Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

NMS/GU > Test #2 > Flashcards

Test #2 Flashcards

1
Q

Failure of development of a bone; congential absence of a rib or clavicle or fingers or toes

A

Adactyly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

A congenital abnormality characterized by an absence of upper or lower extremities or both

A

Amelia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Fusion of the fingers or the toes

A

Syndactyly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Development of long “spider-like fingers”; classic feature of Marfan’s syndrome; also observed in various disorders involving gnees that encode extracellular matrix proteins as in Ehlers Danlos Syndrome

A

Arachnodactyl

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Type of dwarfism that is caused by a deficiency of thyroid hormones

A

Cretinism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

This type of dwarfism is associated with pituitary abnormalities

A

Growth Hormone secretion (deficiency?)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

This type of dwarfism is caused by a deficiency of growth hormone receptors

A

Laron-type dwarfism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

A congenital form of dwarfism caused by abnormal formation of epiphyseal growth plates of long tubular bones

A

Achondroplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

The deficit for Achondroplasia lies in genes encoding for what?

A

Fibroblast Growth Factor Receptor-3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Individuals with this disorder have short limbs, normal trunks, & relatively large heads. Longevity, intelligence, or reproductive status are not affected

A

Achondroplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

This type of dwarfism usually causes death at birth or shortly thereafter. Caused by mutation of FGFR-3

A

Thanatophoric Dwarfism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Charateristics of Thanatophoric dwarfism

A

shortening of limbs, relative macrocephaly, small chest cavity, bell-shaped abdomen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Disorder where too little bone is formed due to a mutation of the genes encoding the chains of Type I collagen

A

Osteogenesis Imperfecta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

A genetic disorder characterized by abnormal thickening of bones

A

Osteopetrosis AKA Marble Bone Disease AKA Albers-Schonberg Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Osteopetrosis is caused by?

A

An undefined defect in the juntion of osteoclasts, which do not reabsorb the bone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Osteopetrosis causes obliteration of the marrow space which leads to what?

A

anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

A deficiency of this enzyme in osteopetrosis keeps osteoclasts from solubilizing hydroxyapatite crystals

A

Carbonic anhydrase II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Type of osteopetrosis where mutation in the genes causes osteoclast dysfunction by interfering w/ what?

A

chloride channels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

A disease characterized by increase porosity of the skeleton resulting from reduced bone mass

A

Osteoporosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Most common forms of osteoporosis?

A

Senile & post menopausal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What two endocrine diseases are assoc. w/ osteoporosis?

A

Cushing syndrome & hyperthroidism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Literally means “lack of bone”; radiologic term used to denote a decreased amount of bone visible on x-ray

A

Osteopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Characterized by a defect in matrix mineralization related most often to lack of or disturbance of metabolism of Vit. D

A

Rickets (children)/Osteomalcia (Adults)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Miscellanceous bone disease of uncertain cause; characterized by a “collage of matrix madness”; caused by slow viral infection of paramyzovirus

A

Osteitis Deformans (Paget’s Disease)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Paget’s Disease can cause bone overgrowth can cause the bone to be heavier than normal which will lead to what?

A

Secondary osteoarthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What levels are elevated in Paget’s disease?

A

Blood alkaline phosphoatase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Symptoms for Paget’s Disease are readily suppressed by what?

A

Calcitonin & biphosphonates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Assoc. w/ increased bone resorption of cortical bone & replacement of bone trabeculae w/ fibrous tissue

A

Osteitis Fibrosa Cystica AKA Von Recklinghausen Disease AKA Hyperparathyroidism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

The most typical changes in Osteitis Fibrosa Cystica are seen where?

A

Middle phalanges of the index & middle fingers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Bone loss in Osteitis Fibrosa Cystica leads to microfractures & secondary hemorrhages that elicit an influx of multinucleated macrophages & an ingrowth of fibrous tissue creating what?

A

Brown tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What is the hallmark of severe hyperparathyroidism?

