow-inflammation”?

A

Osteomyelitis

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294
Q

What is specifically infected in osteomyelitis?

A

bone marrow

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295
Q

What are signs and symptoms of osteomyelitis?

A

Acute fever, intense malaise, throbbing pain

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296
Q

What is usually the bacterial cause of osteomyelitis?

A

pyogenic bacteria

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297
Q

What is the most common pyogenic bacteria to cause osteomyelitis?

A

Staph aureus

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298
Q

Possible collapse with AVN creates a risk for what condition?

A

OA

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299
Q

What is another name for tuberculous osteomyelitis?

A

skeletal TB

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300
Q

What is the most common form of spread in tuberculous osteomyelitis?

A

Hematogenous (other form would be lymphatics)

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301
Q

Which is more common: secondary or primary bone tumors?

A

secondary

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302
Q

What six factors indicate a larger risk of a bone tumor being the cause of low back pain?

A

50 years of age or older, history of cancer, unexplained weight loss, failure to improve after a month of conservative care, unrelieved with bed rest, and pain duration longer than one month

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303
Q

What category of fibrous dysplasia is most commonly represented?

A

Monostotic (70%)

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304
Q

What category of fibrous dysplasia is asymptomatic and results in minimal bony distortion or enlargement?

A

monostotic

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305
Q

When does polystotic fibrous dysplasia typically develop?

A

Late childhood and adolescence (earlier than monostotic)

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306
Q

Craniofacial involvement is present in about half of the cases of which category of fibrous dysplasia?

A

polystotic

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307
Q

What is the metastatic pathway of sarcomas?

A

Hematogenous

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308
Q

What is the metastatic pathway of carcinomas?

A

lymphatic

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309
Q

What is the most common type of secondary metastasis to bone?

A

Mixed- both osteoblastic and osteolytic

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310
Q

What are the most common forms of secondary metastasis to bone in adults?

A

Prostate (blastic), breast (lytic), lung

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311
Q

In pyogenic osteomyelitis, what is the reactive woven/lamellar bone that surrounds the infected bone called?

A

Involucrum

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312
Q

In pyogenic osteomyelitis, what is that dead bone seen at the site of infection?

A

Sequestrum

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313
Q

In pyogenic osteomyelitis, what is the ruptured periosteum that leads to an abscess in the surrounding soft tissue?

A

Draining sinus

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314
Q

What is fibrous dysplasia?

A

Arrested development of bone resulting in a benign lesion (failure of bone differentiation)

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315
Q

McCune-Albright syndrome is part of what category of fibrous dysplasia?

A

Polystotic + café-au-lait spots & endocrinopathy

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316
Q

What is the age range of monostotic fibrous dysplasia?

A

10-30

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317
Q

The “ground glass” appearance on X-ray is characteristic of what bone condition?

A

Fibrous dysplasia

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318
Q

How can you distinguish Paget’s disease from fibrous dysplasia on X-ray?

A

Fibrous dysplasia tends to be focused on the proximal region of the bone, while Paget’s disease is throughout the entire bone

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319
Q

What is the treatment for fibrous dysplasia?

A

Excision, othropedic hardware

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320
Q

McCune-Albright syndrome is more common among what gender?

A

females

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321
Q

What types of endocrine hyperfunction is associated with McCune-Albright syndrome?

A

Hyperthyroidism, pituitary adenoma, and adrenal hyperplasia

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322
Q

Are the skin and bony lesions of McCune-Albright Syndrome commonly unilateral or bilateral?

A

unilateral

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323
Q

What is PNET?

A

Primitive neuroectodermal tumor

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324
Q

What causes Ewing sarcoma or PNET?

A

Translocations of genetic bits of information

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325
Q

“Small round cell tumors of bone” is associated with what bone condition?

A

ewing sarcoma or PNET

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326
Q

What is the appearance of the cells involved with an Ewing sarcoma?

A

highly primitive and undifferentiated

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327
Q

What is the second most common pediatric bone cancer (#1 osteosarcoma)?

A

Ewing sarcoma

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328
Q

What is the difference between Ewing sarcoma and PNET?

A

PNET has some neural differentiation while Ewing sarcoma is virtually completely undifferentiated

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329
Q

What is the most common age range of Ewing sarcoma and PNET?

A

10-20

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330
Q

What race is more likely to be affected by Ewing sarcoma or PNET?

A

caucasians (9X)

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331
Q

What gender is at a greater risk for Ewing sarcoma and PNET?

A

males

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332
Q

What is the most common location of bone for Ewing sarcoma or PNET?

A

femur

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333
Q

What effect does an Ewing sarcoma or PNET mimic?

A

That of infection: pyrexia, leukocytosis, increased erythrocyte sedimentation rate

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334
Q

What is the most common mode of infection with osteomyelitis?

A

Hematogenous (sepsis)

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335
Q

“Onion-skinning” appearance of the periosteum is associated with what bone condition?

A

ewing sarcoma

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336
Q

What percentage of patients with Ewing sarcoma or PNET end up with complete remission?

A

50%

337
Q

Homer-Wright pseudo rosettes are associated with what bone condition?

A

PNET

338
Q

What is the age range for giant-cell tumors of bone (GCT)?

A

20-40

339
Q

What is the most common location for giant-cell tumors of bone?

A

knee

340
Q

What type of bone condition produces arthritis like pain, is osteolytic, and is painful?

A

giant cell tumor of bone

341
Q

What is the most common location of metastasis for a giant-cell tumor of bone, although it rarely metastasizes?

A

lungs

342
Q

The “soap bubble” appearance on X-ray is associated with which bone condition?

A

giant cell tumor of bone

343
Q

What part of the skeleton is the most common location for secondary metastasis of bone?

A

axial skeleton

344
Q

What destroys the bone in osteomyelitis?

A

Leukocytes

345
Q

What are other modes of infection for osteomyelitis besides the most common one?

A

Adjacent infection or traumatic implantation

346
Q

What is the common location of chondrosarcomas?

A

Intramedullary

347
Q

What are the usual osseous locations of chondrosarcomas?

A

Large joints like the pelvis, shoulder, ribs, and proximal femur

348
Q

Which type of cartilage forming tumor creates an expansile glistening mass?

A

chondrosarcoma

349
Q

What is the usual age range for osteomalacia (benign)?

A

40-50

350
Q

What effect can chondrosarcomas have on the cortex?

A

it may thicken or erode it

351
Q

Which type of chondrosarcoma is more common?

A

low grade

352
Q

Where do high grade chondrosarcomas tend to metastasize the most?

A

lungs

353
Q

What is the survival rate of a high grade chondrosarcoma?

A

40%

354
Q

What percentage of high grade chondrosarcomas metastasize?

A

70%

355
Q

At what size is an chondrosarcoma indicative of a poor prognosis and being extremely fatal?

A

> 10 cm

356
Q

What is the treatment for chondrosarcomas?

A

wide excision and chemo

357
Q

What does exophytic mean?

A

growing out, not growing in

358
Q

What is the usual specific location of osteoid osteomas?

A

Near cortex of long bones like the femur and tibia

359
Q

What is the most common location of chondroma development?

A

medullary cavity

360
Q

What is the typical age range for a chondroma?

A

20-50

361
Q

Which is more common: chondrosarcoma or osteosarcoma?