A

Increased bone cell activity peritrabecular fibrosis & cystic brown tumors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Skeletal changes of chronic renal disease such as increased osteoclastic bone resorption mimicking Osteitis Fibrosa Cystica, delayed matrix mineralization, osteosclerosis, growth retardation, & osteoporosis

A

Osteodystrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Two major types of osteodystrophy

A

High turnover osteodystrophy & Low turnover disease (aplastic)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Osteodystrophy is found in what type of patient?

A

Chronic renal dialysis patient

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Osteodystrophy causes deposits of what two substances?

A

Aluminium & amyloid (derived from beta-2-microglobulin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Osteonecrosis can be caused by?

A

mechanical vascular interruption (fracture, corticosteroids, thrombosis & embolism, vessel injury, increased interrosseous pressure, venous hypertension

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

These type of infarcts cause chronic pain

A

Subchondral infarcts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

These type of infarcts are silent except for large ones

A

Medullary infarcts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Results from bacterial seeding of bone by hematogenous spread, extension from contiguous infection, & open fx of surgical procedure

A

Pyogenic Osteomyelitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Organism that usually causes Pyogenic Osteomyelitits?

A

Staph. Aureus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Patients w/ sickle cell anemia are prone to what type of infection?

A

Salmonella infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

TB osteomyelitis of the spine is known as what?

A

Potts Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Congenital form of syphilitic bone disease appears at birth & is marked by what?

A

Periostitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Congenital form of syphilitc bone disease gives what appearance on xray?

A

Crew-haircut like appearance & new bone formation on cortex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Saber shin of the tibia is seen with which type of Syphilitic bone disease?

A

Congential

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

The acquired form of syphilitic bone disease appears in what form & is manifested by what in the bone?

A

Tertiary form; gummas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Results from incompletely healed or persistent suppurative acute infection (draining sinus tract has developed)

A

Chronic osteomyelitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Lytic enzymes from the inflammatory cells cut channels through the bone leading to the formation of bone fragments that ultimately detach from the main bone. This is known as what?

A

Sequestrum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Wall of the abscess cavity is composed of newly formed bone which is known as?

A

Involucrum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Hat size may increase with this disease?

A

Paget Disease (Osteitis Deformans)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Paget’s is characterized by what?

A

“Collage of matrix madness”; periods of severe osteoclastic activity followed by hectic bone formation which is laid down in a disorderly fashion which is architecturally unsound

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Paget’s disease may lead to what?

A

Severe secondary osteoarthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Paget’s disease increases the risk for what?

A

Osteosarcoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Paget’s Disease (Osteitis Deformans) is caused by a slow viral infection by what virus?

A

Paramyxovirus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

How does paramyxovirus affect osteoclasts?

A

Enters cytoplasm & nucleus increasing their resorptive activity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

High-output cardiac failure in Paget’s results from what?

A

Multiple functional arteriovenous shunts w/i highly vascular early lesions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Hearing loss in Paget’s is caused by what?

A

Narrowing of the auditory foramen or direct involvement of the bones of the middle ear

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Paget’s increases what enzyme’s activity?

A

serum alkaline phosphatase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Osteopetrosis is AKA what?

A

Marble bone disease, Albers-Schonberg disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

What is osteopetrosis?

A

Genetic disorder characterized by abnormal thickening of bones

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

What is the cause of osteopetrosis?

A

Unidentified defect in the function of osteoclasts causing them to not reabsorb bone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

How does osteopetrosis cause anemia?

A

Bones grow but don’t remodel which lead to obliteration fo bone marrow spaces resulting in anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Fibrous replacement of resorbed bone may lead to formation of non-neoplastic tumor-like masses known as?

A

Brown tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

Brown tumors are due to what?

A

Hyperthyroidism; high levels of PTH leads ot cystic changes in bone due to osteoclastic resorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

The brown color of a brown tumor is due to what?

A

Vascularity, hemorrhage, & hemosidirin deposition

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

Hyperparathyroidism is AKA?

A

Osteitis fibrosa cystica & Von Recklinghausen disease of bone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

Almost all joint arthropathies are assoc. w/ what?

A

Inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

Two types of osteoarthritis AKA DJD

A

Primary DJD & Secondary DJD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

This type of DJD most often occurs after 50 years of age w/o an obvious cause

A

Primary DJD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

This type of DJD occurs in joints damaged by known mechanisms, including mechanical factors (trauma), congenital malformation, metabolic injury of chondrocytes (cartilage cells)

A

Secondary DJD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

Fragmented cartilage which may float freely in the articular cavity

A

Joint mice

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

The biochemical changes in DJD mainly involve what?

A

Proteoglycans (mucopolysaccharides)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Bone that appears like ivory

A

Bone eburnation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

These are produced by reactive bone formation at the margins of the joint

A

Osteophytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Osteoblasts develop when the mysenchymal tissue of the synovium does what?

A

Differentiates into osteoblasts & chondroblasts to form a mass of cartilage & bone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

Osteophytes may cause compression of?

A

Cervical & lumbar nerve roots causing pain, muscle spasms, & neurological deficit

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

Osteophytes are the base of the distal interphalangeal joints are called?

A

Heberden nodes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

Osteophytes at the proximal interphalangeal joints are called?

A

Bouchard nodes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

Clinical features of osteoarthritis

A

Deep aching pain w/ movement of joints along w/ stiffness
Morning joint stiffness for less than 30 mins
Joint pain is often worse later in the day w/ activity
Pain relieved by rest
Restricted joint motion
Crepitations
Reactive bone formation at the margins of the joints produces osteophytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

This is a systemic disease of unknown etiology that is assoc. w/ chronic inflammatory disorder primarily affecting the synovial joints but other organs of the body can be affected as well

A

Rheumatoid arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

Joints that are typically involved in RA

A

Small hand joints, especially prox. interphalangeal & metacarpophalangeal joint, foot joints, especially the metatarsophalangeal joint, wrist & elbows

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

Important genetic locus that predisposes to RA?

A

Human Leukocyte Antigen (HLA) II Genes; especially a specific set of HLA-DR alleles DR4, DR1, DR10, DR14

83
Q

Whats the ratio of men to women affected by RA?

A

Women affected more than men 3:1

84
Q

Typically disease onset occurs when?

A

b/w 30-60 years

85
Q

What % of pts w/ classic RA are positive for Rheumatoid Factor (RF)?

A

80%

86
Q

Rheumatoid Factor represents which antibodies?

A

Mostly IgM, but sometimes IgG or IgA

87
Q

The presence of IgG type RF is sometimes assoc. w/ what?

A

Development of necrotizing vasculitis

88
Q

What test is most commonly employed for the diagnosis of RA?

A

Anticyclic Citrunillated Protein Antibody (anti-CCP) test

89
Q

How often is the anti-CCP test positive (sensitivity)?

A

about 67% of cases (about roughly the same as seen in RF)

90
Q

Why is the anti-CCP test important?

A

Rarely positive in the absence of RA (high specificity)

91
Q

What is vimentin?

A

Vimentin is secreted & citrullinated by macrophages in response to apoptosis, or by proinflammatory cytokines, such as tumor necrosis factor-alpha (TNF-alpha)

92
Q

A manifestation of lymphoplasmacytic lymphoma, a B-cell neoplasm of lymphoid cells of an intermediate stage b/w B lymphocytes & plasma cells referred to as plasmacytoid lymphocytes

A

Waldenstrom macroglobulinemia

93
Q

What makes Waldenstrom macroglobulinemia unique?

A

The neoplastic cells produce a monoclonal IgM immunoglobulin of either kappa or lambda specificity occurring as an M protein

94
Q

Light skinned individuals develop darkening of the skin

A

Kala azar

95
Q

What does Kala azar mean?

A

Black sickness

96
Q

What organisms cause Kala azar?