A

osteosaroma

362
Q

What is the usual age range of chondrosarcomas?

A

40-60

363
Q

What gender is more commonly affected by chondrosarcomas?

A

males (2X)

364
Q

What type of bone tumors are the most common?

A

matrix and fibrous producing tumors

365
Q

In regards to bone tumors, are benign or malignant tumors more common?

A

benign

366
Q

What are the two most common benign bone tumors?

A

Osteochondroma, fibrous cortical defects

367
Q

What are the most common primary bone cancers?

A

Osteosarcoma, chondrosarcoma, Ewing sarcoma

368
Q

What population has an increased risk of bone cancer?

A

elderly

369
Q

What bones are usually affected by bone tumors?

A

long bones of the extremities

370
Q

How are bone tumors diagnosed?

A

incidental or pathological fracture

371
Q

What is usually the age range for osteosarcomas?

A

10-20

372
Q

What is the usual location of osteosarcomas?

A

knee

373
Q

What is the usual location of osteomas?

A

Facial bones, skull (frontal bone)

374
Q

How are bone forming tumors usually diagnosed?

A

biopsy

375
Q

What are the main types of bone forming tumors?

A

Osteoma, osteoid osteoma & osteoblastoma, osteosarcoma

376
Q

Which type of bone forming tumor is now said to be a developmental anomaly or reactive growth following trauma?

A

osteoma

377
Q

Which type of bone forming tumor creates matter very similar to normal bone?

A

osteoma

378
Q

Which type of bone forming tumor is completely non-invasive and benign?

A

osteoma

379
Q

Who is at a greater risk for osteoid osteomas/osteoblastomas?

A

males (2X)

380
Q

Which type of bone forming tumor is smaller and found in the extremities?

A

osteoid osteoma

381
Q

Which type of bone forming tumor is large and found in the spine?

A

osteoblastoma

382
Q

What age range is usually affected by osteoid osteomas and osteoblastomas?

A

10-20

383
Q

What is the middle part of the oval lesion with an osteoid osteoma or osteoblastoma?

A

Central “nidus” which is radiolucent

384
Q

What surrounds the central nidus in the lesions involved with osteoid osteomas and osteoblastomas?

A

Rim of sclerosis

385
Q

What is a common complaint and feature of osteoid osteomas and osteoblastomas?

A

Localized nocturnal pain

386
Q

What is the unique method of relief for osteoid osteomas?

A

aspirin

387
Q

What is the size of osteoblastomas?

A

2-6 cm

388
Q

What is the specific location of osteoblastomas?

A

Spinous and transverse processes of vertebrae

389
Q

What is the treatment for osteoblastomas?

A

Excision, possible irradiation

390
Q

Which is associated with mild pain that is unrelieved by aspirin: osteoid osteoma or osteoblastoma?

A

osteoblastomas

391
Q

Which type of bone forming tumor is aggressively malignant?

A

osteosarcoma

392
Q

What is the most common primary bone cancer?

A

osteosarcoma

393
Q

How do osteosarcomas usually metastasize?

A

Hematogenously

394
Q

Who is at a larger risk for osteosarcomas?

A

adolescent males (1.5X)

395
Q

What age range makes up 75% of osteosarcoma cases?

A

10-20

396
Q

What is usually the cause of osteosarcomas among those over the age of forty?

A

Co-morbid bone pathology like Paget disease, AVN, or irradiation

397
Q

What X-ray feature is a red flag for osteosarcomas?

A

codman triangle

398
Q

What is the usual location of an osteosarcoma among adults over 40?

A

femur, humerus and pelvis

399
Q

What is the usual location of an osteosarcoma among adolescents?

A

Knee area: metaphysis of distal femur, proximal tibia, and humerus too

400
Q

What is the end result of the Codman triangle as seen with osteosarcomas?

A

Periosteal cortex destroyed resulting in “starburst” appearance on X-ray

401
Q

What is a big risk with osteosarcomas?

A

pathological fractures

402
Q

What is the most common location of metastasis of an osteosarcoma?

A

lungs (10-20%)

403
Q

What genetic issue leads to a 1000 times increased risk of developing an osteosarcoma?

A

Retinoblastoma syndrome

404
Q

Are cartilage forming tumors most commonly benign or malignant?

A

benign

405
Q

What types of cartilage are formed in cartilage forming tumors?

A

Hyaline and myxoid

406
Q

What are the three main types of cartilage forming tumors?

A

Osteochondroma, chondroma, chondrosarcoma

407
Q

What is another name for osteochondroma?

A

Exostosis

408
Q

What type of cartilage forms with an osteochondroma?

A

hyaline

409
Q

Which gender is at a greater risk for an osteochondroma?

A

males (3X)

410
Q

What is the age range for osteochondromas?

A

10-30 years

411
Q

What is the most common way osteochondromas are developed?

A

solitary or isolated incidents

412
Q

Where do osteochondromas usually originate?

A

Near the growth plates on the metaphysis of long bones

413
Q

What is the most common location of osteochondromas?

A

knee

414
Q

What is the usual size of osteochondromas?

A

1-20cm

415
Q

Osteochondromas rarely occur after what time period?

A

skeletal maturity

416
Q

What is another term commonly used for chondroma?

A

enchondroma

417
Q

What cartilage type forms with a chondroma?

A

hyaline

418
Q

Where does the cartilage with chondromas develop specifically on the skeleton?

A

Metaphysis of small tubular bones of the hands and feet

419
Q

What is the name of the condition where multiple lesions of chondromas occur?

A

Ollier disease (enchondromatosis or multiple enchondromas)

420
Q

The “o-ring” sign of sclerosis is associated with which type of cartilage forming tumor?

A

Chondromas

421
Q

Are chondromas typically asymptomatic or symptomatic?

A

asymptomatic

422
Q

Chondromas make up what percentage of all benign bone tumors?

A

10%

423
Q

Solitary chondromas of the hands are usually seen where?

A

proximal phalanges

424
Q

What makes up the motor unit?

A

Lower motor neuron/peripheral axon, neuromuscular junction, innervated myocytes

425
Q

What is the parenchyma of the PNS?

A

neuron

426
Q

What makes the myelin in the PNS?

A

Schwann cells

427
Q

What kind of peripheral neuropathy involves direct injury to the axon and degeneration of peripheral segments?

A

Axonal neuropathy

428
Q

What is the secondary loss of myelin called with axonal neuropathy?

A

Wallerian degeneration

429
Q

After repair of axonal neuropathy, how is the axon different from before?

A

Decreased axon density and overall amplitude is decreased, as well

430
Q

What kind of peripheral nerve injury involves damage to Schwann cells or myelin?

A

Demyelinating neuropathy

431
Q

What is the pattern of destruction for demyelinating neuropathy?

A

Random internode demyelination (segmental demyelination)

432
Q

How is the myelin different after repair of demyelinating neuropathy?

A

Thinner myelin and shorter internodes leading to slowed nerve conduction velocity (NCV)

433
Q

A shoulder dislocation can lead to which mononeuropathy?

A

axillary nerve palsy

434
Q

Carpal tunnel syndrome can lead to which mononeuropathy?

A

thenar atrophy

435
Q

What two things can cause mononeuropathies?