A

Leishmaniae (protozoans) that are transmitted to humans by insect bites (Sandflies)

97
Q

What are the symptoms of Kala azar?

A

Unrelenting fever, progressive weight loss, enlarged spleen & liver, anemia, thrombocytopenia, & leukopenia

98
Q

Fibrin that aggregate into small bodies is known as what?

A

Rice bodies

99
Q

Lymphocytes that aggregate into lymphoid follicles is known as?

A

Allison-Ghormley bodies

100
Q

Due to contraction of the scarred joint capsule, chronic RA leads to typical joint deformities known clinically as what?

A

Opera Glass deformity

101
Q

This covers articular cartilage & isolates it from synovial fluid

A

Pannus

102
Q

Pannus erodes the articular cartilage & adjacent bone probably through secretion of what?

A

Collagenase

103
Q

When a joint gets destroyed & undergoes fibrous fusion, it’s called what?

A

Ankylosis

104
Q

What is bony ankylosis?

A

Long-term cases of RA may lead to bony bridging of the joint

105
Q

Where are rheumatoid nodules most often located?

A

Extensor side of the forearm

106
Q

Diseases that involve the joints of the vertebral columns

A

Spondyloarthropathy

107
Q

Syndrome composed of seronegative arthritis, urethritis, conjunctivitis

A

Reiter’s Syndrome

108
Q

Form of arthritis seen in Whipple diseae?

A

Enteropathic artritis

109
Q

Type of arthritis that follows genital infections & most frequently involves the knee; most often monoarticular

A

Gonococcal arthritis

110
Q

This arthritis is caused by infection w/ Borrelia burgdorferi which is transmitted to humans via Ixodes dammini tick

A

Arthritis of Lyme Disease

111
Q

Name of the skin lesions in the first stage Lyme disease?

A

Bull’s-eye lesion

112
Q

This arthritis is caused by staph. or mixed bacteria that may be encountered in immunosuppressed drug addicts prone to septicemia

A

Suppurative arthritis

113
Q

A chronic, progressive, monoarticular arthritis that affects weight-bearing joints. Systemic symptoms may or may not be present

A

Tuberculous arthritis

114
Q

A form of non-inflammatory arthritis characterized by progressive joint destruction due to a primary neurological disorder such as peripheral neuropathy or central motor abnormality

A

Neuropathic Joint disease (Charcot Joint)

115
Q

Small cystic nodule arising in the tendon sheath or the joint capulse of the wrist is thought to be caused by myxoid degeneration of connective tissue

A

Ganglion cyst

116
Q

Assoc. of the pulmonary rheumatoid nodules w/ other pneumoconioses, such as silicosis or asbestosis

A

Caplan’s Syndrome

117
Q

Metabolic disease characterized by hyperuricemia w/ plasma uric acid level of more than 7mg/dl

A

Gouty Arthritis

118
Q

Type of gout that is idiopathic and accounts for approx. 90% of all cases of gout

A

Primary gout

119
Q

Type of gout that is occurs as a complication of other diseases

A

Secondary gout

120
Q

Disease caused by lead nephropathy

A

Saturnine gout

121
Q

Urate crystals in a protein matrix surrounded by fibrous connective tissue, demonstrating a foreign body giant cell reaction; located about joints, in the helix, & antihelix of the ear

A

Tophi

122
Q

Competitive inhibitor of anthine oxidase, the enzyme that converts xanthine & hypoxanthine to uric acid

A

Allopurinol

123
Q

Calcium Pyrophosphate Dihydrate Deposition Disease is AKA?