A

entrapment or trauma

436
Q

Which axons are more at risk to be affected by polyneuropathies?

A

Distal segments of long axons

437
Q

Stocking and glove paresthesia is seen among what kind of peripheral nerve injury?

A

polyneuropathies

438
Q

What kind of peripheral nerve injury damages random portions of individual nerves?

A

Polyneuritis multiplex

439
Q

What type of peripheral nerve injury is common among autoimmune diseases?

A

Polyneuritis multiplex

440
Q

What are various categories of causes of peripheral nerve injury?

A

Nutritional, toxic, vascular, inflammatory, genetic

441
Q

What is the effect of Guillain-Barré syndrome on the nervous system?

A

Acute motor neuron demyelination

442
Q

What is unique about the paralysis of Guillain-Barré syndrome?

A

Ascending paralysis leading to “rubbery legs”

443
Q

How can Guillain-Barré syndrome be lethal?

A

respiratory failure

444
Q

What reflex is decreased with Guillain-Barré syndrome?

A

deep tendon reflexes

445
Q

Why is Guillain-Barré syndrome considered an auto immune disease?

A

Macrophages are seen near the nerve roots

446
Q

What is the cause of Guillain-Barré syndrome?

A

60% the cause is unknown, but the remaining 40% are due to viruses such as C. jejuni, EBV, CMV, HIV (most of these except for HIV are minor viruses)

447
Q

What is unique about the age range likelihood for diagnoses of Guillain-Barré syndrome?

A

Biphasic distribution (males ages 15-35 and 50-57)

448
Q

What is the treatment for Guillain-Barré syndrome?

A

Ventilation, plasmapheresis, IV antibodies

449
Q

What is CIDP?

A

Chronic inflammatory demyelinating polyneuropathy

450
Q

What is essentially the chronic and relapsing version of Guillain-Barré syndrome?

A

Chronic inflammatory demyelinating polyneuropathy (CIDP)

451
Q

CIDP is associated with which immune disorders?

A

Lupus, HIV

452
Q

What are symptoms for CIDP?

A

Pain, paresthesia, ataxia, decreased deep tendon reflexes

453
Q

What is the #1 cause of peripheral neuropathy?

A

Diabetic peripheral neuropathy

454
Q

Vascular tissue injury as a result from hyperglycemia ultimately leading to nerve damage is associated with what cause of peripheral nerve injury?

A

Diabetic peripheral neuropathy

455
Q

What is the name of the blood vessels that are damaged in diabetic peripheral neuropathy?

A

Vasa nervorum

456
Q

What is the genetic cause of periphery nerve injury?

A

Charcot-Marie-Tooth disease

457
Q

Charcot-Marie-Tooth disease is most common amount what population?

A

young adults

458
Q

Extreme pes cavus is seen with what cause of peripheral nerve injury?

A

Charcot-Marie-Tooth disease

459
Q

What is the pathway for myofiber depolarization?

A

Pre synaptic calcium influx —> acetylcholine release —> post synaptic ACh receptor

460
Q

What neuromuscular junction disorder involves the degradation of post synaptic ACh receptors disabling the neuronal impulse?

A

Myasthenia gravis

461
Q

What effects of the thymus can be see with myasthenia gravis?

A

Hyperplasia (60%) or a benign thymoma (20%)

462
Q

What is the result of thymic lesions from myasthenia gravis on the individual cells?

A

Autoreactive T cells and B cells

463
Q

What is the hallmark sign for myasthenia gravis?

A

Extraocular weakness, diplopia, and ptosis

464
Q

What gender is more likely to be affected by myasthenia gravis?

A

females

465
Q

What is the typical age range for females acquiring myasthenia gravis?

A

20-30

466
Q

What is the typical age range for males acquiring myasthenia gravis?

A

50-70

467
Q

What effect does e-stimulation have on those with myasthenia gravis?

A

worsened condition

468
Q

What neuromuscular junction disorder involves the degradation of pre synaptic calcium channels disabling the neuronal impulse?

A

Lambert-Eaton syndrome (LES)

469
Q

What effect does e-stimulation have on Lambert-Eaton syndrome?

A

Improves condition

470
Q

What gender is more likely to have Lambert-Eaton syndrome?

A

females

471
Q

What is the usual age range for Lambert-Eaton syndrome?

A

60

472
Q

Paraneoplastic syndrome or small cell lung cancer is associated with which neuromuscular junction disorder?

A

lambert-eaton syndrome

473
Q

What condition involves increased acetylcholine released to contract the muscles consistently leading to neuromuscular junction disorders?

A

tetanus

474
Q

How do clostridium tetani and botulinum spread to cause infection and release neurotoxins?

A

through soil

475
Q

What condition involves decreased acetylcholine release resulting in descending paralysis?

A

Botulism (clostridium botulinum)

476
Q

Does a peripheral axon innervate just one or multiple myocytes?

A

multiple

477
Q

Which muscle fiber type involves slow twitch movement?

A

type 1

478
Q

Which muscle fiber type involves dark tissue?

A

type 1

479
Q

Which muscle fiber type is associated with fat metabolism and being aerobic?

A

type 1

480
Q

Why do type I muscle fibers appear dark?

A

excess myoglobin

481
Q

Which muscle fiber type is associated with fast twitch movement?

A

type 2

482
Q

Which muscle fiber type appears white?

A

type 2

483
Q

What muscle fiber type is associated with glycogen metabolism and being anaerobic?

A

type 2

484
Q

What determines myofiber type?

A

peripheral axons

485
Q

What is the definition of myopathy?

A

disease of muscular origin, weakness

486
Q

What effect do neuropathic changes have on motor units?

A

They become fewer in number and larger in size

487
Q

Disuse atrophy primarily affects what type of muscle fibers?

A

type 2

488
Q

Glucocorticoid atrophy primarily affects which type of myofiber?

A

type 2

489
Q

What is the most common way to develop glucocorticoid atrophy?

A

Exogenously (endogenously would occur due to something like a pituitary tumor in Cushing’s disease)

490
Q

Static hypotonia is a characteristic of what kind of inherited disorder of skeletal muscle?

A

congenital myopathies

491
Q

Is muscular dystrophy congenital?

A

no

492
Q

Which disorder of skeletal muscle is progressive in nature, inherited, but not congenital?

A

Muscular dystrophy

493
Q

What is the most common cause of muscular dystrophy?

A

Dystrophinopathies (dystrophin gene mutation on the X chromosome)

494
Q

What gender is more likely to be affected by muscular dystrophy?

A

males (x-linked)

495
Q

What occurs with muscular dystrophy?

A

Myocyte degeneration outpaces repair

496
Q

What are the two types of muscular dystrophy?

A

Duchenne and Becker

497
Q

Which type of muscular dystrophy is associated with the complete absence of dystrophin?

A

duchenne

498
Q

Which type of muscular dystrophy is more severe?

A

duchenne

499
Q

What is the age and pattern of progression of Duchenne muscular dystrophy?

A

Weakness by age 5, wheelchair by teens, fatal by young adulthood

500
Q

Which type of muscular dystrophy involves abnormal dystrophin and variable severity?

A

becker

501
Q

Which is more common: Duchenne of Becker muscular dystrophy?

A

duchenne

502
Q

Which type of muscular dystrophy has an adolescent onset and usually a normal life span?

A

becker

503
Q

What specific areas exhibit extreme weakness in those with dystrophinopathies?

A

Pelvic girdle (also possible shoulder girdle weakness in advanced stages)

504
Q

Elevation of what substance seen in a blood test can indicate dystrophinopathies?

A

creatine kinase

505
Q

How do those with dystrophinopathies usually die?

A

Cardiorespiratory insuffiency (specifically cardiomyopathy, arrhythmia, pneumonia

506
Q

Gower’s sign is associated with what condition?

A

muscular dystrophy

507
Q

What is Gower’s sign?

A

Using hands to push on legs “crawling up” on oneself in order to stand

508
Q

In polymyositis, T cells attack what structure to induce inflammation?

A

endomysium

509
Q

What gender and age range are likely to develop polymyositis?

A

males 45-60

510
Q

What is the most common inflammatory myopathy in children?

A

Dermatomyositis

511
Q

Which inflammatory myopathy is also considered a paraneoplastic syndrome in adults?

A

Dermatomyositis

512
Q

What inflammatory myopathy involves tau proteins in the cytoplasm, progressive weakness, wasting, and dysphagia that may lead to degeneration?

A

Inclusion body myositis

513
Q

What is the most common inflammatory myopathy in the elderly (>60)?

A

Inclusion body myositis

514
Q

Which toxic myopathy involves the overproduction of thyroxine (thyrotoxicosis)?

A

Thyrotoxic myopathy

515
Q

Goiter and Graves’ disease are what kind of myopathy?

A

Thyrotoxic myopathy

516
Q

Rhabdomyolysis is associated with what toxic myopathy?

A

ethanol myopathy

517
Q

Binge drinking can result in what toxic myopathy?

A

ethanol myopathy

518
Q

What is the most common cause of drug myopathy?

A

statins

519
Q

Peripheral nerve sheath tumors are most common among what age group?

A

adults

520
Q

What is the issue associated with a benign peripheral nerve sheath tumor?

A

impingement can lead to pain and loss of function

521
Q

Are Schwannomas usually benign or malignant?

A

benign

522
Q

What cranial nerve is most commonly involved with a Schwannoma?

A

VIII (vestibular Schwannoma)

523
Q

Which type of peripheral nerve sheath tumor is 90% sporadic?

A

schwannoma

524
Q

What is Schwannomatosis?

A

Multiple CNS and cutaneous Schwannomas

525
Q

Is cranial nerve VIII also usually affected by Schwannomatosis?

A

no usually avoided

526
Q

What is a neurofibroma?

A

benign and cutaneous nerve sheath tumor

527
Q

When can neurofibromas become a greater issue?

A

When involved in a plexiform area like the brachial or sacral plexus

528
Q

What condition involves bilateral vestibular Schwannomas?

A

Neurofibromatosis type 2 (NF2)

529
Q

What is the genetic pattern of neurofibromatosis type 2?

A

autosomal dominant

530
Q

What types of issues are seen with those with neurofibromatosis type 2?

A

Vision and hearing difficulties (no cutaneous lesions)

531
Q

What condition involves pronounced neurofibromas and vascular stenosis?

A

Neurofibromatosis type 1

532
Q

What are some effects of neurofibromatosis type 1?

A

Decreased condition and seizures

533
Q

What is the genetic pattern of neurofibromatosis type 1?

A

autosomal dominant

534
Q

What sign on the iris is associated with neurofibromatosis type 1?

A

Lisch nodules

535
Q

Axillary freckling, multiple neurofibromas, and cafe au lait spots are associated with what peripheral nerve sheath tumor condition?

A

neurofibromatosis type 1

536
Q

What is the most common location for malignant peripheral nerve sheath tumors?

A

plexiform

537
Q

Malignant nerve sheath tumors are the result of the transformation of what previous condition?

A

Neurofibroma

538
Q

50% of all malignant peripheral nerve sheath tumors arise in patients with what condition?

A

neurofibromatosis type 1

539
Q

What is the common location for traumatic neuroma?

A

Metacarpals and metatarsals

540
Q

Are traumatic neuromas malignant?

A

no

541
Q

What makes up a traumatic neuroma?

A

Schwann cells, axons, and connective tissue

542
Q

Both stroke and Bell palsy cause paralysis of what part of the face?

A

Lower part

543
Q

What part of the face has dual innervation?

A

the upper part

544
Q

In strokes, what ability of the face is maintained that is still paralyzed with Bell palsy making it possible to distinguish the condition?

A

wrinkling the forehead (raising the eyebrows)

545
Q

What type of tissue lacks a lot of collateral blood supply therefore making them more susceptible to ischemia?

A

Deep tissues (like the thalamus, basal ganglia, deep white matter)

546
Q

What is the third leading cause of mortality in the U.S.?

A

Cerebrovascular disease (CVA)

547
Q

What is the most common type of arteriovenous malformation?

A

Cerebrovascular malformation

548
Q

How long does the inflammation of the facial nerve typically last in Bell palsy?

A

2-8 weeks

549
Q

Where can ependymal cells be found?

A

lining ventricles and spinal cord

550
Q

What is the most common age range for Bell palsy?

A

15-45

551
Q

What neural viral infection produces the sign of Negri bodies?

A

rabies

552
Q

What is the treatment for Bell palsy?

A

Facial exercises, eye drops/patch

Corticosteroids, antivirals

553
Q

What neural viral infection produces the sign of “owl’s eye”?

A

Cytomegalovirus

554
Q

Which neurodegenerative disease is associated with neurofibrillary tangles, beta-amyloid plaques, and Tau proteins?

A

Alzheimer’s disease

555
Q

What are two causes of functional hypoxia?

A

Decreased oxygen partial pressure

Decreased oxygen availability

556
Q

What is intracellular inclusion is known known as the aging pigment due to lipid accumulation?

A

Lipofuscin

557
Q

What is disrupted enabling increased permeability with vasogenic edema?

A

blood brain barrier

558
Q

What is the effect of hydrocephalus in those under two years of age?

A

crainial enlargement

559
Q

What is the effect of hydrocephalus in those above two years of age?

A

Increased cranial pressure, ventricular enlargement

560
Q

What is the most common cause of neurological morbidity?

A

Cerebrovascular disease (CVA)

561
Q

Which type of brain herniation involves a displaced cingulate gyrus (located under falx cerebri)?

A

Subfalcine (cingulate)

562
Q

What can result from subfalcine herniation?

A

abnormal posturing, coma

563
Q

What type of brain herniation involves a displaced temporal lobe (under the anterior tentorium)?

A

Transtentorial (uncinate)

564
Q

Which type of brain hemorrhage involves displaced cerebellar tonsils through the foramen magnum?

A

tonsillar

565
Q

What is the more common type of Arnold-Chiari malformation?

A

type 1

566
Q

Which type of Arnold-Chiari malformation is more severe?

A

type 2

567
Q

Type I Arnold-Chiari malformation occurs among what population?

A

adults

568
Q

Type II Arnold-Chiari malformation occurs among what population?

A

infants

569
Q

Which Arnold-Chiari malformation involves the low-lying cerebellar tonsils and downward extension through the foramen magnum?

A

type 1

570
Q

What is the treatment for Arnold-Chiari malformations?