A

Pseudogout or Chondrocalcinosis

124
Q

Heritable condition in which activity of alkaline phosphatase in serum & tissue is deficient

A

Hypophosphatasia

125
Q

Passive transplacental transfer of IgG antibodies from the mother w/ lupus

A

Neonatal Lupus sydrome

126
Q

Chronic skin disease characterized by well demarcated, erythmatous discoid plaques, typically on the face & scalp but w/o systemic symptoms

A

DISCOID Lupus erythmatous

127
Q

Disease where lesions of the embryonic CNS predominate in the periventricular areas & are characterized by necrosis & calcification

A

Cytomegalovirus Encephalitis

128
Q

A relentlessly destructive focal disease caused by JC virus; classic feature is a peripheral area of demylination that contains enlarged oligodendrocytes

A

Progressive Multifocal Leukoencephalopathy

129
Q

An uncommon disease that affects children several years after a known attack of measles. Characterized by degeneration of neurons in the cerebral gray matter & basal ganglia

A

Subacute Sclerosing Panencephalitis (SSPE)

130
Q

Slow virus that consists of protein only (no nucleic acid)

A

Prions

131
Q

Most common form of human prion disease w/ predominant symptom of dementia & possibly ataxia

A

Creutzfeldt-Jakob Disease

132
Q

Type of CJD that accounts for about 75% of all cases; cause is not known

A

Sporadic CJD

133
Q

Characterized by a profound disturbance of sleep-wake dysfunction are common; dysautonomia, abnomral endocrine function, & cerebral dysfunction are common; caused by point mutation in codon 178 of the PRNP gene

A

Inherited CJD: B2-Fatal familial Insomnia

134
Q

A condition in which a disagreeable sensation is produced by ordinary stimuli

A

Dysesthesias

135
Q

Occurs mainly in cannibalistic tribes of Papua, New Guinea, where the disease is believed to be transmitted by eating brain tissue of infected humans

A

Kuru

136
Q

A systemic granulomatous disease of unkown cause, especially involving the lungs w/ resulting fibrosis, but also involving lymph nodes, skin, liver, spleen, & pharyngeal bones & parotid glands

A

Sarcodosis

137
Q

A condition resulting from widespread dissemination of carcinoma in multiple sites in various organs or tissues of the body

A

Carcinomatosis

138
Q

Inflammation of the intima of an artery

A

Endoarteritis

139
Q

Microglia in elongated forms encrusted w/ iron

A

Rod cells

140
Q

Inflammation of the cellular membrane lining the central canal of the spinal cord & the brain ventricles

A

Nodular ependymitis

141
Q

An indefinite term applied to nodular inflammatory lesions, usually small or granular, firm, persistent, & containing compactly grouped mononuclear phagocytes

A

Granuloma

142
Q

Protozoa parasite that has its definitive cycle in the intestines of cats

A

Toxoplasma gondii

143
Q

Mosquito-borne, hemolytic, febrile illness

A

Cerebral malaria

144
Q

AKA sleeping sickness

A

African trypanosomiasis

145
Q

Due to the larval form of Taenia solium, the pork tapeworm

A

Cysticercosis

146
Q

Due to the larval form of Echinococcus granulosus

A

Hydatid cyst

147
Q

Due to Entamoeba histolytica which causes brain abscess

A

Amebiasis

148
Q

Star-shaped cells w/ a process containing bundles of fibrils composed of glial fibrillary acidic protein; process extends into the surrounding tangled network of unmyelinated nerve fibers

A

Astrocytes

149
Q

Form myelin sheathes around axons in the CNS by enveloping them w/ concentric layers of plasma membrane; can be distinguished from astrocytes by having fewer & thinner processes; they don’t possess gap junctions

A

Oligodendrocytes

150
Q

Smaller glial-type cells of uncertain origin that act as scavenger cells

A

Microglia

151
Q

Inherited disturbances in the formation & preservation of myelin

A

Leukodystrophies

152
Q

An autosomal recessive disorder of myelin metabolism that is characterized by the accumulation of cerebroside (galactosyl sulfatide) in the white matter of hte brain & peripheral nerves; M/C leukodystrophy

A

Metachromatic leukodystrophy

153
Q

A rapidly progressive, invariably fatal, autosomal recessive disorder, caused by a deficiency of galactocerebroside beta-galactosidase; defined by the presence of perivascular aggregates of mononuclear & multinucleated “globoid cells” in the white matter

A

Krabbe Disease AKA Globoid Cell Dystrophy

154
Q

Macrophages that contain undigested galactocerebroside (galactosylceramide)

A

Globoid cells

155
Q

An X-linked (Xq28) inherited disorder in which dysfunction of the adrenal cortex & demyelination of the nervous system are assoc. w/ high levels of saturated very-long-chain fatty acids in tissues & body fluids

A

Adrenoleukodystrophy

156
Q

A rare, neurological disorder of infants, characterized by a loss of myelin in the brain & a striking accumulation of irregular, extracellular fibers (known as Rosenthal fibers) in astrocytes; caused by mutations in the gene encodgin Glial Fibrillary Acidic Protein

A

Alexander Disease

157
Q

A rare group of diseases believed to have a pathogenesis similar to that of experimental allergic encephalomyelitis

A

Acute Disseminated Encephalomyelitis

158
Q

M/C cause of dementia in the elderly; most often presents w/ subtle onset of memory loss followed by a slowly progressive dementia that has a course of several years

A

Alzheimer’s Disease

159
Q

Any one of a series of two or more different genes that may occupy the same position (or locus) on a specific chromosome

A

Allele

160
Q

An extremely uncommon cause of presenile dementia, occurring in the age group of 40-65 yrs; Hallmark is symmetric lobar atrophy of temporal &/or frontal lobes which can be visualized by neuroimaging studies & is readily apparent at autopsy

A

Pick’s Disease

161
Q

Swollen or ballooned surviving neurons which frequently contain silver-staining cytoplasmic inclusions referred to as?

A

Pick bodies

162
Q

Characterized by proteinaceous tissue deposits w/ common morphological, structural, & staining properties but w/ variable protein composition

A

Amyloidosis

163
Q

A rare disease that is inherited as an autosomal dominant trait characterized by involuntary movements of all parts of the body, deterioration of cognitive dysfunction, & often severe emotional disturbance

A

Huntington’s Disease (Huntington’s Chorea)

164
Q

Disease characterized by loss of neurons, primarily in substantia nigra & several other brainstem nuclei

A

Idiopathic Parkinson’s Disease

165
Q

Intracytoplasmic rounded eosinophilic inclusion bodies that contain neuronal proteins, including alpha-synuclein

A

Lewy Bodies

166
Q

A progressive encephalomyopathy involving the autonomic system, characterized by intractable hypotension, external opthalmoparesis, atrophy of the iris, incontinence, anhydrosis, impotence, tremor, & muscle wasting

A

Shy-Drager Syndrome

167
Q

An inability to coordinate the muscle in the execution of voluntary movement

A

Ataxia

168
Q

A degenerative disease of motor neurons of the brain & spinal cord that results in progressive weakness & wasting of the extremities & eventually impairment of the respiratory muscles

A

Amyotrophic Lateral Sclerosis

169
Q

Deficiency of this nutrient causes pellagra

A

Niacin

170
Q

3 D’s of pellagra

A

Dermatitis, Diarrhea, Delirium/Dementia

171
Q

Vit. B1 deficiency causes what?

A

Beriberi

172
Q

Vit. B6 causes what problems in children?

A

Irritability & seizures

173
Q

Vit. B12 deficiency causes what?

A

Pernicious Anemia

174
Q

This is characterized by peripheral dilation, warm, dry skin as well as decreased circulation time

A

Cardiac Beriberi

175
Q

A consequence of thiamine deficiency, a nutritionla deficiency commonly found in chronic alcoholics

A

Wernicke-Korsakoff syndrome

176
Q

This is a rare complication characterized by focal areas of selective ,myelin loss w/i the pons

A

Central pontine myelinolysis

177
Q

A rare complication chracterized by coagulative necrosis of midline structures. It is a degenrative process involving the corpus callosum that occurs predominantly in chronic alcoholics, particularly red wine drinkers

A

Marchiafava-Bignami disease

178
Q

Autosomal recessive diseasee affecting copper metabolism

A

Wilson’s Disease

179
Q

A greenish-yellow pigmented ring encircling the cornea just w/i the corneoscleral margin