A

Neurosurgery (decompression)

571
Q

Cardiac arrest, shock, and severe hypotension’s effect on blood pressure leads to what condition?

A

Global cerebral ischemia

572
Q

What condition is cause by an arterial occlusion due to localized ischemia?

A

focal cerebral ischemia

573
Q

Neutrophils, red neurons, and edema are seen in what stage of a cerebral infarction?

A

First stage (roughly 12-48 hours)

574
Q

Nuclear fragmentation (karyorrhexis) is seen in what stage of a cerebral infarction?

A

Second stage (rough to 48 hours to 2 weeks)

575
Q

Macrophages and gliosis are most commonly seen in what stage of a cerebral infarction?

A

Third stage (roughly months to years, also cavitation)

576
Q

Ischemic strokes are treated with what kind of medication?

A

thrombolytic

577
Q

What is the condition involving a cerebral microbleed?

A

Primary parenchymal hemorrhage

578
Q

What is the most common age for a primary parenchymal hemorrhage?

A

60

579
Q

What condition is so commonly associated with the phrase from patients, “This is the worst headache I’ve ever had.”?

A

Subarachnoid hemorrhage

580
Q

It is currently hypothesized that what virus is associated with Bell palsy?

A

HSV-1

581
Q

What is the fatality rate of a first episode of a subarachnoid hemorrhage?

A

25-50%

582
Q

A saccular (berry) aneurysm makes up what percentage of all intracranial aneurysms?

A

80-90%

583
Q

Where is the most common location in the circle of Willis for a saccular (berry) aneurysm?

A

Anterior circulation of the circle of Willis

584
Q

What is a lacunar infarct?

A

Occlusion of a single artery resulting from hypertension

585
Q

What diastolic blood pressure is seen with acute hypertensive encephalopathy?

A

> 130 which means there is severe increased intracranial pressure

586
Q

“Red neurons” is a sign present with which type of neuronal injury?

A

irreversible

587
Q

What occurs with reversible neuronal injury?

A

Decreased axonal transport, swelling of soma, displacement of the Nissl substance (central chromatolysis)

588
Q

What occurs with irreversible neuronal injury?

A

Soma shrinkage, pyknosis, eosinophilia, “red neurons”, cerebral edema, loss of nucleolus and Nissl body

589
Q

Which neurodegenerative disease is associated with Lewy bodies?

A

Parkinson’s disease

590
Q

What are the two kinds of cerebral edema?

A

Vasogenic and cytotoxic

591
Q

Which is associated with extracellular edema: vasogenic or cytotoxic edema?

A

vasogenic

592
Q

Which is associated with intracellular edema: vasogenic or cytotoxic edema?

A

cytotoxic

593
Q

What resorbs the CSF produced by the choroid plexus?

A

Arachnoid granulations (villi)

594
Q

What rare condition can cause overproduction of CSF?

A

Choroid plexus tumor

595
Q

What condition involves compensatory hydrocephalus accompanied by infarct and neurodegeneration?

A

Hydrocephalus ex vacuo

596
Q

What is the most common type of brain herniation?

A

Subfalcine (cingulate)

597
Q

Which cranial nerve can suffer from compression from a transtentorial brain herniation, and what is the result?

A

Cranial nerve III (oculomotor) resulting in abnormal vision (“blown pupil”)

598
Q

What is the secondary hemorrhage associated with transtentorial brain herniations?

A

duret hemorrhage

599
Q

What type of hemorrhage is also known as the “flame-shaped” hemorrhage?

A

duret hemorrhage

600
Q

What type of hemorrhage occurs when vessels that enter the pons are disrupted by herniation?

A

duret hemorrhage

601
Q

What are the most common locations of emboli that cause focal cerebral ischemia?

A

Middle meningeal artery and internal carotid artery

602
Q

What is the most common cause of primary parenchymal hemorrhage?

A

hypertension

603
Q

What is the most common cause of a subarachnoid hemorrhage?

A

Ruptured saccular (berry) aneurysm

604
Q

What defect is associated with a ruptured saccular (berry) aneurysm that most commonly causes a subarachnoid hemorrhage?

A

tunica media defect

605
Q

What gender and age group are most at risk for arteriovenous malformation?

A

males 10-30

606
Q

What condition is a systemic autoimmune condition with fibrinoid necrosis involving the small cerebral arteries and heart that results from vasculitis within the CNS?

A

Polyarteriosis nodosa

607
Q

Primary angiitis of the CNS is limited to what locations?

A

brain and spinal cord

608
Q

What is the treatment for primary angiitis of the CNS and polyarteritis nodosa?

A

immunosuppression

609
Q

Neuronophagia is associated with what cells of the CNS?

A

microglia

610
Q

What can cause localized vasogenic edema?

A

tumors, infection, inflammation

611
Q

What can cause generalized vasogenic edema?

A

severe trauma

612
Q

Lethal tonsillar herniation (and eventually respiratory failure) occurs when what condition is left untreated?

A

Hydrocephalus

613
Q

What form of abnormal posturing involves brachial flexion, extension of the legs along with internal rotation?

A

Decorticate rigidity

614
Q

What is the location of injury associated with decorticate rigidity?

A

Between the cortex and red nuclei (midbrain)

615
Q

What form of abnormal posturing involves extension of all 4 limbs, pronation of arms, and plantar flexion?

A

Decerebrate rigidity

616
Q

What condition involves the paralysis of the facial nerve results in a rapid onset of unilateral facial weakness?

A

bell palsy

617
Q

What is the most common cause of focal cerebral ischemia?

A

emboli

618
Q

Where do primary parenchymal hemorrhages tend to occur?

A

Basal ganglia, thalamus, pons, cerebellum

619
Q

What are the usual locations for hypertensive cerebrovascular disease?

A

Basal ganglia, white matter, brainstem

620
Q

What is the most common gender and age group affected by primary angiitis of the CNS?

A

males 30-60

621
Q

What is the location of injury associated with decerebrate rigidity?

A

Between red nuclei & vestibular nuclei (brainstem)

622
Q

What condition is responsible for 50% of post traumatic comas?

A

Diffuse axonal injury

623
Q

What is the term for when the location of injury occurs at the impact site of a cerebral contusion?

A

coup injury

624
Q

Epidural hematoma affect what blood vessels?

A

dural arteries

625
Q

Subdural hematomas affect what blood vessels?

A

veins

626
Q

Which occur more rapidly: epidural or subdural hematomas?

A

epidural hematomas

627
Q

Which is more common: epidural or subdural hematomas?

A

Subdural hematomas (epidural hematomas make up 2% of severe head traumas)

628
Q

What artery is most likely affected by epidural hematomas?

A

middle meningeal artery

629
Q

Which is more of a medical emergency: epidural or subdural hematomas?

A

epidural

630
Q

What is a myelomeningocele?

A

Extension of CNS through vertebral defect leading to lower extremity motor/sensory defects

631
Q

What is the most common location of the spine for myomeningoceles?

A

lumbosacral region

632
Q

What condition develops following trauma to the cerebrum that injures and disrupts vessels leading to hemorrhage?

A

contusion

633
Q

What is the serious issue with epidural abscesses?

A

Can compress the spinal cord (neurosurgical emergency)

634
Q

What is meningitis?

A

Subarachnoid inflammation of leptomeninges

635
Q

What type of bacteria is likely to cause meningitis in young children?

A

Haemophilus influenzae

636
Q

What bacteria is likely to cause meningitis in adolescents and young adults?

A

Neisseria meningitidis

637
Q

What constitutes post-concussion syndrome?

A

Decreased cognition, loss of consciousness, amnesia, nausea, depression, anxiety, irritability, headache, dizziness, photophobia

638
Q

What is the term for when the location of injury occurs at the opposite side of the site of a cerebral contusion?

A

Contrecoup injury

639
Q

What bacteria is likely to cause meningitis among older adults?

A

Strep. pneumoniae, listeria monocytogenes

640
Q

What kind of necrosis can be produced from a bacterial abscess?

A

Liquefaction necrosis

641
Q

What does poliovirus attack?

A

Gray matter of the spinal cord/brainstem

642
Q

What is usually the recovery time period for a concussion?

A

7-10 days without treatment (80% recovery rate)

643
Q

What is the most common disorder of myelin?

A

Multiple sclerosis (MS)

644
Q

Which disorder of myelin is known to be “relapsing-remitting”?

A

MS

645
Q

What is the activity of an active plaque in MS?

A

ongoing myelin breakdown

646
Q

What is the condition of movement of one brain region relative to another region?

A

Diffuse axonal injury (DAI)

647
Q

What kinds of movements can cause diffuse axonal injury?

A

Angular acceleration or shaking

648
Q

What condition is a tearing of cerebral parenchyma that disrupted vasculature that leads to hemorrhage?

A

laceration

649
Q

What is an encephalocele?

A

CNS diverticulum through cranium

650
Q

Type 1 Arnold-Chiari malformation is associated with what condition?

A

Syringomyelia

651
Q

What is syringomyelia (syrinx)?

A

cyst within the spinal cord

652
Q

What is the most common location for diffuse axonal injury?

A

lateral ventricles and brain stem

653
Q

What is the most common way that infections of the nervous system are spread?

A

Hematogenously (other ways = direct implant by trauma, local extension, PNS)

654
Q

What is a subdural empyema?

A

Infection of skull or sinus in the subdural space

655
Q

What makes up the leptomeninges?

A

Arachnoid and pia mater

656
Q

What three signs/symptoms are extremely indicative of acute pyogenic (bacterial) meningitis?

A

Headache, nuchal rigidity, and photophobia

657
Q

What bacteria are likely to cause meningitis in neonates?

A

E. Coli and group B strep (strep agalactiae)

658
Q

What is affected with MS?

A

White matter (demyelination)

659
Q

What vision ailment is commonly seen with MS?

A

Diplopia (unilateral vision impairments leading to double vision)

660
Q

What are some signs and symptoms of MS?

A

Cranial nerve dysfunction, ataxia, motor/sensory impairments, bowel/bladder dysfunction, sexual dysfunction, seizures, decreased cognition, depression

661
Q

Deficiency in what substance associated with CNS issues like Wernicke-Korsakoff syndrome, confusion, memory, ataxia, chronic alcoholics, chronic gastritis?

A

Thiamine (B1)

662
Q

Necrosis and macrophages in the thalamus can be seen with what kind of deficiency?

A

Thiamine (B1)

663
Q

What are the effects of hypoglycemia?

A

Mimics global hypoxia (edema)

664
Q

What part of the brain is susceptible to injury with hypoglycemia?

A

Hippocampus

665
Q

What are the effects of hyperglycemia?

A

Hyperosmolar state, confusion, stupor, coma, glycosuria, decreased water

666
Q

What condition involves the extension of the fourth ventricle?

A

Hydromyelia

667
Q

What location of the cerebrum are more susceptible to a brain contusion?

A

Gyri of the frontal and temporal lobes

668
Q

What kind of white blood cells are seen with viral encephalitis?

A

Mononuclear (monocytes, macrophages)

669
Q

What are the signs and symptoms of polio?

A

Gastritis, motor neuron damage leading to wasting and flaccidity

670
Q

What is the cause of the myelin deterioration in MS?

A

Autoimmune inflammatory attack from lymphocytes and macrophages resulting in white matter lesions (plaques)

671
Q

Increased vascular resistance can lead to what vascular condition?

A

hypertension

672
Q

What is malignant hypertension?

A

Around 200/120 BP (5% of all hypertension cases) which is usually lethal within 1-2 years

673
Q

How can malignant hypertension be detected early on?

A

Papilledema or retinal hemorrhage

674
Q

What are the steps of vascular wall responses to injury?

A
  1. Endothelial injury/dysfunction
  2. Smooth muscle cell recruitment
  3. Growth of smooth muscle cell & ECM
  4. Irreversible intimal thickening
675
Q

What can irreversible intimal thickening as a result of vascular injury lead to?

A

Vessel stenosis (tissue ischemia)

676
Q

What is the term for hardening of the arterioles?

A

ArterioLOsclerosis

677
Q

What is term for the hardening of arteries?

A

Arteriosclerosis

678
Q

What condition involves the calcification of the tunica media?

A

Monckeberg medial sclerosis

679
Q

What is the usual age range of Monckeberg medial sclerosis?

A

> 50 years old

680
Q

What is the most common form of arteriosclerosis?

A

Atherosclerosis

681
Q

What is atherosclerosis?

A

Formation of atheromas (plaques)

682
Q

What form of arteriosclerosis is age-related?

A

Monckeberg medial sclerosis

683
Q

What organs are most sensitive to hyperplastic arteriolosclerosis?

A

kidneys

684
Q

Which arteriole reaction occurs in response to benign hypertension?

A

hyaline arteriolosclerosis

685
Q

Which type of arteriole reaction occurs in response to severe hypertension?

A

Hyperplastic arteriolosclerosis

686
Q

Onionskin appearance is indicative of what arteriole response to hypertension?

A

Hyperplastic arteriolosclerosis

687
Q

Prolonged diabetes mellitus is associated with which arteriole response to hypertension?

A

Hyaline arteriolosclerosis

688
Q

What is the number one cause of morbidity and mortality in the U.S.?

A

Atherosclerosis (CAD, myocardial infarction, stroke)

689
Q

What effect does atherosclerosis have on blood flow?

A

decrease

690
Q

What layer of the vessel is weakened in atherosclerosis?

A

Tunica media

691
Q

A weakened tunica media is a risk for what issue?

A

Aneurysm

692
Q

What are the foam cells seen in an atherosclerosis plaque?

A

Fat-laden macrophage

693
Q

Which type of plaque has inflammation present, as well?

A

vulnerable

694
Q

Which type of plaque has a fibrous cap providing support?

A

stable plaque

695
Q

Too many LDLs and too little HDLs (hyperlipidemia) increase the risk of what vascular condition?

A

Atherosclerosis

696
Q

What usually causes hypertension?

A

95% idiopathic

697
Q

What is the response to injury hypothesis involved with atherosclerosis?

A

Chronic endothelial injury —> atherosclerosis

698
Q

Metabolic syndrome presents a risk for what condition?

A

CVD

699
Q

What issues can make up metabolic syndrome?

A

Central obesity, hypertension, insulin resistance, dyslipidemia, hypercoagulability/pro-inflammatory state (adipokines)

700
Q

Peripheral vascular disease can lead to what condition following atherosclerosis?

A

gangrene

701
Q

What is the difference between a true and false aneurysm?

A

True aneurysm - all three layers of a vessel spread out

False aneurysm - defect in vascular wall leading to extravascular hematoma

702
Q

What are some common locations for a true aneurysm?

A

Aortic arch, abdominal aorta, iliac arteries

703
Q

What is the size of an AAA?

A

Dilation of >50% of normal

Diameter >5cm

704
Q

Who is most at risk for an aortic dissection?

A

males 40-60

705
Q

What type of genetic pattern is seen with Wilson disease?

A

autosomal recessive

706
Q

What is Wilson disease?

A

Abnormal copper ion transpiration leading to excessive copper accumulation

707
Q

How is the liver affected by Wilson disease?

A

Steatohepatitis

708
Q

How is the brain affected by Wilson disease?

A

Psychosis, Parkinsonism (the movement disorder, not Parkinson’s disease)

709
Q

How does Wilson disease affect the eye?

A

Kayser-Fleischer ring

710
Q

Pain with an aortic dissection can resemble what other condition?

A

MI

711
Q

Where is pain felt with an aortic dissection?

A

Anterior chest, projects posteriorly between the scapulae

712
Q

What type of aortic dissection is the most common and most severe?

A

type A

713
Q

Which type of aortic dissection is in the ascending aorta?

A

type A

714
Q

Which type of aortic dissection is seen in the left subclavian artery?

A

type B

715
Q

Vasculitis is most common in what kind of arteries?

A

small arteries

716
Q

What kind of infection could cause vasculitis?

A

hepatitis B

717
Q

What most common type of vascular tumor?

A

benign

718
Q

A Kaposi sarcoma is an example of what type of vascular tumor?

A

fairly aggressive

719
Q

An angiosarcoma is an example of what type of vascular tumor?

A

Rare and highly malignant sarcoma

720
Q

What is a hemangioma?

A

Common benign vascular tumor affecting 1:200 newborns

721
Q

What virus is responsible for a Kaposi sarcoma?

A

HHV-8

722
Q

Angiosarcomas are more common among what population?

A

older adults

723
Q

What are the most common locations for angiosarcomas?

A

skin, breast, liver

724
Q

How can endovascular stenting affect the luminal wall layers?

A

Possible intimal hyperplasia

725
Q

What causes giant cell arteritis?

A

Idiopathic but T cell activity and autoimmune hypothesis

726
Q

Giant cell arteritis involves what arteries?

A

large and small arteries of the head

727
Q

What artery is most commonly affected during giant cell arteritis (50% of cases)?

A

ophthalmic artery

728
Q

What is usually the quick treatment for giant cell arteritis?

A

Corticosteroids (especially to prevent blindness)

729
Q

What kind of inflammation is present in giant cell arteritis?

A

Granulomatous

730
Q

What is also known as the “pulseless disease”?

A

Takayasu arteritis

731
Q

What form of arteritis is exclusively seen in the aortic arch and its branches?

A

Takayasu arteritis

732
Q

What causes the loss or minimized pulse of Takayasu arteritis?

A

Narrowing of the lumen of branches off of the aortic arch

733
Q

Takayasu arteritis is most common among what population?

A

Younger individuals

734
Q

What kind of necrosis is seen with polyarteritis nodosa?

A

transmural necrosis

735
Q

What is polyarteritis nodosa?

A

System wide vasculitis of small and medium sized arteries

736
Q

What can happen with polyarteritis nodosa?

A

Tissue atrophy, ischemia, infarction

737
Q

What arteries are avoided by polyarteritis nodosa?

A

pulmonary

738
Q

Polyarteritis nodosa is most common among what population?

A

young adults

739
Q

What chronic infection is seen among 1/3 of polyarteritis nodosa cases?

A

hepatitis B

740
Q

Around what fraction of polyarteritis nodosa cases are said to be autoimmune or idiopathic in nature?

A

2/3

741
Q

What is seen with polyarteritis nodosa of the renal artery?

A

Rapidly increasing blood pressure (renovascular hypertension)

742
Q

What is seen with polyarteritis nodosa of the GI arteries?

A

abdominal pain, bloody stools

743
Q

What is the treatment for polyarteritis nodosa?

A

Corticosteroids

744
Q

What happens if polyarteritis nodosa is left untreated?

A

Fatal

745
Q

Segmental fibrinoid necrosis and thrombosis is seen among what widespread vascular condition?

A

Polyarteritis nodosa

746
Q

The “rosary sign” is seen among what vascular condition?

A

Polyarteritis nodosa

747
Q

Kawasaki disease primarily affects what population?

A

pediatrics

748
Q

What is the fatality rate of Kawasaki related heart attacks in children?

A

1%

749
Q

How does CAD influence the prognosis of Kawasaki disease?

A

Patients without CAD recover fully

Severity of CAD determines prognosis

750
Q

What is the cause of Kawasaki disease?

A

Idiopathic but suggestive of type IV hypersensitivity and possibly viral antigens, genetics

751
Q

What gender and race are more likely to get Kawasaki disease?

A

Asian descent and males

752
Q

What is the hallmark sign of Kawasaki disease?

A

Acute/persistent fever

753
Q

What are the unique signs of the skin and tongue seen with Kawasaki disease?

A

Desquamation of extremities, strawberry tongue (oral erythema)

754
Q

What type of hypersensitivity is seen with Wegener granulomatosis?

A

type 2

755
Q

Wegener granulomatosis is seen in what locations?

A

kidneys and upper/lower respiratory tracts

756
Q

What is seen with Wegener granulomatosis?

A

Granulomas, systemic vasculitis of small/medium arteries, glomerulonephritis

757
Q

What population is more likely to be affected by Wegener granulomatosis?

A

Middle aged (40 years old), males

758
Q

What lung condition is seen in 95% of cases of Wegener granulomatosis?

A

Bilateral pneumonitis

759
Q

How are the kidneys affected by Wegener granulomatosis?

A

Hematuria, proteinuria, possible renal failure

760
Q

How is the nasopharyngeal area affected by Wegener granulomatosis?

A

Rhinitis, sinusitis, nose bleeds

761
Q

What is another name for Buerger disease?

A

Thromboangiitis obliterans

762
Q

What group of people are at risk for thromboangiitis obliterans?

A

Heavy tobacco smokers

763
Q

Vascular insufficiency of what arteries and locations is seen with thromboangiitis obliterans?

A

Radial and tibial arteries, leading to cold feet and hands

764
Q

What is unique about the pain felt with thromboangiitis obliterans?

A

seen at rest

765
Q

What is the most common vasculitis in older adults?

A

Giant cell arteritis (temporal arteritis)

766
Q

What kind of condition is Raynaud phenomenon?

A

Exaggerated arteriole vasoconstriction

767
Q

In chronic conditions, what can result from Raynaud phenomenon?

A

Atrophy of skin, connective tissue, and/or muscles

768
Q

What are traditional triggers for primary Raynaud phenomenon?

A

Cold and emotional triggers (5% of the U.S.)

769
Q

Raynaud phenomenon can be secondary to what other conditions?

A

Atherosclerosis, Buerger disease, Lupus, scleroderma

770
Q

What condition is also known as “broken heart syndrome”, stress cardiomyopathy, or “cardiac Raynaud”?

A

Takotsubo cardiomyopathy

771
Q

What is the location of exaggerated vasoconstriction of Takotsubo cardiomyopathy?

A

coronary arteries

772
Q

What is usually the cause of broken heart syndrome?

A

psychological stress

773
Q

What kinds of hormones are produced more heavily with cardiac Raynaud?

A

Catecholamine (stress hormones)

774
Q

What occurs with Takotsubo cardiomyopathy?

A

Vasospasm, increased heart rate and contractility, fatal arrhythmia (ventricular fibrillation), possible ischemia and heart attack in severe cases

775
Q

What is the term for venous inflammation?

A

Phlebitis

776
Q

90% of venous disease fall into what three categories?

A

Varicose veins, phlebothrombosis, thrombophlebitis

777
Q

What is phlebothrombosis?

A

Venous thrombosis without previous inflammation

778
Q

What is thrombophlebitis?

A

Venous thrombosis following inflammation

779
Q

What is a risk factor for thrombophlebitis?

A

Immobilization (examples: recent surgery, pregnancy, obsesity), congestive heart failure, genetics, any pro-thrombotic medication, oral contraceptive

780
Q

What could be the first indication of thrombophlebitis?

A

Pulmonary embolism

781
Q

What test can be used to diagnosis thrombophlebitis?

A

Dorsiflexion of the foot (Homan sign)

782
Q

What is important to note with the Homan sign?

A

Could produce clot or embolism (only about 50% sensitive to thrombophlebitis)

783
Q

What are signs of superior vena cava syndrome?

A

Congested upper extremity veins, edema in head, neck, and arms

784
Q

What conditions are associated with superior vena cava syndrome?

A

Bronchogenic carcinoma, mediastinal lymphoma

785
Q

What conditions are associated with inferior vena cava syndrome?

A

Hepatocellular carcinoma, renal cell carcinoma

786
Q

What are some signs of inferior vena cava syndrome?

A

Congestion of lower extremity veins, edema, proteinuria

787
Q

Which form of lymphedema is congenital?

A

primary

788
Q

What is another name for congenital lymphedema?

A

Milroy disease

789
Q

What can cause secondary lymphedema?

A

Neoplasia, infection (filariasis), thrombosis, fibrosis (surgery)

790
Q

What appearance of the skin is associated with acute lymphedema?

A

Peau d’orange

791
Q

What skin appearance is seen with chronic lymphedema?

A

Brawny induration (hardening of the skin)

792
Q

What is the term for when there is passage of blood through layers of a vascular wall?

A

dissection

793
Q

What makes up the tunica intima?

A

endothelial cells

794
Q

What makes up the tunica media?

A

smooth muscle

795
Q

What makes up the tunica adventitia?

A

CT, nerves, vessels

796
Q

Activation of what part of the vascular vessel wall via vascular lesions or thrombosis is pro-inflammatory?

A

endothelial lining

797
Q

What is the function of the endothelial cells?

A

Regulate vasoreactivity by NO & endothelin

Regulate cell growth

798
Q

What is fibromuscular dysplasia?

A

Local thickening of medium/large artery walls that leads to ischemia (non-atherosclerotic and non-inflammatory)

799
Q

Fibromuscular dysplasia is more common with what gender?

A

females

800
Q

Aldosterone helps regulate blood volume and vascular tone in what area?

A

adrenals

801
Q

Atrial natriuretic peptide (ANP) helps regulate vascular tone and blood volume in what organ?

A

heart

802
Q

How does atrial natriuretic affect the heart and blood pressure?

A

Stretches heart and decreases blood pressure (reduced sodium resorption)

803
Q

The kidney can produce what substances that initiate vasodilation and decrease blood pressure?

A

Prostaglandins and NO

804
Q

Without treatment, 50% of those with hypertension will die from what complication?

A

MI

805
Q

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

A

tau proteins

806
Q

What is the culprit for CNS degeneration?

A

abnormal protein accumulation

807
Q

What is Parkinsonism?

A

Abnormal motor function, tremor, rigidity, bradykinesia, instability

808
Q

What neurons are damaged in Parkinsonism?

A

dopaminergic neurons

809
Q

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

A

lewy bodies

810
Q

What is the genetic factor involved in Huntington disease?

A

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

811
Q

Chorea of the entire body is associated with what condition?

A

huntington disease

812
Q

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

A

ALS

813
Q

What is significant about the stages of CNS tumors?

A

No premalignant (in situ) stages

814
Q

What are the three broad categories of gliomas?

A

Astrocytoma, oligodendroglioma, ependymoma

815
Q

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

A

Astrocytoma and oligodendroglioma

816
Q

Are diffuse astrocytomas benign or malignant?

A

malignant

817
Q

What is the most common location for ependymomas in pediatrics?

A

periventricular regions

818
Q

What is the most common mechanism of heart disease?

A

Contractile (pump) failure

819
Q

What are the risks for systolic dysfunction?

A

CAD, systemic hypertension, decreased pH (shock)

820
Q

What causes systolic dysfunction?

A

weak contraction

821
Q

What causes diastolic dysfunction?

A

failure to relax which inhibits filling

822
Q

What is common with an adult form of aortic coarctation?

A

Infolding near the ligamentum arteriosum (asymptomatic)

823
Q

What is the most common cause of left-side heart failure?

A

Ischemic heart disease (CAD)

824
Q

How can left-sided heart failure affect the pulmonary system?

A

Decreased cardiac output leads to pulmonary edema

825
Q

How is heart rate affected by left-sided heart failure?

A

Tachycardia (Over 100bpm)

826
Q

Right-sided heart failure most commonly results from what prior condition?

A

Left-sided heart failure (backward failure)

827
Q

What is the condition involved with pulmonary hypertension and isolated right-sided heart failure?

A

cor pulmonale

828
Q

What valves can be impaired in right-sided heart failure?

A

Pulmonary or tricuspid

829
Q

Peripheral congestion and edema are seen in what vascular structures during right-sided heart failure?

A

systemic and portal veins

830
Q

What are some clinical features seen with right-sided heart failure?

A

Ascites and hepatosplenomegaly

831
Q

Over half of congenital heart disease cases involve which septal defects?

A

Ventricular and atrial septal defects

832
Q

What defects can result from right-to-left shunts in congenital heart disease?

A

Tetralogy of Fallot, transposition of great arteries

833
Q

What type of septal defects is most commonly asymptomatic until adulthood?

A

atrial septal defect

834
Q

A patent ductus arteriosus is associated with what type of shunt?

A

Left-to-right

835
Q

What is the most common type of congenital heart disease to cause cyanosis?

A

Tetralogy of Fallot

836
Q

What are the four features of tetralogy of Fallot?

A

Large ventricular septal defect
Valve stenosis with right ventricular outflow obstruction
Overriding aorta between ventricles
Right ventricular hypertrophy

837
Q

What are the features of coronary atherosclerosis?

A

Inflammation, thrombosis, vasoconstriction

838
Q

What is the unique type of angina pectoris?

A

Variant angina (Prinzmetal angina)

839
Q

Which artery is most likely to be involved in a heart attack?

A

Left anterior descending coronary artery (40-50%)