A

Kayser-Fleishcer Ring

180
Q

A deposition of bilirubin in the nuclei of the basal ganglia of neonates born w/ severe hemolytic anemia

A

Kernicterus

181
Q

A multisystem hamartoma producing the typical triad of seizures, mental retardation, skin nodules of the face

A

Tuberous sclerosis

182
Q

A focal malformation that resembles a neoplasm, grossly & even microscopically, but results from faulty development in an organ

A

Hamartoma

183
Q

Disease where mutation of NF-1 gene has no effect on “ras” which leads to unopposed cell growth leading to tumor formation; classic form of the disease characterized by numerous neurofibromas in the skin & peripheral nerves assoc. w/ cafe au lait spots; results from abnormal gene on chromosome 17

A

Neurofibromatosis

184
Q

Neurofibromatosis is AKA

A

von Recklinghausen’s Disease AKA Elephantiasis Neurofibromatosa AKA Elephant Man Disease

185
Q

M/C primary brain tumor in adults

A

Glioblastoma multiforme

186
Q

M/C primary malignant brain tumor in children

A

Medulloblastoma

187
Q

Cell of origin for an astrocytoma, oligodendroglioma, ependymoma, glioblastoma

A

Glial cells

188
Q

Cell of origin for a medulloblastoma & neuroblastoma

A

Primitive neuroectodermal cells

189
Q

Cell of origin for a meningioma

A

Arachnoidal cell

190
Q

Cell of origin for a schwannoma & neurofibroma

A

Nerve sheath cells

191
Q

Cell of origin for a lymphoma

A

lymphoreticular cells

192
Q

Any neoplasm derived from one of the various types of cells that form the interstitial tissue of the brain, spinal cord, pineal gland, or retina

A

Glioma

193
Q

A Grade IV astrocytoma-a glioma consisting chiefly of undifferentiated anaplastic cells that are precursors of astrocytes

A

Glioblastoma

194
Q

Glioma composed of well-differentiated astrocytes; commonly arise in the cerebellum in children & in the cerebral hemispheres in adults

A

Astrocytoma

195
Q

A neoplasm of children & adolescents & accounts for 25% of intracranial neoplasms in children under 10 yrs. M/C occurs in the cerebellar hemispheres & can be resected completely as these tumors are extremely well circumscribed

A

Juvenile Pilocytic Atrocytoma

196
Q

Uncommon tumor that affects adults (30-50yrs) & occurs most often in cerebral hemispheres. It’s a well-circumscribe slowly growing tumor.

A

Oligodendral Glioma

197
Q

Tumor is derived from ependymla cells lining the ventricle & spinal canal. Occurs at all ages, but is relatively more common in children

A

Ependymoma

198
Q

Malignant tumor derived from neuroectodermal cells. Childhood tumor, it is extremely rare after the age of 40 yrs. Involves vermis of cerebellum in children; in older individuals it may involve the cerebellar hemispheres

A

Medulloblastoma

199
Q

Benign tumor originating from arachnoid cells (meningothelial cells) of the leptomeninges. Occurs most frequently in middle-aged women

A

Meningioma

200
Q

A benign tumor composed of endothelial cells forming small blood vessels surrounded by lipid-laden cells

A

Hemangioblastoma

201
Q

Autosomal dominant cancer syndrome characterized by the development of hemangioblastomas in the brain, retinal angiomas, clear cell renal cell carcinoma, pheopchromocytoma, & cysts in various organs

A

von Hippel-Lindau disease

202
Q

A tumor derived from Schwann cells, which produce both collagen & myelin

A

Schwannoma

203
Q

Believed to be derived from Rathke’s pouch, the epithelial remnant of th eforegut that contributes to the origin of the pituitary gland. Occurs mainly in childhood in the suprasellar region adjacent to the pituitary stalk

A

Craniopharyngioma

NMS/GU (3 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions